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BACKGROUND: In clinical practice, the cytopenias may result from relatively benign causes such as viral infections and Vitamin B12/folic acid deficiency to more sinister causes such as bone marrow failure. In this study, we looked into the clinicohematological profile and etiological factors of bicytopenia and pancytopenia. OBJECTIVES: To study the etiology and clinicohematological profile in patients of peripheral blood cytopenias. MATERIALS AND METHODS: This was a cross-sectional study conducted at a tertiary care hospital over a period of 1 year. Cytopenias were defined as pancytopenia when there was simultaneous presence of hemoglobin

BACKGROUND: Hematopoietic stem cell transplantation (HSCT) has emerged as a curative measure for life-threatening hematological disorders. It can be autologous or allogeneic depending on the disease characteristics. Providing transfusion support to the transplant patients can be challenging, especially in AB-mismatched allogeneic HSCT. In this study, we investigated the impact of ABO incompatibility in patients undergoing allogeneic HSCT. MATERIALS AND METHODS: A retrospective review was conducted in 76 patients with hematological diseases who underwent allogeneic HSCT. Transfusion requirements, engraftment profile, incidence of graft versus host disease (GvHD), and mortality for a period of 1 year were analyzed. RESULTS: ABO incompatibility between donor and the patient did not significantly affect the neutrophil and platelet (PLT) engraftment time (P = 0.389, 0.349, respectively), packed red blood cells transfusion requirement, and duration of initial hospital stay. However, patients of ABO-incompatible HSCT received more PLT transfusions posttransplant which was statistically significant. 29.1% of ABO compatible and 16.7% incompatible HSCT patients developed GVHD. Mortality rates in the two groups were 16.7% and 8.3%, respectively. However, differences in both the parameters were not statistically significant. CONCLUSION: Our study showed that ABO incompatibility does not significantly affect the outcome and should not be a limiting factor for selection of donor. Donor availability and human leukocyte antigen (HLA) matching remain the critical selection criteria. [ABSTRACT FROM AUTHOR]

Background and Objectives: Transfusion of stored, packed red blood cells (PRBCs) is a common therapeutic intervention; however, development of hemolysis during storage might contributes to some negative consequences due to the release of free hemoglobin (fHb), a potent nitric oxide scavenger, which may impair vasodilation and microcirculatory perfusion after transfusion. We undertook this study to find out the change in fHb levels in recipients, whether this affects microvascular oxygenation posttransfusion. Methods: Eighty stable transfusion recipients were selected for this prospective observational study and were divided into three groups as per the age of blood transfused (<7 days, 7-14 days, and >14 days). Blood samples were collected to analyze the plasma levels of fHb in the bag. The patient's circulating plasma Hb (FHb) and pO2 by venous blood gas analysis were studied before and after 1 h of transfusion. Data were analyzed through paired t-test, ANOVA, and Pearson's correlation coefficient using the SPSS version 23. Results: Overall, there was a significant difference between the three groups in terms of change in plasma fHb (g/dL) from pretransfusion to the 1 h posttransfusion time points (P < 0.05). Significant increase in pO2 Levels was noticed from the pretransfusion to 1 h posttransfusion time points in bags <7 days old (t = -4.6, P < 0.001). Furthermore, a strong negative correlation was obtained between FHb and pO2 using Pearson's correlation coefficient (r = -0.738; P < 0.001). Conclusion: This study concludes that red blood cell transfusion can significantly increase posttransfusion plasma Hb levels depending upon the age of PRBC units and has an implication on microvascular oxygenation. However, the effect on the clinical outcome of the patient was not studied, and it would be worthwhile to investigate whether the age of the blood transfused affects the patient morbidity or mortality. [ABSTRACT FROM AUTHOR]

Mothers of children diagnosed with cancer are found to have coping difficulties in various studies. Most of the studies were done on parents after their child has been newly diagnosed with malignancy, and very few studies were done on coping skills intervention. Hence, this study has been done to assess the impact of cognitive behavioural intervention on caregiver burden in mothers of children diagnosed with cancer. Twenty mothers coming to the outpatient department of paediatric oncology from 01 September 2018 to 30 April 2019 were enrolled for the study. The participants were administered General Health Questionnaire, Brief Coping Operation Preference Enquiry Scale, Zung Self-Rating Anxiety Scale, and Coping Inventory for Stressful Situations-21 (CISS-21) Scale. Sixteen sessions of cognitive behavioural intervention were given over 8 weeks to all the participants. Reassessment was done after 3 months by use of the above mentioned scales. Participants' mean anxiety score was 49.40 (standard deviation [SD] ±8.89). They used adaptive (active coping and positive reframing) more than the maladaptive (denial and self-blame) coping strategies. Task- and emotion-focused coping mean score on CISS-21 revealed 19.25 (SD ±6.20) and 18.90 (SD ±5.76), respectively. Reassessment after cognitive behavioural intervention revealed statistically significant improvement in maladaptive coping styles, mean anxiety index score, avoidance, and emotion-focused coping. The study has revealed mild to moderate anxiety and the use of both adaptive and maladaptive coping strategies by participants. There is statistically significant improvement in anxiety, maladaptive coping strategies with cognitive behavioural intervention. [ABSTRACT FROM AUTHOR]

Treatment with high-dose chemotherapy and stem cell transplantation has prolonged survival in patients of multiple myeloma (MM). A dose-response relationship between number of CD34+ cells infused and leukocyte and platelet recovery, exists. Patients receiving dose of <2.0 × 106 CD34+ cells/kg have delayed engraftment. The level of optimal cutoff for accelerated engraftment is yet to be validated. Hence, this study was undertaken to study the association of CD 34+ cell dose with engraftment kinetics in patients of MM who underwent autolgous peripheral blood stem cell transplant (PBSCT). We retrospectively analyzed 19 patients of MM who underwent PBSCT at our center between December 2016 to December 2018. Complete blood counts were carried out daily after transplantation to record neutrophil and platelet engraftment. Based on the CD34+ cell dose given : <5 × 106/kg (category 1), 5−10 × 106/kg (category 2), >5 × 106/kg (category 3), the mean (SD) neutrophil engraftment time was 11.3 (0.5) days, 10.6 (0.9) days, and 10.2 (1.3) days respectively. Platelet engraftment time was 12.4 (2.60) days, 10.6 (1.14) days, and 11.2 (1.64) days for category 1, 2, and 3 patients, respectively. Correlation co-efficient between CD 34+cell dose and days for neutrophil and platelet engraftment was found to be −0.24 and −0.20, respectively. Time for neutrophil engraftment was found to be significantly associated with CD34+ cell dose category. CD 34+ cell dose appears as the strongest predictor of leukocyte and platelet engraftment. CD 34+ cell dose of >5.0 × 106 cells/kg leads to an accelerated neutrophil and platelet engraftment in patients of MM. [ABSTRACT FROM AUTHOR]

Abstract Introduction Diamond-Blackfan anemia and Duchenne muscular dystrophy are two rare congenital anomalies. Both anomalies occurring in the same child is extremely rare. Allogeneic hematopoietic stem cell transplantation is a well-established therapy for Diamond-Blackfan anemia. However, in patients with Duchenne muscular dystrophy, stem cell therapy still remains experimental. Case presentation We report the case of a nine-year-old boy of north Indian descent with Diamond-Blackfan anemia and Duchenne muscular dystrophy who underwent successful allogeneic hematopoietic stem cell transplantation. He is transfusion-independent, and his Duchenne muscular dystrophy has shown no clinical deterioration over the past 45 months. His creatine phosphokinase levels have significantly decreased to 300 U/L from 14,000 U/L pre-transplant. The patient is 100% donor chimera in the hematopoietic system, and his muscle tissue has shown 8% to 10.4% cells of donor origin. Conclusion Our patient's Diamond-Blackfan anemia was cured by allogeneic hematopoietic stem cell transplantation. The interesting clinical observation of a possible benefit in Duchenne muscular dystrophy cannot be ruled out. However, further clinical follow-up with serial muscle biopsies and molecular studies are needed to establish this finding.

Background: Pancytopenia is characterized by decreased hemoglobin (adult males <13 g/dl, pregnant females <10 g/dl, nonpregnant females and children <11 g/dl), total leukocyte count (<4 × 109/L), and platelet count (<100 × 109/L). Depending on the severity, patients can present with varied symptoms. Vast majority of conditions causing pancytopenia are known. Hence, a systematic approach is a mandatory requirement to identify the cause of pancytopenia for appropriate management. Aim: Clinicopathological study of new onset pancytopenia and to determine the frequency of different etiologies causing pancytopenia. Materials and Methods: A prospective study of 451 consecutive cases who had pancytopenia and presented in outpatient or emergency room of a tertiary care center of Western India, from July 2017 to September 2019. The clinical data of the patients were obtained and hematological investigations were analyzed in the hematopathology section of the pathology department. Bone marrow (BM) aspirates and biopsies were performed in 355 cases. Data were analyzed using SPSS software. Results: Patients age ranged from 6 months to 84 years with mean standard deviation of 45.89 ± 19.21 years. Overall male to female ratio was 1.87:1. Hematolymphoid malignancy was the most common cause (n = 137, 30.37%) followed by nutritional anemia (n = 97, 21.5%), infections (n = 68, 15.07%), BM failure (n = 66, 14.63%), hypersplenism (n = 55, 12.19%), and autoimmune disorder (n = 11, 2.44%). Conclusion: Pancytopenia is a common hematological entity with the multifactorial etiologies. It can present as a life-threatening condition or can be merely transient. Hematolymphoid malignancy was found to be the most common underlying cause in our study. An accurate etiological break will further assist in appropriate management of these patients. [ABSTRACT FROM AUTHOR]

Background Donor factors have a variable correlation with cluster of differentiation (CD)34+ cell dose in allogeneic peripheral blood stem cell (PBSC) harvests. CD34+ cell dose affects the speed of hematopoietic recovery and percentage of donor chimerism in the recipient. Methods A total of 25 allogeneic PBSC transplants performed during a 3-year period were included. All donors underwent mobilization with filgrastim. Leukapheresis, flowcytometric CD34+ cell enumeration, and chimerism analysis were performed and correlated with recipient outcome. Results Besides age, all other donor parameters had a positive correlation with CD34+ cell count. Engraftment kinetics and chimerism had a positive correlation with the CD34+ yield of the PBSC product. Acute graft-vs-host disease (GVHD) was observed in patients with complete chimerism at day 30 after transplantation. Conclusion Adequate CD34+ cell yield happens in healthy donors, independent of donor demographic patterns with G-CSF only. A diverse population of donors can thus be approached for Matched Unrelated Donor (MUD) transplants. An accurate quantitative analysis of early donor chimerism in the recipient (at day 30) is an excellent tool for post-transplant monitoring for acute GvHD. [ABSTRACT FROM AUTHOR]

PURPOSE: Chronic myeloid leukemia (CML) is the commonest hematologic malignancy in India. There are not many systematic Indian studies that assess response to generic Imatinib mesylate therapy on a large scale, since most published trials from the West and India are with Gleevec (Novartis). METHODS: 101 patients of Chronic Myeloid Leukemia in chronic phase (CML-CP) with a minimum follow up period of 2 years, who had presented to a tertiary care hospital in Western India between 2007 and Feb 2017, were included in this study. All patients received treatment with generic Imatinib Mesylate therapy. Clinical and laboratory examination done at baseline and on follow-up at 1,3,6,9,12 and 24 months was noted. Molecular monitoring with RQPCR at 3 months, 6 months, 12 months and 24 months for BCR: ABL was done. RESULTS: The study was carried out in 101 CML patients (78 males and 23 females) with a median age of 38 years (4 - 77) years at the time of diagnosis. Complete hematological response was noted in 63 (62.4%) at 1 month, 94 (93.1 %) at 3 months, 95 (94.1 %) at 6 months, 97 (96%) at 9 months and 99 (98%) at 12 months. At 12 months, 56 (55.4%) patients achieved Major molecular response (MMR) which was defined as BCRABL: ABL gene levels < 0.1 %. At 24 months, 66 (65.34%) patients were in MMR. CONCLUSIONS: Our study using the Indian generic molecule of Imatinib Mesylate demonstrated comparable hematological and molecular responses as published for the parent molecule, Gleevec (Novartis). [ABSTRACT FROM AUTHOR]

Background: Waldenstrom macroglobulinemia (WM) is a rare mature B cell Neoplasm, characterized by monoclonal Immunoglobulin M (IgM) in the serum and lymphoplasmacytic infiltration in the bone marrow. It accounts for approx 2% of Non Hodgkin Lymphomas. The disease is slightly more common in elderly males, the median age at diagnosis being 63 years. The disease is very infrequent in India and only a few studies have been published on the disease. Hence, we bring out this study on Clinico-Pathological Profile of WM. Aims and Objectives: To study clinico-pathological profile of WM cases and to distinguish them from other plasma cell neoplasms as both the diseases have different treatment protocols and prognosis. Materials and Methods: A cross sectional observational study was conducted at tertiary care center over a period of two years. Six cases of WM were encountered and enrolled for the study due to their rarity and morphological overlap with plasma cell neoplasms. The WHO criteria were followed and patients fulfilling those criteria were included in the study. The diagnosis of WM was based on the morphologic and immunohistochemistry findings of biopsy specimens, the presence of a monoclonal IgM, and the presence of distinctive clinical characteristics. Results: Median age of patients was 60.5 years with a male preponderance. 33% of cases presented with B symptoms and 100% cases presented with moderate to severe anaemia. 50% of the cases had thrombocytopenia and hepatomegaly. All cases had albumin:globulin ratio reversal. Peripheral smear showed background staining, rouleaux formation, relative lymphocytosis. Plasmacytoid cells were present in two cases. Bone marrow aspirate smears showed features of lymphoplasmacytic infiltration with suppression of other lineages. BM biopsies were hypercellular and showed a diffuse pattern of marrow infiltration by lymphoplasmacytic cells. The immunostaining of the infiltrating lymphocytes were CD 20+ and plasma cells were CD138+. Serum protein electrophoresis and immunofixation electrophoresis confirmed IgM type of monoclonal gammopathy. Conclusion: WM is relatively a rare mature B cell neoplasm which at times can have morphological overlap with other plasma cell neoplasms. We conclude that correlation with clinical laboratory test results while making diagnostic decision is an integral part of recognition of WM due to low grade indolent course of the disease and better outcome. [ABSTRACT FROM AUTHOR]