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Objective: Pseudoexfoliation (PEX) syndrome is a systemic condition associated with age, and its exact cause remains elusive. Inflammatory processes heighten the risk of PEX development. This study marks the first attempt to jointly investigate the Systemic Inflammatory Response Index (SIRI) and Pan-Immune Inflammation Value (PIV) biomarkers in PEX patients. Materials and Methods: A total of 84 patients and 71 healthy controls underwent examination. Ratios of neutrophils to lymphocytes (NLR) and platelets to lymphocytes (PLR), along with the Systemic Inflammation Index (SII), SIRI, and PIV values, were computed as indicators linked to the inflammatory cascade. Results: The study encompassed 155 subjects, including 71 healthy controls averaging 73.8±7.7 years and 84 PEX patients averaging 71.3±8.9 years. Statistically significant differences in neutrophil and lymphocyte levels were evident between the groups (p<0.05). A notable statistical distinction was observed in the NLR, PLR, derived Neutrophil to Lymphocyte Ratio (dNLR), SII, SIRI, and PIV indices when comparing the groups (p<0.05). However, hemoglobin, platelet, mean platelet volume (MPV), white blood cell (WBC), and C-reactive protein (CRP) values did not show significant differences between the groups (p>0.05). Conclusion: This study highlights that SIRI and PIV could provide insights into the relationship between PEX and inflammation, offering a glimpse into the potential systemic implications of PEX-related inflammation. [ABSTRACT FROM AUTHOR]

Aim: Some hematological changes are frequently observed in the clinical course of brucellosis. This study aimed to predict the diagnosis of brucellosis based on some hematological biomarkers with the help of ensemble classification methods. Materials and Methods: A total of 23 ensemble classification methods, including 10 bagging, 9 boosting, and 4 stacking approaches were applied to the brucellosis data set. Each subject in the brucellosis data set contains 13 features, including age, gender, and 10 hematological variables. Results: This study included a total of 257 participants [173 (67.3%) brucellosis patients and 84 (32.7%) healthy controls]. The mean values of white blood cells (WBC), hemoglobin (HGB), neutrophil (NEUT), neutrophil/lymphocytes (NEUT/LYMP), and monocytes/lymphocytes (MO/LYMP) of brucellosis patients were found to be significantly lower than those of healthy controls. Random Forest with Gini criterion (RF2) was selected to be the best fit model with a mean accuracy of 0.728. HGB (mean score = 0.1814), age (0.1311), NEUT/LYMP (0.0938), WBC (0.0817) and mean platelet volume (MPV) (0.0815) were determined as most diagnostic parameters in brucellosis. Conclusion: The lower levels of HGB, WBC, and NEUT/LYMP and higher levels of age and MPV may be important indicators for the diagnosis of brucellosis. [ABSTRACT FROM AUTHOR]

Les myélodysplasies (SMD) peuvent se présenter sous forme de manifestations systémiques telles que des connectivites ou des vascularites. Des manifestations rhumatologiques sont également décrites. Nous rapportons l'observation d'un patient suivi pour une leucémie myélomonocytaire chronique (LMMC) ayant développé un tableau systémique associant « pseudo-polyarthrite rhizomélique » et péricardite. Un homme de 78 ans consultait pour l'exploration de douleurs des épaules d'horaire inflammatoire associées à un syndrome inflammatoire biologique évoluant depuis deux mois. Il présentait également une altération de l'état général avec asthénie, anorexie et perte de 5 kg en un mois. Il était suivi pour une LMMC depuis trois ans, en abstention thérapeutique. L'ensemble des explorations réalisées montrait un aspect typique de pseudo-polyarthrite rhizomélique. Un épanchement péricardique nécessitant un drainage en urgence était découvert de manière synchrone. L'ensemble des symptômes est survenu de façon concomitante à une aggravation de son hémopathie. L'évolution a été favorable sur le plan articulaire après une courte corticothérapie et la péricardite n'a pas récidivé après 2 ans de suivi. Il faut savoir rechercher un SMD ou accélération de ce dernier devant un tableau systémique, notamment chez des patients âgés, a fortiori, en cas d'anomalie de la NFS évocatrice (cytopénie, macrocytose et monocytose). Myelodysplasia (MDS) can occur as systemic manifestations such as connective tissue diseases or vasculitis. Rheumatological manifestations are also described in such context. Herein, we report the observation of a patient with chronic myelomonocytic leukemia (CMML) who developed systemic manifestations: polymyalgia rheumatica and pericarditis. A 78-year-old patient was referred for the exploration of two months history of inflammatory shoulder pain associated with biological inflammatory syndrome. He presented with asthenia, anorexia and loss of 5 kg in one month. He had a three years follow-up for a CMML without any specific treatment. All of the explorations carried out showed a typical polymyalgia rheumatica. A pericardial effusion requiring emergency drainage was synchronously diagnosed. All the symptoms occurred during a worsening of his hematological disease. The rheumatological manifestation was favorable after a short corticosteroid therapy and pericarditis did not recur after 2 years of follow-up. It should be necessary to screen patients for MDS in a context of systemic manifestation, especially in elderly patients with an abnormal blood count (cytopenia, macrocytosis and monocytosis). [ABSTRACT FROM AUTHOR]

Aim: Acute graft-versus-host disease (GVHD) is an complication after allogeneic hematopoietic stem cell transplantation (alloHSCT), manifesting with an erythematous maculopapular rash followed by liver disease and dysfunctions of the gastrointestinal tract. This study aimed to retrospectively investigate the demographic and clinical characteristics of pediatric patients diagnosed with acute GVHD after undergoing allo-HSCT due to various hematological diseases. Material and Methods: The study included 33 pediatric patients that underwent their first allogeneic bone marrow transplantation in the Pediatric Bone Marrow Transplant Unit between 2015 and 2018 and were consecutively diagnosed with acute GVHD in consultation with the department of dermatology. The demographic characteristics, clinical findings, donor characteristics, treatments, and survival status of each patient were recorded. Results: Between 2015 and 2018, bone marrow transplantation was performed in 150 pediatric patients, of whom 33 (22%) developed acute GVHD. Of the 33 pediatric patients, 19 (57.6%) were boys and 14 (42.4%) were girls. At the time of transplantation, the mean age of the patients was 8.5 years, and the median age was 7 years. Twenty patients (60.6%) had only skin GVHD while 13 (39.4%) had skin and gastrointestinal and/or hepatic GVHD. It was found that the rashes had appeared on average 27.4 days after allo-HSCT. Palmoplantar involvement was detected in 13 patients (39.4%), painful erosion in the oral mucosa in 11 (33%), and conjunctivitis in seven (21.2%). The factors of being a male patient, being a female donor, transplantation from unrelated donors, liver involvement, and palmoplantar involvement were found to create risk of mortality, but not significantly (respectively p=0,753;p=0,145,0,66). Five patients (15%) progressed from acute to chronic GVHD. In patients that progressed from acute to chronic had, the mean donor age was higher, the mean time from transplantation to rash was longer, and oral involvement was higher, but not significantly (respectively; p=0,596;p=0,980;p=0,193. Conclusion: In acute GVHD, the skin is the first and most involved organ; therefore, dermatologists play an important role in its diagnosis. This study is important for determining the factors affecting the decrease in progression to chronic GVHD and prolonging survival. [ABSTRACT FROM AUTHOR]

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Background and aims: The predictive value of traditional risk factors for vascular events in patients with manifest vascular disease is limited, underscoring the need for novel biomarkers to improve risk stratification. Since hematological parameters are routinely assessed in clinical practice, they are readily available candidates. Methods: We used data from 3,922 vascular patients, who participated in the Second Manifestations of ARTerial Disease (SMART) study. We first investigated associations between recurrent vascular events and 22 hematological parameters, obtained from the Utrecht Patient Oriented Database (UPOD), and then assessed whether parameters associated with outcome improved risk prediction. Results: After adjustment for all SMART risk score (SRS) variables, lymphocyte %, neutrophil count, neutrophil % and red cell distribution width (RDW) were significantly associated with vascular events. When individually added to the SRS, lymphocyte % improved prediction of recurrent vascular events with a continuous net reclassification improvement (cNRI) of 17.4% [95% CI: 2.1, 32.1%] and an increase in c-statistic of 0.011 [0.000, 0.022]. The combination of lymphocyte % and neutrophil count resulted in a cNRI of 22.2% [3.2, 33.4%] and improved c-statistic by 0.011 [95% CI: 0.000, 0.022]. Lymphocyte % and RDW yielded a cNRI of 18.7% [3.3, 31.9%] and improved c-statistic by 0.016 [0.004, 0.028]. However, the addition of hematological parameters only modestly increased risk estimates for patients with an event during follow-up. Conclusions: Several hematological parameters were independently associated with recurrent vascular events. Lymphocyte % alone and in combination with other parameters enhanced discrimination and reclassification. However, the incremental value for patients with a recurrent event was limited. [ABSTRACT FROM AUTHOR]

Background: Cancer risk is associated with serum iron levels. The aim of this study was to evaluate whether haematological parameters reflect serum iron levels and may also be associated with cancer risk.Methods: We studied 1564 men and 1769 women who were enrolled in the Busselton Health Study, Western Australia. Haematological parameters evaluated included haemoglobin (Hb), mean cell volume (MCV), mean cell haemoglobin (MCH) and mean cell haemoglobin concentration (MCHC) and red cell distribution width (RCDW). Statistical analyses included t-tests for quantitative variables, chi-square tests for categorical variables and Cox proportional hazards regression modelling for cancer incidence and death.Results: There was marginal evidence of an association between MCV (as a continuous variable) and non-skin cancer incidence in women (HR 1.15, 95% CI 1.013, 1.302; p = 0.030) but the hazard ratio was attenuated to non-significance after adjustment for serum ferritin (SF), iron and transferrin saturation (TS) (HR 1.11, 95% CI 0.972, 1.264; p = 0.126). There was strong evidence of an association between MCHC and prostate cancer incidence in men; the estimated hazard ratio for an increase of one SD (0.5) in MCHC was 1.27 (95% CI 1.064, 1.507; p = 0.008). These results remained significant after further adjustment for SF and iron; the estimated hazard ratio for an increase of one SD (0.5) in MCHC was 1.25 (p = 0.014, 95% CI 1.05 to 1.48).Conclusions: The MCHC and MCV were associated with cancer incidence in a Western Australian population, although only MCHC remained associated with prostate cancer after adjusting with serum iron and TS (circulating iron) and SF (storage iron). Haematological parameters are thus of limited utility in population profiling for future cancer risk. [ABSTRACT FROM AUTHOR]

Introduction Bone marrow aspiration (BMA) and bone marrow biopsy (BMB) is an indispensable diagnostic tool for evaluating haematological and non-haematological disorders and patient follow-up in present era. We have compared the advantages of trephine biopsy over bone marrow aspiration in these patients. Aim and objective To evaluate sensitivity and specificity of trephine biopsy test for haematological and non haematological disorder patients in comparison to bone marrow aspiration test. Materials and method In this 1 year prospective study (June 2014–May 2015), we evaluated the haematological and non-haematological disorder patients by BMA and BMB (aided with I.H.C. when ever needed). The sensitivity and specificity of the tests were calculated. Results Among, final 504 hemotological/non haematological disorder patients, 416 cases were diagnosed (+ve) in BMA test, where as it was 494 in BMB test and with chi 2 test it was highly significant as p = 0.0001. It was concluded that True positive cases were 416, True negative were 9 cases, false negative 78 cases and false positive was in one case only. The sensitivity and specificity of bone marrow trephine biopsy test was 84% and 90% respectively. Conclusion BMB (aided with I.H.C) is a gold standard test for detecting different haematological and non hamatological disorders. In our study the sensitivity and specificity of BMB test was 84% and 90% respectively. When performed in association with BMA in the same sitting, significantly augments the chances of reaching a correct diagnosis. [ABSTRACT FROM AUTHOR]

The article presents a study on long-term outcomes for patients with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes (POEMS) syndrome. It discusses the characterization of POEMS syndrome, the hematologic response rate and neurologic evaluations conducted in the patients.

Single-cell microfluidic devices are poised to substantially impact the hematology field by providing a high-throughput and rapid device to analyze disease-mediated biophysical cellular changes in the clinical setting in order to diagnose patients and monitor disease prognosis. In this Feature, we cover recent advances of single-cell microfluidic devices for studying and diagnosing hematological dysfunctions and the clinical impact made possible by these advances. [ABSTRACT FROM AUTHOR]

Equine piroplasmosis in donkeys has been recognised as a serious problem of major economic importance. The present molecular study is the first investigation of the presence of Theileria equi and Babesia caballi in Balkan donkeys and of the possible haematological alterations related to it. A total of 70 apparently healthy donkeys from Serbia were included in this study. The overall prevalence of T. equi infection in donkeys tested with multiplex PCR was 50%. There was no B. caballi-positive sample. Infections in donkeys included in this study seem to be associated with decreased red blood cell count, haemoglobin concentration, haematocrit and platelet count, and with increased white blood cell count, mean corpuscular haemoglobin and mean corpuscular haemoglobin concentration. Altered haematological parameters in donkeys can lead to a decrease in working capacity and production performance. Further molecular research and long-term monitoring of equine piroplasmosis is needed in Serbia and throughout Europe. [ABSTRACT FROM AUTHOR]

Drug rash, eosinophilia, and systemic symptoms ( DRESS) syndrome is a severe systemic hypersensitivity reaction that usually occurs within 6 weeks of exposure to the offending drug. Diagnosis is usually straightforward in patients with pyrexia, skin rash, hepatitis, and eosinophilia with a preceding history of exposure to agents often associated with DRESS syndrome, such as aromatic anticonvulsants and sulfa drugs, but diagnosis of DRESS may still be a challenge. We report a 4-year-old child with probable DRESS syndrome complicated by multiple hematologic complications that developed 1 month after exposure to fluoxetine, a drug not known to be associated with such severe reactions. [ABSTRACT FROM AUTHOR]

Juvenile systemic lupus erythematosus (JSLE) is a complex multisystemic autoimmune disorder of unknown cause. It accounts for about one in five cases of SLE. The tendency for SLE to run a fulminant course when it starts in childhood has made JSLE a potentially more severe disease than adult SLE. Reports of JSLE from sub-Saharan Africa are scanty in spite of the increasing reports of adult SLE. We conducted a 4-year retrospective study of JSLE cases seen at the Lagos State University Teaching Hospital between January 2010 and December 2014. Out of the 12 patients studied, eight were girls and four were boys. All patients had positive antinuclear antibody and extractable nuclear antibody tests. Anti-dsDNA antibody was positive in 10 patients. Eight patients had renal disease while four patients had neuropsychiatric manifestations. Haematological abnormalities and constitutional symptoms were present in all patients. Patients were treated with pulse methylprednisolone, oral prednisolone, hydroxychloroquine and azathioprine. Three patients also received rituximab. In conclusion, JSLE exists in Nigeria and exhibits clinical and immunological characteristics similar to its pattern in other parts of the world. It is, however, diagnosed late and is possibly being underdiagnosed as there is no paediatric rheumatologist in the country. [ABSTRACT FROM AUTHOR]

Coping with cancer can potentially modify couples’ quality of life (QoL). The predictive impact of dyadic coping (DC) on QoL was investigated within patients with hematological cancer and their partners. Data were collected in a multi-centre longitudinal study (N = 208 couples). We use the SF-12 questionnaire (QoL) and the Dyadic Coping Inventory. Data were mainly analyzed with actor–partner interdependence models (APIM). Within the APIM, baseline QoL was the most significant predictor for QoL (t2). Regarding DC, there were partner effects on physical QoL (t2) and actor and partner effects on mental QoL at t2. Different subtypes of DC have intra- and/or interpersonal impact on patient’s or partner’s QoL. In a clinical setting, patients as well as partners should be seen as both a source of support for each other and as individuals in need of support themselves. Such awareness and targeted intervention may help couples adapt to and cope with cancer. [ABSTRACT FROM PUBLISHER]

Hematological abnormalities, especially thrombocytopenia (TCP), are highly prevalent among patients with systemic lupus erythematosus (SLE) and at the same time it has been reported as a significant prognostic factor of SLE course. We further investigate the correlation between platelet count and the clinical manifestations and disease activity of SLE, in a cohort of Saudi Arabian female patients. A retrospective analysis was done for the medical records of 100 SLE female patients, selected from all patients diagnosed and treated for SLE at the Rheumatology outpatient clinics in Hera’a General Hospital, Holly Makkah, Saudi Arabia. The data collected from every patient’s file included laboratory investigations (complete blood count, platelet parameters, ESR, anti-double-stranded DNA antibody, ANA), clinical manifestations, as well as SLE disease activity index (SLEDAI-2k) scores throughout a period of six sequential follow-up visits. Patients were divided into three groups according to the SLEDAI-2k: mild, moderate, and high-activity group. We found that, out of 100 patients, TCP was the most prevalent hematological abnormality evident in 15%, more than leucopenia (14%) and anemia (2%). TCP was acute in onset and associated with arthritis, neurologic manifestations, and nephritis. Platelet count showed a significant negative correlation with disease activity, in all of the three groups of patients. We concluded that platelet count has a negative correlation with disease activity in SLE patients, whatever the associated manifestations, and it should be considered as a prognostic factor, identifying patients with aggressive disease course. [ABSTRACT FROM PUBLISHER]

Background: Babesiosis, a zoonotic parasitic infection transmitted by the Ixodes tick, has become an emerging health problem in humans that is attracting attention worldwide. Most cases of human babesiosis are reported in the United States and Europe. The disease is caused by the protozoa of the genus Babesia, which invade human erythrocytes and lyse them causing a febrile hemolytic anemia. The infection is usually asymptomatic or self-limited in the immunocompetent host, or follows a persistent, relapsing, and/or life threatening course with multi-organ failure, mainly in the splenectomized or immunosuppressed patients. Hematologic manifestations of the disease are common. They can range from mild anemia, to severe pancytopenia, splenic rupture, disseminated intravascular coagulopathy (DIC), or even hemophagocytic lymphohistiocytosis (HLH). Case presentation: A 70 year old immunocompetent female patient living in New York City presented with a persistent fever, night sweats, and fatigue of 5 days duration. Full evaluation showed a febrile hemolytic anemia along with neutropenia and thrombocytopenia. Blood smear revealed intraerythrocytic Babesia, which was confirmed by PCR. Bone marrow biopsy was remarkable for dyserythropoiesis, suggesting possible HLH, supported by other blood workup meeting HLH-2004 trial criteria. Conclusion: Human babesiosis is an increasing healthcare problem in the United States that is being diagnosed more often nowadays. We presented a case of HLH triggered by Babesia microti that was treated successfully. Also, we presented the hematologic manifestations of this disease along with their pathophysiologies. [ABSTRACT FROM AUTHOR]

Objective The objective of this study was to conduct a systematic review of case reports documenting the development of antiphospholipid syndrome or antiphospholipid syndrome-related features after an infection. Methods We searched Medline, EMBASE, Web of Science, PubMed ePubs, and The Cochrane Library – CENTRAL through March 2015 without restrictions. Studies reporting cases of antiphospholipid syndrome or antiphospholipid syndrome-related features following an infection were included. Results Two hundred and fifty-nine publications met inclusion criteria, reporting on 293 cases. Three different groups of patients were identified; group 1 included patients who fulfilled the criteria for definitive antiphospholipid syndrome (24.6%), group 2 included patients who developed transient antiphospholipid antibodies with thromboembolic phenomena (43.7%), and group 3 included patients who developed transient antiphospholipid antibodies without thromboembolic events (31.7%). The most common preceding infection was viral (55.6%). In cases that developed thromboembolic events Human immunodeficiency and Hepatitis C viruses were the most frequently reported. Parvovirus B19 was the most common in cases that developed antibodies without thromboembolic events. Hematological manifestations and peripheral thrombosis were the most common clinical manifestations. Positive anticardiolipin antibodies were the most frequent antibodies reported, primarily coexisting IgG and IgM isotypes. Few patients in groups 1 and 2 had persistent antiphospholipid antibodies for more than 6 months. Outcome was variable with some cases reporting persistent antiphospholipid syndrome features and others achieving complete resolution of clinical events. Conclusions Development of antiphospholipid antibodies with all traditional manifestations of antiphospholipid syndrome were observed after variety of infections, most frequently after chronic viral infections with Human immunodeficiency and Hepatitis C. The causal relationship between infection and antiphospholipid syndrome cannot be established, but the possible contribution of various infections in the pathogenesis of antiphospholipid syndrome need further longitudinal and controlled studies to establish the incidence, and better quantify the risk and the outcomes of antiphospholipid-related events after infection. [ABSTRACT FROM AUTHOR]

Background: Scarce studies have addressed hematological differences of malaria in urban and rural regions.Methods: Full or complete blood cell counts from 46 and 75 individuals (age range from < 1 to 92 years) with uncomplicated malaria infection living in urban (Accra) and rural (Dodowa) Ghana, respectively, were assessed. Sickle cell trait and patients were excluded from the study.Results: Between overall groups, patients from Accra had significantly lower parasite count (p < 0.0001) and granulocyte number (p = 0.026). Children in Accra had a significantly lower parasitemia (p = 0.0013), hemoglobin (p = 0.0254), platelet count (p = 0.0148) and red blood cell levels (p = 0.0080) when compared with the children of Dodowa. In adults, mean cell hemoglobin (p = 0.0086) and parasite count (p < 0.0001) were significantly higher in Dodowa.Conclusion: These results indicate that children living in urban setting may experience a greater anemic effect to malaria as compared with those living in a rural setting. [ABSTRACT FROM AUTHOR]

Objectives Multiple myeloma ( MM) is an incurable haematological disease. Due to novel agents, overall survival has improved in this group, yet there are no systematic reviews to understand the symptom profiles resulting from disease and treatment-related toxicities. We aimed to synthesise data on the prevalence of symptoms in patients with MM. Methods A systematic database and grey literature search were conducted in six databases. Random-effects meta-analysis with inverse variance weighting to pool prevalence data was performed. Results Thirty-six studies were included of which 34 studies ( N = 3023) provided data for meta-analysis. Twenty-seven distinct symptoms were reported, with the majority of studies focusing on pain ( n = 27), fatigue ( n = 19) and problems with functioning ( n = 15). The most prevalent symptoms were fatigue (98.8%, 95% CI 98.1-99.2%), pain (73%, 39.9-91.7), constipation (65.2%, 22.9-92.2) and tingling in the hands/feet with 53.4% (0.4-99.7). The most common problems were decreased physical functioning (98.9%, 98.2-99.3), decreased cognitive functioning (80.2%, 40-96.1) and financial difficulties (78.4%, 39.1-95.4). These problems were present in newly diagnosed to advanced disease stage. Conclusions Optimal quality of life and good symptom management in this incurable disease can only be achieved by routinely assessing symptoms throughout the disease trajectory. [ABSTRACT FROM AUTHOR]

Healthy red blood cells (RBCs) have remarkable deformability, squeezing through narrow capillaries as small as 3 microns in diameter without any damage. However, in many hematological disorders the spectrin network and lipid bilayer of diseased RBCs may be significantly altered, leading to impaired functionality including loss of deformability. We employ a two-component whole-cell multiscale model to quantify the biomechanical characteristics of the healthy and diseased RBCs, including Plasmodium falciparum-infected RBCs (Pf- RBCs) and defective RBCs in hereditary disorders, such as spherocytosis and elliptocytosis. In particular, we develop a two-step multiscale framework based on coarse-grained molecular dynamics (CGMD) and dissipative particle dynamics (DPD) to predict the static and dynamic responses of RBCs subject to tensile forcing, using experimental information only on the structural defects in the lipid bilayer, cytoskeleton, and their interaction. We first employ CGMD on a small RBC patch to compute the shear modulus, bending stiffness, and network parameters, which are subsequently used as input to a whole-cell DPD model to predict the RBC shape and corresponding stress field. For Pf-RBCs at trophozoite and schizont stages, the presence of cytoadherent knobs elevates the shear response in the lipid bilayer and stiffens the RBC membrane. For RBCs in spherocytosis and elliptocytosis, the bilayer-cytoskeleton interaction is weakened, resulting in substantial increase of the tensile stress in the lipid bilayer. Furthermore, we investigate the transient behavior of stretching deformation and shape relaxation of the normal and defective RBCs. Different from the normal RBCs possessing high elasticity, our simulations reveal that the defective RBCs respond irreversibly, i.e., they lose their ability to recover the normal biconcave shape in successive loading cycles of stretching and relaxation. Our findings provide fundamental insights into the microstructure and biomechanics of RBCs, and demonstrate that the two-step multiscale framework presented here can be used effectively for in silico studies of hematological disorders based on first principles and patient-specific experimental input at the protein level. [ABSTRACT FROM AUTHOR]

Impurity has been suggested as an important factor determining toxicity following exposure to single-walled carbon nanotubes (SWCNTs). In this study, we first compared immunotoxicity based on iron content on day 90 after a single intratracheal instillation of SWCNTs in male and female mice. The inflammatory responses were generally stronger in mice exposed to acid-purified (P)-SWCNTs compared to raw (R)-SWCNTs. In addition, both R- and P-SWCNTs induced Th1-polarized immune responses with apoptotic death of BAL cells and systemically impaired the function of antigen-presenting cells (APC). We also screened reproductive and developmental toxicity by cohabitating male and female mice on day 14 after instillation. Interestingly, the pregnancy rate rapidly decreased following exposure to both types of SWCNTs, especially R-SWCNTs. In addition, we investigated developmental immunotoxicity of the offspring on day 28 after exposure to both types of SWCNTs. Their hematological changes were clearer relative to those of the parents and a significant decrease in the alkaline phosphatase and potassium levels was observed in mice of both sexes exposed to the higher dose of R- and P-SWCNTs. In conclusion, we suggest that SWCNTs may induce Th1-polarized immune responses accompanied by suppression of APC function on day 90 after a single instillation without significant iron content dependance. In addition, the consecutive exposure of SWCNTs to the subsequent generation may exacerbate metabolic and hematological disturbance. Furthermore, our results underscore the need to clarify the reproductive and developmental health effects of SWCNTs. [ABSTRACT FROM PUBLISHER]

Objectives: Methotrexate (MTX) is the most commonly used cost‑effective disease‑modifying antirheumatoid drug (DMARD). Its main dose‑limiting adverse effects are hepatic and hematopoietic. This cross‑sectional, observational study evaluated the prevalence of hepatic and hematological adverse effects with long‑term low‑dose MTX therapy. Materials and Methods: Rheumatoid arthritis (RA) patients taking ≤15 mg/week MTX for at least 2 years were enrolled from the rheumatology outpatient department. Demographic, disease, drug treatment profiles, and hematological and hepatic enzyme levels were noted. Results: Of the 204 patients enrolled, the frequency of raised alanine transaminase level (≥3‑fold rise above the upper limit of normal) was 6.37% (95% confidence interval of 3.76–10.59) including two biopsy‑proven hepatic fibrosis cases. About 5.4% had severe anemia (<8 g/dl) and 4.4% had leukopenia. Conclusion: Long‑term low‑dose MTX is safe in RA patients in the Indian population. The patterns of adverse effects were similar to those documented in earlier studies. However, our study results suggest that disease duration, cumulative MTX dose, concomitant DMARD intake are not risk factors associated with hepatic or hematological adverse effects. [ABSTRACT FROM AUTHOR]

Objectives: To study the ocular manifestations in patients diagnosed with leukemia or myeloproliferative disorders and to derive an association between the ocular manifestations and hematological parameters of the patients. Materials and Methods: This observational and descriptive study was conducted in a Tertiary Care Center of Dehradun, North India over a period of 1 year. All the diagnosed cases of leukemia and myeloproliferative diseases attending the Oncology Department were included. Patients underwent a detailed ocular examination. The hematological parameters were also recorded including hemoglobin (Hb), total leukocyte count (TLC), and platelet count. Results: The study included 102 patients out of which, there were 66 males (64.7%), and 36 were females (35.3%) with an age range of 5-80 years. A total of forty patients showed ocular manifestations (39.3%). Extraocular manifestations were seen in 10.8% patients while intraocular manifestations were noted in 45.1% patients of acute leukemia and 7.7% patients of chronic leukemia. These ocular manifestations showed a significant association with low Hb (P = 0.000), raised TLC (P = 0.004), and low platelet count (P = 0.000). The most common ocular manifestation was intraretinal hemorrhages followed by roth spots. Among acute myeloid leukemia patients (23.5%), the presence of intraretinal hemorrhages was significantly associated with low Hb (Mann-Whitney U-test = 25.000, P = 0.007). The patients of acute lymphoblastic leukemia (25.4%) showed significant association between intraretinal hemorrhages and low platelet count (Mann-Whitney U-test = 44.000, P = 0.046) and roth spots with low Hb (Mann-Whitney U-test = 11.000, P = 0.000). Ocular manifestations were more commonly seen in acute leukemias as compared to chronic leukemias. Conclusion: Ocular examination is recommended in all leukemic patients as ocular manifestations may go unnoticed, most patients being asymptomatic and could be an early sign of worsening of disease. [ABSTRACT FROM AUTHOR]

A high-flow nasal cannula (HFNC) is a newly developed device that enables high-flow oxygen therapy for patients with serious cardiopulmonary problems, but there are few data regarding its use in patients with hematological disease. The efficacy and tolerability of HFNCs for patients who developed ARF during the treatment of various hematological diseases was evaluated. Fifty-six patients underwent HFNC therapy during the last 2 years, and the causes of ARF were mainly pneumonia (n = 37) or acute congestive heart failure (n = 7). Only 11 patients (20 %) showed a good response to HFNC therapy, and remaining 45 patients (80 %) failed to respond to the initial HFNC therapy and, therefore, underwent second-line therapy including endotracheal intubation with mechanical ventilation (n = 15), non-invasive positive pressure ventilation (n = 1), or narcotic palliation alone (n = 29). Thus, HFNC appear not to be a viable treatment option in 4 out of 5 patients in this cohort of patients with hematological disease, but it was well tolerated in most patients (96 %); no major complications except for nasal soreness (n = 2) were observed. Multivariate analysis showed that the cause of ARF (pneumonia, odds ratio 11.2, 95 % CI 1.76-71.5, p = 0.01) was the only risk factor for treatment failure. [ABSTRACT FROM AUTHOR]

Background. Anemia is a wide spread public health problem in India which affects children. The present study evaluates the prevalence of anemia and status of various hematological parameters among children of Khurda district, Odisha. Method. A total of 313 children aged 0–12 years were enrolled for the study which included preschool (0–5 years) and school aged (6–12 years) groups. Hematological indicators were measured by standard procedures, which include red blood cell (RBC) indicators, white blood cell (WBC) indicators, and plasma ferritin. Results. Mean hemoglobin (Hb) of the study population was 10.43±3.33 g/dL and prevalence of anemia was 62%. In this population, boys had a lower mean Hb value than that of the girls. All grades of anemia were higher among school age children than preschool children. Mean plasma ferritin was found to be higher in school age boys than their counterpart girls. The mean level of WBC count was found to be higher among preschool age boys than among the school age boys (p=0.025). Conclusion. The prevalence of anemia was higher with concomitant acute infection among study population, which is a matter of concern. Since the hematological parameters are interrelated with each other as well as with the age and gender, relevant intervention strategy and constant monitoring are needed while providing public health nutrition programs to eradicate anemia. [ABSTRACT FROM AUTHOR]

The only curative therapy for primary myelofibrosis (PMF) is allogeneic stem cell transplantation (ASCT). However, although we know that patients can benefit from ASCT, we do not know the extent of the changes of the expression profile of cytokines and matrix modulation factors. In this first systematic analysis, we evaluated the expression profile of 103 factors before and after transplantation to identify potential biomarkers. The expression of fibrosis-, inflammation-, and angiogenesis-associated genes was analyzed in a total of 52 bone marrow biopsies: PMF patients (n = 14) before and after ASCT and, for control purposes, post-ASCT multiple myeloma patients (n = 14) and non-neoplastic hematopoiesis (n = 10). In post-ASCT PMF cases, decreased expression of tissue inhibitor of metalloproteinases (TIMP) and platelet-derived growth factor alpha (PDGFA) correlated with bone marrow remodeling and hematological remission. Expression of several other matrix factors remained at high levels and may contribute to post-ASCT remodeling. This is the first systematic analysis of cytokine expression in post-ASCT PMF bone marrow that shows that normalization of bone marrow microenvironment is paralleled by decreased expression of TIMP and PDGFA. [ABSTRACT FROM AUTHOR]

The present study was undertaken to evaluate the leaf extracts of Carica papaya (Linn.) on hematologic and biochemical changes occurring due to irradiation of mice to sub-lethal doses of Electron Beam Radiation (EBR). The immunomodulatory response of C. papaya (Linn.) extract to the radiological effects by determining the levels of interleukin-6. The serum total antioxidants, lipid peroxidation and the biochemical changes in liver and kidney function were assessed. Reduction in the hematological parameters like hemoglobin, RBC, WBC and platelets were seen in the irradiated groups compared to the control group. The elevated serum malondialdehyde levels indicate increased lipid peroxidation and reduced glutathione levels and total antioxidant capacity suggest prominent oxidative stress in the irradiated groups. C. papaya (Linn.) (pre-treatment + radiation) treated groups have shown significant improvements in the hematological parameters. Reduction in the malondialdehyde levels and increased reduced glutathione levels indicate amelioration of the oxidative stress. There is decrease in the cytogenetic damages in the C.papaya (Linn.) (pre-treatment + radiation) treated groups compared to the radiation control. Significant changes were seen in the interleukin-6 levels of C. papaya (Linn.) (pre-treatment + radiation) treated groups indicate enhanced immune response compared to the irradiation control groups. The results indicate that the C. papaya (Linn.) aqueous extracts play a protective role in radiation induced hematological and antioxidant suppression. Also it enhances membrane stabilization and reduces cytogenetic damages. [ABSTRACT FROM AUTHOR]

Several clinical and biological features of lymphoproliferative diseases have been associated with an increased risk of developing autoimmune manifestations. Acquired deficiency of C1-inhibitor (C1-INH) (AAE) is a rare syndrome clinically similar to hereditary angioedema (HAE) characterized by local increase in vascular permeability (angioedema) of the skin and the gastrointestinal and oro-pharyngo-laryngeal mucosa. Bradykinin, a potent vasoactive peptide, released from high molecular weight kininogen when it is cleaved by plasma kallikrein (a serine protease controlled by C1-INH), is the mediator of symptoms. In total 46% of AAE patients carry an underlying hematological disorder including monoclonal gammopathy of uncertain significance (MGUS) or B cell malignancies. However, 74% of AAE patients have anti-C1-INH autoantibodies without hematological, clinical or instrumental evidence of lymphoproliferative disease. Unlike HAE patients, AAE patients usually have late-onset symptoms, do not have a family history of angioedema and present variable response to treatment due to the hypercatabolism of C1-INH. Experiments show that C1-INH and/or the classical complement pathway were consumed by the neoplastic lymphatic tissues and/or anti-C1-INH neutralizing autoantibodies. Therapy of AAE follows two directions: 1) prevention/reversal of the symptoms of angioedema; and 2) treatment of the associated disease. Different forms of B cell disorders coexist and/or evolve into each other in AAE and seem to be dominated by an altered control of B cell proliferation, thus AAE represents an example of the strict link between autoimmunity and lymphoproliferation. [ABSTRACT FROM AUTHOR]

Early in the human immunodeficiency virus (HIV) epidemic, infected patients presented to medical attention with striking abnormalities in each of the major blood cell lineages. The reasons for these derangements remain complex and multifactorial. HIV infects multipotent haematopoietic progenitor cells and establish latent cellular reservoirs, disturbs the bone marrow microenvironment and also causes immune dysregulation. These events lead to cytokine imbalances and disruption of other factors required for normal haematopoiesis. Activation of the reticulo-endothelial system can also result in increased blood cell destruction. The deleterious effects of medications, including first and second generation anti-retroviral agents, on haematopoiesis were well documented in the early years of HIV care; in the current era of HIV-care, the advent of newer and less toxic anti-retroviral drugs have had a more beneficial impact on haematopoiesis. Due to impaired regulation of the immune system and potential side effects of one or more anti-retroviral agents, there is also an increase in coagulation abnormalities such as thromboembolism, and less frequently, acquired disorders of coagulation including thrombotic thrombocytopenic purpura, immune thrombocytopenic purpura and acquired inhibitors of coagulation. In this article we review the epidemiology and aetiology of select non-oncological haematological disorders commonly seen in people living with HIV-acquired immune deficiency syndrome. [ABSTRACT FROM AUTHOR]

Systemic lupus erythematosus is characterized by the presence of circulating anti-nuclear antibodies (ANA) and systemic damage that includes nephritis, haematological manifestations and pulmonary compromise, among others. Although major progress has been made in elucidating the molecular mechanisms responsible for autoimmunity, current therapies for lupus have not improved considerably. Because the exposure of carbon monoxide (CO) has been shown to display beneficial immunoregulatory properties in different immune-mediated diseases, we investigated whether CO therapy improves lupus-related kidney injury in lupus mice. MRL-Fas lpr lupus mice were exposed to CO and disease progression was evaluated. ANA, leucocyte-infiltrating populations in spleen, kidney and lung and kidney lesions, were measured. CO therapy significantly decreased the frequency of activated B220+ CD4− CD8− T cells in kidneys and lungs, as well as serum levels of ANA. Furthermore, we observed that CO therapy reduced kidney injury by decreasing proliferative glomerular damage and immune complexes deposition, decreased proinflammatory cytokine production and finally delayed the impairment of kidney function. CO exposure ameliorates kidney and lung leucocyte infiltration and delays kidney disease in MRL-Fas lpr lupus mice. Our data support the notion that CO could be explored as a potential new therapy for lupus nephritis. [ABSTRACT FROM AUTHOR]

Hemoglobin (Hb) H disease is a moderate form of α- thalassemia resulting from various genetic defects. HbH disease is not necessarily a benign disorder as has been generally thought. We present hereby a 25- year- old Iranian pregnant woman whom referred to our hospital for blood transfusion. She exhibited the clinical and hematological manifestation of HbH disease. Her father carries a common α-thalassemia deletion while her mother was normal in the hematological profile. The object of this report was to provide information about pregnancies affected by HbH disease. [ABSTRACT FROM AUTHOR]

Multisystem deterioration occurs mainly in older individuals and may be related to physiological tissue degeneration. However, genetic predisposition may be unmasked by inappropriate functional and structural system deficiencies. McLeod syndrome (MLS) is a rare, multisystem disease which is X-chromosomal inherited and belongs to the neuroacanthocytosis syndromes (NAS). The main clinical manifestations contain progressive neuro-psychiatric and cognitive deterioration, choreatic movement disorder, as well as myopathy, sensory motor axonal neuropathy and cardiomyopathy. In addition, MLS patients have red blood cell abnormalities including immune-hematological, morphological and functional impairments of red blood cells. In large deletions, contiguous gene syndrome may arise, including Duchenne muscular dystrophia, cellular immunodeficiency or retinitis pigmentosa. Hematological abnormalities such as blood group abnormalities in Kell- and XK blood group system, formation of anti-public red blood cell alloantibodies, acanthocytosis and elevated creatinine phosphokinase may precede clinical disease manifestation for decades and provide tools for early diagnosis. Patients with unexplained neuro-muscular deterioration and/or neuro-psychological pathologies accompanied with hematological abnormalities should be investigated for MLS. [ABSTRACT FROM AUTHOR]

Background/aim: To determine the hematologic manifestations at the time of diagnosis of celiac disease in children and the effects of a gluten-free diet on hematologic signs upon follow-up. Materials and methods: The records of patients with celiac disease who received a follow up examination at the Pediatric Gastroenterology Clinic between June 2006 and June 2013 were retrospectively examined. Results: Ninety-one patients were included in the study. The mean age at diagnosis was 8.1 ± 4.21 years and 59 patients (64.8%) were female. Thirty-two patients (35.2%) had hematologic signs at the time of diagnosis. Anemia (24.2%) was the most common hematologic sign, followed by thrombocytosis (16.5%) and leukopenia (4.4%). The tTG IgA titers were screened in 80 of the 91 patients during diagnosis. Follow-up examinations found that remission for anemia (P = 0.017), thrombocytosis (P = 0.039), and decreases in tTG IgA titers (P = 0.034) were more prominent in patients who had followed a strict gluten-free diet. Conclusion: Approximately one-third of the celiac disease patients had hematologic manifestations at the time of diagnosis. Remission in hematologic signs and decrease in tTG IgA titers were more prominent in patients who had adhered to a gluten-free diet. [ABSTRACT FROM AUTHOR]

The aim of this study was to investigate the frequency of hematological and hepatic alterations and possible association with serum levels of beta-hexachlorocyclohexane (beta-HCH),p,p′-DDE, and hexachlorobenzene (HCB) among residents in an area heavily contaminated with organochlorine (OC) pesticides. A cross-sectional study was conducted in 415 male and 432 female residents aged >14 years. Serum samples were collected and analyzed for OC pesticides concentrations and biochemical parameters. Frequencies of hematological and hepatic alterations were calculated for each gender. Association between beta-HCH,p,p′-DDE (1,1-dichloro-2,2-bis(p-chlorophenyl) ethylene), and HCB levels and presence of alterations was determined by logistic regression stratified by gender and controlling for confounders. Highest frequencies were observed for eosinophilia (23% men and 18% women), low hemoglobin (12% men and 15% women), and low erythrocyte count (12% men). High levels of bilirubin, glutamic-oxaloacetic transaminase (GOT), and glutamic-pyruvic transaminase (GPT) were observed, respectively, in 10, 11, and 12% of men and <10% of women. Gamma-glutamyl transferase (GGT) was elevated in 26 and 25% of males and females, respectively. Multivariate analysis revealed associations between eosinophilia and beta-HCH in men (OR = 1.06, 95%CI = 1.01–1.12) and women (OR = 1.05, 96%CI = 0.99–1.11),p,p′-DDE in men (OR = 1.03, 95%CI = 0.99–1.06) and women (OR = 1.02, 95%CI = 0.99–1.06), and HCB in women (OR = 1.54, 95%IC = 0.85–4.45). Beta-HCH was found to be associated with increased risk of elevated bilirubin in females (OR = 1.18, 95%CI = 1.07–1.29) and males (OR = 4.21, 95%CI = 1.87–9.47 for fourth vs. first quintile). Thus, OC pesticides may exert adverse effects on hematopoietic tissue and liver in populations chronically exposed to high levels of these compounds. [ABSTRACT FROM PUBLISHER]

The article offers information on the essential thrombocythemia (ET). Topics discussed include background information about ET including its characteristics and the prevalence of the disease, provides details on a research study conducted to determine the hematological and clinical differences between Janus kinase 2 and calreticulin mutations

Extracellular vesicles (EVs), comprised of exosomes, microparticles, apoptotic bodies, and other microvesicles, are shed from a variety of cells upon cell activation or apoptosis. EVs promote clot formation, mediate pro-inflammatory processes, transfer proteins and miRNA to cells, and induce cell signaling that regulates cell differentiation, proliferation, migration, invasion, and apoptosis. This paper will review the contribution of EVs in hematological disorders, including hemoglobinopathies (sickle cell disease, thalassemia), paroxysmal nocturnal hemoglobinuria, and hematological malignancies (lymphomas, myelomas, and acute and chronic leukemias). [ABSTRACT FROM AUTHOR]

Nonadherence to medical recommendations is a widespread problem well documented in a multitude of clinical settings. Nonadherence may adversely affect clinical outcomes such as survival and quality of life and increase health-care-related costs. An understanding of the factors driving nonadherence is key to developing effective adherence-enhancing interventions (AEIs). There are ongoing attempts in contemporary adherence research to better define the various components of adherence, to find optimal measures of adherence and correlations with clinical outcomes, and to create a classification system for AEIs. Nonadherence is also widely prevalent among adolescents and young adults (AYAs) with chronic hematological diseases, affecting up to 50% of patients and increasing with age. Combined use of objective (i.e. electronic monitoring, EM) and subjective (i.e. self-report) measures of adherence may be the preferred approach to assess adherence. The unique physical, social and emotional aspects of the AYA life stage are closely related to intricate causes of nonadherence in AYAs such as problems in transition to adult care. Until proven otherwise, the empirical target in AYAs with hematological disorders should be perfect adherence. Multilevel AEIs, EM feedback and behavioral interventions are among the most effective types of AEIs. Despite the magnitude of the problem, only a handful of AEIs have been evaluated among AYAs with hematological disorders. Thus, this is a field with unmet needs warranting high-quality trials using standardized and well-specified assessment methods and interventions. This review discusses the prevalence, definition, causes and clinical implications of nonadherence among AYAs with hematological disorders, along with strategies to measure and improve adherence. © 2014 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Aim The interaction between inflammation and cancer is well established. Surrogate markers of systemic inflammation, such as the neutrophil/lymphocyte ratio ( NLR), may be associated with the long-term oncological outcome. The present study aimed to characterize the relationship between several ratios derived from haematological indices using a classification and regression tree analysis. Method Haematological white-cell ratios were established for all patients undergoing colonic cancer resection with curative intent ( n = 436) in a regional cancer centre. The optimal ratios associated with overall survival ( OS) were established in a training set ( n = 386) using a classification and regression tree ( CRT) technique. The association between ratios and OS was assessed in a separate test set ( n = 50). Within the test set, two groups were generated based on each ratio (one group above and one group below the cut-off value identified in the training set). The association between ratios and OS was assessed using a stepwise Cox proportional-hazards regression model. Results The following ratios, identified by the CRT, were associated with adverse OS in the test set: an NLR of ≥ 3.4 [hazard ratio ( HR) = 3.4, P < 0.001]; and a white-cell-count/lymphocyte ratio ( WLR) of ≥ 5.28 ( HR = 4.1, P = 0.03). Conclusion This is the first study to apply recursive partitioning in determining the relationship between haematological ratios and OS in colon cancer. Haematological ratios were predictive of oncological outcome. [ABSTRACT FROM AUTHOR]

"Spatial statistics" is an academic field that deals with the statistical analysis of spatial data, and has been applied to econometrics and various other policy fields. These methods are easily applied by hematologists and oncologists using better and much less expensive software. To encourage physicians to use these methods, this review introduces the methods and demonstrates the analyses using R and FleXScan, which can be freely downloaded from the website, with sample data. It is demonstrated that spatial analysis can be used by physicians to analyze hematological diseases. In addition, applying the technique presented to the investigation of patient prognoses may enable generation of data that are also useful for solving health policy-related problems, such as the optimal distribution of medical resources. [ABSTRACT FROM AUTHOR]

Aim: In this study, sub-acute toxicity of deltamethrin on hematological and biochemical blood parameters of male albino Swiss mice was evaluated. Materials and Methods: Generally, the maximum permissible residue level (MRL) of deltamethrin for food products lies between 0.01 to 0.5 mg/kg body weight. So the mice were exposed orally with two doses of pesticide i.e. 0.1 and 0.5 mg/kg body weight. The doses were given on a daily basis for a period of 15 days and 30 days respectively. Ground nut oil was used as control treatment. Samples of blood were collected at the end of the treatment. Hepatotoxicity was evaluated by quantitative analysis of the serum enzymes alanine transaminase (ALT), aspartate transaminase (AST), alkaline phosphatase (ALKP), total bilirubin (TBIL) and serum urea. Alterations of hematological parameters were analysed by total leukocyte, differential leukocyte count and hemoglobin levels. Results: Significant increase in the levels of hepatic enzymes (ALT, AST, ALKP) were observed for both doses, but no considerable differences were found by histological analysis. The hematological parameters showed significant alterations for 0.5 mg/kg body weight dose which is indicated by leukocytosis, lymphocytosis and neutropenia in long duration study. Conclusions: The results indicated that even very low dose of deltamethrin can promote hematological and hepatic alterations. Thus it is imperative to do further studies on the detrimental effect of the low levels of pyrethroid commonly present in our food, which further necessitate the reduction of maximum permissible levels of residual synthetic pyrethroid levels in foods and feed. [ABSTRACT FROM AUTHOR]

Routine assayed blood parameters are health indicators in elderly patients. We evaluated the presence of abnormalities in some hematologic and biochemical parameters determined in patients admitted to an adult nursing home, between monthes November-December 2011. Analysis were performed in 95 subjects, 37.9% women and 62.1% men, aged 50 to 103 years old; to determine routine hematological values, serum glucose, total cholesterol, triglycerides, creatinine, urea and uric acid. Other relevant collected data were age, sex and preexisting diseases. Results revealed that 38.9% had arterial hypertension, 15.8% dyslipidemia and 12.7% mental disorders; 8.45% presented with hypercholesterolemia, and 20% hypertriglyceridemia. Type 2 diabetes mellitus was found in 8.4% and out of them, 3.16% showed hyperglycemia; all diabetics were male. In addition, 67.37% suffer anemia; these blood analysis and biochemical assays help to identify the presence of non-communicable chronic diseases in this vulnerable population. [ABSTRACT FROM AUTHOR]

Purpose: Anemia is one of the most common hematological manifestations in SLE patients, occurring in about 50 % of active cases. STAT1 is a critical signaling molecule required for the production of type-1 interferon (I-IFN), CCL2, and CXCL10, all of which are upregulated in SLE. Overexpression of STAT1 has been described to be involved in anemia in animal models. The aim of this study is to analyze how these components are involved in SLE-associated anemia. Methods: Blood samples were collected from 39 healthy donors and 101 SLE patients fulfilling ACR criteria. Samples were collected from a total of 180 visits (58 patients had 2 or more visits) of which 52 visits included a diagnosis of anemia. Healthy donors had only single visit. Total RNA, isolated from leukocytes, was analyzed by Taqman qPCR. Relative expression levels of I-IFN signature genes, chemokines, and miR-146a were determined by the ΔΔCT method. Results were correlated with clinical data and analyzed by the Wilcoxon/Kruskal-Wallis test and Fisher's exact test. Results: Significant increases in IFN score ( p < 0.0001), STAT1 ( p < 0.0001), miR-146a ( p < 0.0005), CCL2 ( p = 0.0047), and CXCL10 ( p = 0.017), as well as a significant decrease in pri-miR-146a ( p = 0.0002), were detected in the anemic SLE patient visits ( n = 52) compared to non-anemic SLE visits ( n = 128). Regardless of disease activity, lupus nephritis, or race, anemic SLE patients displayed significantly elevated levels of STAT1 and miR-146a compared to non-anemic SLE patients. Conclusions: STAT1 and miR-146a may be upregulated during inflammation and via proinflammatory cytokines and chemokines in SLE. Prolonged upregulation of STAT1 and miR-146a appears to play an important role in anemia in SLE patients. [ABSTRACT FROM AUTHOR]

PMF of infancy is a recently described autosomal recessive disorder presenting with severe bone marrow failure, accelerated neutrophil apoptosis, and significant platelet dysfunction, caused by a mutation in the VPS45 gene. In this study, we update our group of patients with PMF, highlighting different aspects of this disease, and evaluating the effectiveness of HSCT for the treatment of this disorder. Update of clinical data, hematological features, molecular studies, treatment and final outcome of four children diagnosed with VPS 45-associated PMF of infancy. The patients described had clinical and hematological findings consistent with MF. Molecular studies showed that all patients were homozygous for the Thr224Asn mutation in the VPS 45 gene. HSCT was carried out in three patients and was successful in two. VPS 45-associated MF is a novel primary immune deficiency that can be successfully corrected by HSCT if applied early in the course of disease using appropriate conditioning. The diagnosis of VPS 45-associated PMF should be considered in all children presenting with SCN with subsequent development of pancytopenia. Long-term follow-up of these patients is necessary to identify extra-hematological manifestations of VPS45 deficiency. [ABSTRACT FROM AUTHOR]

Autologous stem cell transplantation (ASCT) has improved the prognosis of selected patients with immunoglobulin amyloid light-chain (AL) amyloidosis. However, there exist little data regarding Asian patients receiving ASCT. We retrospectively analyzed 24 patients who were treated with ASCT for AL amyloidosis between 2007 and 2012. The median age at the time of ASCT was 56 years (range: 40-62 years), and in 63% of patients, the AL amyloidosis involved two or more organs. All patients except one received induction treatment such as cyclophosphamide, dexamethasone or thalidomide before ASCT, but only approximately half of these patients showed a hematologic response. After ASCT, the overall hematologic response was increased to 92% (22/24), which translated into increased organ response in 58% of patients (14/24). There was no transplantation-related mortality (0%) even though cardiac amyloidosis patients were included in our series. In conclusion, our results of ASCT in patients with AL amyloidosis were comparable to that of Western countries in terms of response and survival outcomes. Therefore, ASCT is an effective and feasible treatment approach for Asian patients with AL amyloidosis. [ABSTRACT FROM AUTHOR]

The objective of this observational study is to describe clinical, haematological and serum biochemical findings of horses affected with alfalfa dodder (Cuscuta campestris) toxicity. Twenty horses naturally exposed to alfalfa dodder toxicity were examined and information was collected on history and clinical signs. Physical examination was done on horses in the premises (n=20), and venous blood samples of 12 horses were submitted for haematology and serum biochemical examination for each horse. Abnormal clinical signs started around 36 hours after horses were fed the contaminated alfalfa. Abnormal signs were seen in 11 horses and those included diarrhoea (n=8), decreased appetite (n=7), neurological signs (n=4) and abdominal pain (n=1). Some horses had multiple clinical signs of the above. The results of complete blood cell count revealed leukocytopenia, neutropenia and thrombocytopenia. Serum biochemical analysis revealed decreased ALP, AST and CPK levels and increased direct bilirubin level. The used alfalfa was stopped immediately and a different alfalfa from a new container that did not contain any weeds was fed. Horses on the premises were observed closely, and the abnormal clinical signs resolved within three days. No treatment was implemented. Knowledge about toxicity of horses by Cuscuta species is scarce in the English veterinary literature and very limited. [ABSTRACT FROM AUTHOR]

Background: Cachexia is a state of involuntary weight loss common to many chronic diseases. Experimental data, showing that cachexia is related to the enhancement of acute phase response reaction, led to the new definition of cachexia that included, aside from the principal criterion of weight loss, other "minor criteria", Amongst them are levels of C-reactive protein (CRP), albumin and hemoglobin. However, there is paucity of data regarding possible differences of these laboratory parameters in patients with various diseases known to be related to cachexia. Methods: CRP, albumin and hemoglobin were evaluated in 119 patients, divided into two disease groups, hematological (ones with diagnosis of non-Hodgkin lymphoma or Hodgkin disease) and non-hematological (solid tumor patients and patients with chronic heart failure). Patients were further subdivided into two nutritional groups, cachectic and non-cachectic ones according to the principal criterion for cacxehia i.e. loss of body weight. Results: We found that cachectic patients had higher levels of CRP, and lower levels of both hemoglobin and albumin compared to non-cachectic patients, regardless of the disease group they fitted. On the other hand, the group of hematological patients had lower levels of CRP primarily due to the differences found in the non-cachectic group. Higher levels of albumin were also found in the hematological group regardless of the nutritional group they fitted. Limitations of cut-off values, proposed by definition, were found, mostly regarding their relatively low sensitivity and low negative predictive value. Conclusions: As expected, differences in values of routine laboratory parameters used in definition of cachexia were found between cachectic and non-cachectic patients. Their values differed between hematological and non-hematological patients both in cachectic and non-cachectic group. Cut-off levels currently used in definition of cachexia have limitations and should be further evaluated. [ABSTRACT FROM AUTHOR]

Abstract: Hemophagocytic lymphohistiocytosis is a life-threatening condition associated with multiple organ dysfunctions. This entity is related to inappropriate stimulation and proliferation of cytotoxic lymphocytes and macrophages inducing phagocytosis of blood cells. Hemophagocytic lymphohistiocytosis should be considered rapidly in any unexplained febrile cytopenia. Biological markers are high ferritin and triglyceride levels, and low fibrinogen. Hemophagocytic lymphohistiocytosis diagnosis should not be ruled on or out solely on the presence or absence of hemophagocytosis features on smear or biopsy sampling. It is either “primary/genetic” (pediatric or familial disorders) and characterized by a lack of intrinsic cytotoxicity of NK cells or T CD8 lymphocyte, or “secondary/reactive” due to malignancy, infectious or autoimmune origin. Mortality is 50% (including all etiologies), and this severity requires rapid and “aggressive” investigations with multidisciplinary approach including intensive care unit team. The immediate aim of therapy is suppression of the severe hyper-inflammation, which can lead to multiple organ failure. Emergency treatment is currently based on etoposide (VP16), pending to the identification and treatment of underlying cause. [Copyright &y& Elsevier]

In humans, the majority of all protein-coding transcripts contain introns that are removed by mRNA splicing carried out by spliceosomes. Mutations in the spliceosome machinery have recently been identified using whole-exome/genome technologies in myelodysplastic syndromes (MDS) and in other hematological disorders. Alterations in splicing factor 3 subunit b1 (SF3b1) were the first spliceosomal mutations described, immediately followed by identification of other splicing factor mutations, including U2 small nuclear RNA auxillary factor 1 (U2AF1) and serine arginine-rich splicing factor 2 (SRSF2). SF3b1/U2AF1/SRSF2 mutations occur at varying frequencies in different disease subtypes, each contributing to differences in survival outcomes. However, the exact functional consequences of these spliceosomal mutations in the pathogenesis of MDS and other hematological malignancies remain largely unknown and subject to intense investigation. For SF3b1, a gain of function mutation may offer the promise of new targeted therapies for diseases that carry this molecular abnormality that can potentially lead to cure. This review aims to provide a comprehensive overview of the emerging role of the spliceosome machinery in the biology of MDS/hematological disorders with an emphasis on the functional consequences of mutations, their clinical significance, and perspectives on how they may influence our understanding and management of diseases affected by these mutations. [ABSTRACT FROM AUTHOR]