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Background: Cold agglutinin disease (CAD) is an autoimmune hemolytic anemia that induces blood coagulation and hemolysis upon exposure to cold temperatures. Strict temperature control is essential to mitigate these effects, especially during surgical procedures where hypothermia is possible. Case presentation: A 57-year-old male, 165 cm and 72 kg, diagnosed with CAD, underwent cerebral vascular anastomosis. Intraoperatively, mean arterial pressure was maintained at or above 65 mmHg with phenylephrine administration, while body temperature was rigorously controlled between 36.5 °C and 37.5 °C using forced-air warming blankets and heated intravenous infusions. Despite these measures, thrombotic occlusion occurred, necessitating surgical thrombus removal, intravenous heparin administration, and irrigation of the surgical field with warmed saline followed by re-anastomosis. The anastomosis remained patent without recurrence of thrombus formation thereafter. Conclusion: Preventing hypothermia is extremely important in the anesthesia management of CAD patients. However, careful attention must also be paid to temperature regulation in the surgical field. [ABSTRACT FROM AUTHOR]

The article focuses on the complexities of managing autoantibodies and alloantibodies in a patient with Evans syndrome, including the challenges of diagnosis, treatment considerations, and the implications for patient care in autoimmune conditions.

Objective: To study the clinical features and laboratory parameters of neonatal lupus erythematosus (NLE) from India. Patients and methods: We analyzed case records of children diagnosed with NLE in the Pediatric Rheumatology Clinic at tertiary care centre from North India during the period January 1999 - December 2023. Results: Twenty-four babies are diagnosed with NLE during the study period. Median age at diagnosis was 60 days with a female predisposition (Male to female- 1:2). Cutaneous manifestations were reported in 14 (58%) patients. Hepatomegaly was noted in 15 (62.5%), and splenomegaly in 2 (8%). Elevated transaminases were noted in 14 (58%) patients, and cholestatic jaundice in one. Hematological manifestations were noticed in 20 (83.3%), and neurological manifestations were noticed in 8 patients. Most non-cardiac manifestations were self-limiting. Intravenous immunoglobulin was used for phrenic nerve palsy and autoimmune hemolytic anemia. Oral corticosteroid was used in a patient with refractory cytopenia, and four patients required transfusions. Cardiac involvement was reported in 13 (54%) patients – 3rd-degree heart block in 9 patients, and 6 were managed with epicardial pacemaker insertion. One patient required pacemaker reimplantation due to infective endocarditis. Congenital hydrops was seen in 3 patients, and required respiratory support and recovery was uneventful. Conclusion: NLE is associated with significant morbidity. More than half had cardiac manifestation, of which 70% had 3rd-degree heart block requiring pacemaker insertion. Other rare clinical manifestations like phrenic nerve palsy and seizures are noticed in our cohort. [ABSTRACT FROM AUTHOR]

Objective: Antinuclear antibodies (ANA) staining patterns can provide useful information in systemic lupus erythematosus (SLE). In our study, we examined the frequency of ANA staining patterns in disease-related features in childhood-onset SLE patients. Methods: ANA and its staining patterns were assessed in childhood-onset SLE patients. Results: Two hundred twenty-three patients were included (F/M = 3/1). Their median age at diagnosis was 14.3 (11.9-16.1) years. The anti-cell (AC)-4/5 (fine or large speckled) pattern was the most common nuclear ANA pattern (75.8%), while the AC-19 (dense fine speckled) pattern was the most frequently detected cytoplasmic ANA pattern (13.1%). The AC-4/5 (fine or large speckled) patterns were notably seen in fever, acute and chronic cutaneous lupus, arthritis, serositis, hematologic involvement, renal involvement, neuropsychiatric involvement, gastrointestinal involvement, and cardiopulmonary involvement (all p <.001). Conversely, the AC-1 (homogeneous) pattern was significantly detected in oral/nasal ulcers and non-scarring alopecia (both p <.001). Regarding the laboratory features, the AC-4/5 (fine or large speckled) patterns exhibited a predominant seen in autoimmune hemolytic anemia, leukopenia, thrombocytopenia, elevated ESR and CRP, hypocomplementemia, direct Coombs, anti-Smith (Sm), anti-SSA and SS-B, anti-ribonucleoprotein (RNP), anti-histone, anti-ribosomal P, lupus anticoagulant, anti-cardiolipin immunoglobulin (Ig)M/IgG, and anti-β2-glycoprotein IgM/IgG positivities (all p <.001). In contrast, the AC-1 (homogeneous) pattern was detected in anti-double-stranded (ds) DNA and anti-histone positivity (both p <.001). Conclusion: Our study showed that AC-4/5 and AC-1 patterns of ANA are frequently detected in many clinical and serological features of childhood-onset SLE patients. However, further studies are needed in larger populations to verify these results. [ABSTRACT FROM AUTHOR]

Objectives: Warm autoimmune hemolytic anemia (wAIHA) is a rare autoantibody‐mediated disorder, and first‐line treatment primarily relies on corticosteroids. This study assessed overall survival (OS) and treatment patterns of wAIHA in Sweden. Methods: Adults with ≥ 1 primary diagnosis code for wAIHA (or AIHA plus oral corticosteroids (OCS)/immunosuppressants as sensitivity analyses) between 2011 and 2022 were identified from five Swedish national registers and linked through each patient's unique identity number. Kaplan–Meier curves with log‐rank tests and Cox regressions were performed to assess OS for patients with primary versus secondary wAIHA and patients with wAIHA and long‐term versus short‐term (≥ 3 vs. < 3 months) OCS users. Results: The main analysis included 292 patients; 1791 patients were included in the sensitivity analysis. At a median 3.7‐year follow‐up, a median OS in primary wAIHA was not reached versus 6.0 years for secondary wAIHA (log‐rank test: p = 0.003). Subgroup analyses showed no significant difference in risk of death between long‐term and short‐term OCS users; however, in the sensitivity analysis, long‐term OCS users showed significantly higher risk of death (adjusted hazard ratio: 1.45; 95% confidence interval: 1.180, 1.781; p < 0.001) versus short‐term OCS users. Conclusion: Secondary wAIHA or long‐term OCS use was associated with lower OS, underscoring the disease burden and unmet need for efficacious wAIHA treatments. [ABSTRACT FROM AUTHOR]

Background: Evans syndrome is a rare disorder characterized by the simultaneous or sequential combination of autoimmune hemolytic anemia and immunological thrombocytopenia, together with a positive direct antiglobulin test. This syndrome, which can be primary or secondary, is a rare initial manifestation of autoimmune diseases, notably systemic lupus erythematosus, with 1.7–2.7% of patients with systemic lupus erythematosus developing secondary Evans syndrome, sometimes preceding the onset of systemic lupus erythematosus symptoms. Case presentation: A 47-year-old Middle Eastern female presented with symptoms including shortness of breath, chest pain, and weakness. Physical examination revealed pallor, pale conjunctiva, icteric sclera, tachycardia, and tachypnea. She was diagnosed with Evans syndrome owing to hemolytic anemia, thrombocytopenia, and a positive Coombs test, where initial resistance to treatment prompted intensive therapy with methylprednisolone, intravenous immunoglobulin, and rituximab. Subsequent identification of systemic lupus erythematosus on the basis of positive anti-nuclear antibodies and anti-double-stranded deoxyribonucleic acid antibodies led to treatment with mycophenolate mofetil and hydroxychloroquine. Further evaluations uncovered Hashimoto's thyroiditis and pernicious anemia, necessitating thyroxine supplementation and vitamin B12 injections. Conclusion: Hematologic abnormalities play a crucial role in the diagnostic framework of systemic lupus erythematosus. This case highlights a patient initially diagnosed with Evans syndrome, revealing an underlying systemic lupus erythematosus. The presentation underscores the significance of hematologic manifestations as integral components of the diagnostic framework for autoimmune diseases, emphasizing the intricate relationship between Evans syndrome and systemic lupus erythematosus. [ABSTRACT FROM AUTHOR]

Mutations in the recombination-activating gene 1, a pivotal component essential for V(D)J recombination and the formation of T- and B-cell receptors, can result in autoimmune hemolytic anemia, a rare hematological condition characterized by the autoantibody-mediated destruction of red blood cells. Herein, we report the case of a 1-year-and-4-month-old girl who presented with progressively aggravated anemia, fever, and cough. Autoimmune hemolytic anemia was confirmed by bone marrow aspiration and Coombs test. During treatment, the patient experienced two episodes of severe pneumonia and respiratory failure. Next-generation metagenomic sequencing of sputum samples confirmed the presence of cytomegalovirus and Pneumocystis jirovecii infections. Additionally, lymphocyte subset analysis revealed a T-B+ immunodeficiency. Whole exome and Sanger sequencing revealed a pathogenic recombinase-activating gene 1 mutation (c.2095C>T, p.Arg699Trp) and a likely pathogenic variant (c.2690G>A, p.Arg897Gln), resulting in a missense mutation in the amino acid sequence of the coding protein. Consequently, the patient was diagnosed with a recombination-activating gene 1 mutation and autoimmune hemolytic anemia as the initial presentation. This study reports a case of a recombination-activating gene 1 mutation in China and documents a combination of mutation sites and associated clinical phenotypes that were previously unreported. In this study, we outline the diverse clinical phenotypes observed in cases of recombination-activating gene 1 mutations presenting with autoimmune hemolytic anemia, aiming to facilitate timely diagnosis and appropriate treatment. [ABSTRACT FROM AUTHOR]

Background: Allo-HSCT is a curative therapy for patients with transfusion-dependent thalassemia (TDT). The high incidence of transplant-related complications is becoming an obstacle to safe and effective unrelated donor (URD) transplantation. Methods: In this retrospective study, we reported the survival outcomes and complications of transplantation in thalassemia patients using a novel regimen consisting of pre-transplantation immunosuppression (PTIS) and modified myeloablative conditioning based on intravenous busulfan, cyclophosphamide, fludarabine, and rabbit anti-human thymocyte immunoglobulin. Results: A total of 88 thalassemia patients received the novel conditioning regimen (NCR group), while 118 patients received the conventional conditioning regimen (CCR group). The median age at HSCT in the NCR group was older (7 years vs. 4 years, p < 0.05). No patient in the NCR group experienced primary graft failure, while the 3-year probabilities of OS and TFS were 96.6% and 93.2%, respectively. Even when the intensity of conditioning was reduced, OS (94.8% vs. 94.3%, p = 0.848) and TFS (89.8% vs. 92.5%, p = 0.663) in URD transplants in the NCR group were comparable to those in the CCR group, while the risk of autoimmune hemolytic anemia (AIHA) (0% vs. 15.1%) was lower. In addition, the NCR group had lower rates of mixed chimerism (7.1%). Conclusions: URD transplantation can achieve a comparable prognosis to matched sibling donor (MSD) transplantation with a lower incidence of AIHA due to PTIS and modified myeloablative conditioning regimen. [ABSTRACT FROM AUTHOR]

Introduction: Primary bone marrow diffuse large B-cell lymphoma is a rare clinical entity, and the "bone marrow–liver–spleen" type of diffuse large B-cell lymphoma is rarer, with only a few published cases in literature. Though bone marrow–liver–spleen-type diffuse large B-cell lymphoma has unique presentations such as fever, cytopenias, and hemophagocytic lymphohistiocytosis, no cases with cold autoimmune hemolytic anemia have been reported. Case presentation: A 39-year-old Sri Lankan woman, previously healthy, presented with shortness of breath, productive cough, and fever for 4 days. Examination revealed pallor, icterus, and massive hepatosplenomegaly with no peripheral lymphadenopathy. Further investigation revealed pancytopenia (hemoglobin 58 g/L, white blood cell count 1.73 × 109/L, platelets 23 × 109/L, a reticulocyte index of 4.43%, and lactate dehydrogenase levels of 1690 U/L). Blood picture analysis was suggestive of hemolytic anemia, which was confirmed by a positive direct antiglobulin test with anti-C3d. The bone marrow biopsy revealed markedly hypercellular marrow with polymorphic infiltrate of mononuclear cells accounting for about 80–85% of nucleated cells. These cells were predominantly medium to large cells in size with scanty cytoplasm, irregular nuclear margins, prominent nucleoli, and many mitotic figures. These mononuclear cells were positive for immunohistochemical markers of CD20, BCL2, and CD10. The Ki-67 index was 24%. In addition, this patient had cold autoimmune hemolytic anemia. Contrast-enhanced computed tomography of the chest, abdomen, and pelvis revealed homogeneously enlarged liver and spleen with no significant lymphadenopathy. These findings were compatible with the diagnosis of bone marrow–liver–spleen-type diffuse large B-cell lymphoma. The patient was referred for specialized oncological management. Conclusion: Though there are reported cases of primary bone marrow diffuse large B-cell lymphoma presenting with cold autoimmune hemolytic anemia, no such cases of bone marrow–liver–spleen-type diffuse large B-cell lymphoma have been reported. As this unique entity has a rather grim prognosis, it is of utmost importance to identify it early and treat aggressively. Owing to the limited availability of published accounts of this uncommon disease, we believe it is important to document our case to add to the understanding of this rare condition and its various presentations, which can easily be misinterpreted. [ABSTRACT FROM AUTHOR]

Background: Zidovudine is an antiviral drug used to treat acquired immune deficiency syndrome (AIDS). Anaemia is a common adverse effect of zidovudine that usually occurs in the first 2 to 4 weeks of initial treatment. Here, we describe a patient with HIV infection who developed severe aplastic anaemia 11 years after taking zidovudine. To our knowledge, this is the first case of severe aplastic anaemia caused by the long-term use of zidovudine. Case presentation: A 28-year-old male patient with AIDS developed severe aplastic anaemia after taking zidovudine for 11 years. The patient went to the emergency department due to weakness and falls. He was diagnosed with severe anaemia because his haemoglobin (Hb) level was 31 g/L and his red blood cell (RBC) count was 0.89 × 1012/L. Gastrointestinal bleeding, haematological diseases and autoimmune haemolytic anaemia were ruled out by gastroenteroscopy, stool routine examination and bone marrow puncture biopsy after admission. When the causality of adverse drug reactions was assessed, the Naranjo score of zidovudine was 8, indicating that severe aplastic anaemia was likely related to zidovudine. Given that bone marrow suppression is a side effect of zidovudine, this drug was discontinued. Lamivudine and dolutegravir sodium tablets were subsequently used as antiviral therapies. The patient's symptoms of anaemia improved with the transfusion of suspended RBCs, oral polysaccharide iron complex capsules and folic acid tablets. After 2 weeks of treatment, the patient's symptoms of fatigue improved, with a Hb level of 70 g/L and a RBC count of 2.13 × 1012/L, which were significantly better than before, and he was discharged. At the follow-up 6 weeks after discharge, the patient's Hb level was 156 g/L, his RBC count was 4.67 × 1012/L, and the anaemia was resolved. Conclusions: Zidovudine has bone marrow suppression toxicity, which manifests as anaemia and can occur at any stage of use. Thus, we should not only pay attention to the initial stage of medication but also regularly monitor the patient via routine blood tests in the later stage. If anaemia occurs during the use of zidovudine, physicians should consider the possibility that anaemia is related to the administration of zidovudine and prescribe medication cessation and symptomatic treatment. [ABSTRACT FROM AUTHOR]

The article discusses the successful treatment of a patient with immune-mediated thrombotic thrombocytopenic purpura (iTTP) using daratumumab after multiple relapses and treatments, including splenectomy. Daratumumab, a humanized anti-CD38 antibody, led to a rapid immunological response in the patient and other cases reported in the literature. The treatment showed promising results in refractory iTTP patients, although the long-term efficacy and safety of daratumumab remain unclear. [Extracted from the article]

The article discusses the use of pembrolizumab as a salvage treatment for T‐cell/histiocyte‐rich large B‐cell lymphoma (TCRLBCL) and Epstein–Barr virus (EBV)‐positive large B‐cell lymphoma. The study evaluated the efficacy of pembrolizumab in seven patients with these lymphomas who had relapsed or were refractory to standard chemo‐immunotherapy. Results showed that 71% of patients achieved a complete remission with pembrolizumab, with limited treatment toxicities. The study suggests that checkpoint inhibitors like pembrolizumab may be a potential therapeutic alternative for patients who do not benefit from CAR T‐cell therapy. [Extracted from the article]

Thalassaemia, a common inherited blood disorder, involves defective globin production leading to premature erythrocyte destruction. Autoimmune haemolytic anaemia (AIHA), a rare complication in thalassaemia, is characterised by autoantibody production against erythrocytes. We present a case of a 7-month-old boy diagnosed with β-thalassaemia major (BTM), complicated by AIHA six weeks after initiating blood transfusions. Despite daily transfusions and oral Prednisolone, the haemoglobin (Hb) level dropped to a life-threatening level (2.5g/dl). High-dose IV Methylprednisolone and Rituximab were administered, resulting in Hb improvement to 11.9 g/dL. AIHA resolution was indicated by negative C3d and reduced IgG on repeated testing. Recognition of AIHA in BTM is vital for effective management, including immunosuppressive therapy, leucodepleted transfusions, and potential splenectomy or haematopoietic stem cell transplant. Early intervention is crucial to reduce the mortality associated with AIHA in children. [ABSTRACT FROM AUTHOR]

Introduction: Chronic myeloid leukemia (CML) is a myeloproliferative neoplasm characterized by the Philadelphia chromosome and uncontrolled granulocyte production. While autoimmune hemolytic anemia (AIHA) is commonly associated with lymphoproliferative disorders such as chronic lymphocytic leukemia, its occurrence in CML is rare, with only a few documented cases. Here, we report a case of concurrent chronic-phase CML and AIHA. Case Presentation: We present a 34-year-old female diagnosed with warm AIHA as the first presentation of CML. Diagnostic workup, including peripheral smear, revealed hemolysis with elevated lactate dehydrogenase and a positive direct antiglobulin test (DAT), along with a positive Philadelphia chromosome, confirming CML. Steroid therapy successfully managed the AIHA, leading to normalization of hemoglobin levels and subsequent tapering and discontinuation of steroids. Conclusion: While AIHA is a rare cause of anemia in CML, it should be considered during anemia evaluation, particularly in cases of CML relapse. [ABSTRACT FROM AUTHOR]

Copyright of Biomédica: Revista del Instituto Nacional de Salud is the property of Instituto Nacional de Salud of Colombia and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Cold agglutinin disease (CAD), a rare autoimmune hemolytic anemia (AIHA), is characterized by hemolysis triggered by activation of the classical complement pathway. AIHA is estimated to affect one in 100,000 people in the general population; however, its incidence in pregnant women is unclear due to the scarcity of published studies. Here, we present the case of a 37‐year‐old female (G2P1102) who presented for a repeat Cesarean section. Her peripartum course was complicated by anemia (hemoglobin 7.9 g/dL, hematocrit 29.4%) attributed to cold agglutinin disease, necessitating the transfusion of 20 units of blood during this pregnancy. She had been receiving prednisone 10 mg daily for anemia. Cold temperatures can trigger the activation of cold‐reactive antibodies, leading to red blood cells agglutination (clumping together). Preventing hypothermia reduces the likelihood of cold‐induced hemolysis, which is the primary therapeutic strategy for cold agglutinin disease. Anesthetic management for the cesarean section was accomplished via spinal anesthesia, complemented by pain control through bilateral transversus abdominis plane (TAP) blocks. This multidisciplinary approach facilitated effective pain management while considering the patient's underlying condition. [ABSTRACT FROM AUTHOR]

The simultaneous occurrence of acute myeloid leukemia (AML) and autoimmune hemolytic anemia (AIHA) is exceedingly rare, with an incidence of less than 1%. We report the case of a 50–year–old patient newly diagnosed with this uncommon combination. This case underscores the complexity and infrequency of this dual diagnosis, highlighting the diagnostic and therapeutic challenges it presents. [ABSTRACT FROM AUTHOR]

Endo-β-N-acetylglucosaminidases (ENGases) that specifically hydrolyze the Asn297-linked glycan on immunoglobulin G (IgG) antibodies, the major molecular determinant of fragment crystallizable (Fc) γ receptor (FcγR) binding, are exceedingly rare. All previously characterized IgG-specific ENGases are multi-domain proteins secreted as an immune evasion strategy by Streptococcus pyogenes strains. Here, using in silico analysis and mass spectrometry techniques, we identified a family of single-domain ENGases secreted by pathogenic corynebacterial species that exhibit strict specificity for IgG antibodies. By X-ray crystallographic and surface plasmon resonance analyses, we found that the most catalytically efficient IgG-specific ENGase family member recognizes both protein and glycan components of IgG. Employing in vivo models, we demonstrated the remarkable efficacy of this IgG-specific ENGase in mitigating numerous pathologies that rely on FcγR-mediated effector functions, including T and B lymphocyte depletion, autoimmune hemolytic anemia, and antibody-dependent enhancement of dengue disease, revealing its potential for treating and/or preventing a wide range of IgG-mediated diseases in humans. [Display omitted] • Discovery of a family of corynebacterial single-domain IgG-specific endoglycosidases • IgG-specific endoglycosidases target both protein and glycan components of human IgG • Prototypical family member CU43 mitigates diverse IgG-mediated pathologies in vivo • CU43 is at least 4,000-fold more effective than efgartigimod in vivo A family of ENGases secreted by corynebacterial species strictly targeting the Asn297-linked glycan on human IgG antibodies was discovered. The prototypical family member, CU43, demonstrated remarkable efficacy in treating pathologies that rely on Fc γ receptor (FcγR)-mediated effector functions, including T and B lymphocyte depletion, autoimmune hemolytic anemia, and antibody-dependent enhancement of dengue disease activity. [ABSTRACT FROM AUTHOR]

Importance: A global outbreak of clade IIb Monkeypox virus (MPXV) infections spread rapidly across at least 118 countries resulting in a Public Health Emergency of International Concern (PHEIC) from July 2022 to May 2023. This outbreak affected more than 99 000 persons worldwide and caused more than 33 000 infections and 60 deaths in the US. In 2024, there have been approximately 200 new infections per month in the US. On August 14, 2024, the World Health Organization declared mpox a PHEIC for a second time due to a rapid increase in infections with clade I MPXV in Central Africa. Observations: Mpox is primarily acquired through direct skin to skin contact with MPXV. With clade IIb MPXV, infections are most commonly associated with sexual activity among individuals who are gay, bisexual, and other men who have sex with men. After a median incubation period of 7 to 10 days, prodromal symptoms include fever (62%-72%), lymphadenopathy (56%-86%), myalgias (31%-55%), malaise (23%-57%), and headache (25%-55%). Skin lesions progress through 4 well-defined stages (macules, papules, vesicles, and pustules) over 2 to 4 weeks. Clade IIb MPXV is typically a self-limited illness with a low mortality rate (<0.2% in the US); however, severe illness and death may occur in immunocompromised individuals, especially those with advanced HIV (CD4 count <200 cells/μL). Mpox should be suspected in patients with potential exposure to MPXV who have skin lesions, and the diagnosis is confirmed with polymerase chain reaction testing of lesions. Management is supportive and focuses on skin care and symptom relief with analgesics. While no antiviral treatments are currently approved for mpox by the US Food and Drug Administration, several therapeutics, such as tecovirimat, brincidofovir, and vaccinia immune globulin intravenous, are available through expanded access programs or clinical trials. Vaccination with the 2-dose Modified Vaccinia Ankara-Bavarian Nordic vaccine is recommended for high-incidence populations and has an efficacy of 66% to 86%. Conclusions and Relevance: Mpox is a viral infection transmitted primarily through close skin to skin contact that typically causes a self-resolving illness but can result in severe illness and death in immunocompromised individuals. First-line therapy is supportive care, although patients with severe mpox infection may be treated with advanced therapeutics. Mpox vaccination is effective and, if available, should be offered to individuals at risk of exposure to mpox. This review examines the epidemiology, transmission, clinical presentation, assessment and diagnosis, prognosis, management and treatment, and prevention of mpox. [ABSTRACT FROM AUTHOR]

Piperacillin is a beta-lactamase inhibitor frequently used in the treatment of urinary tract infections. It is a broad-spectrum antibiotic with strong antibacterial action against Pseudomonas aeruginosa and Enterobacter , especially extended-spectrum beta-lactamase-producing Enterobacteria and Enterococcus. Side effects of piperacillin include allergic reactions, rashes such as urticaria, leukopenia, interstitial nephritis, asthma attacks, serological reactions, candida infection, and bleeding with more severe reactions resulting in anaphylactic shock. Anemia and hemolytic anemia are rare adverse reactions to piperacillin, with an incidence of 0.01–0.10%. We report herein the case of a severe postoperative immune hemolytic reaction to piperacillin. Fortunately, we quickly recognized and identified the drug reaction caused by piperacillin, immediately stopped the use of piperacillin, and performed a blood transfusion. The patient recovered and was subsequently discharged from the hospital. [ABSTRACT FROM AUTHOR]

Objectives: Neuromyelitis optica (NMO) is an inflammatory, autoimmune and demyelinating disease of the central nervous system and is often characterized by attacks of severe optic neuritis and long segment myelitis. Identifying the disease-specific pathogenic anti-AQP4 autoantibody in NMOSD has allowed the development of highly effective disease-modifying drugs in the treatment phase. Eculizumab is a humanized antibody that binds to complement C5 and inhibits the formation of the C5b-induced membrane attack complex. It is approved for treating many diseases in which tissue damage is accompanied by complement (such as neuromyelitis optica, myasthenia gravis, autoimmune hemolytic anemia and paroxysmal hemoglobinuria). Methods: We present a patient diagnosed with NMO who developed possible drug-induced liver injury three months after the start of eculizumab treatment. Result: After discontinuing eculizumab treatment, liver function tests gradually regressed in a month. Conclusions: Eculizumab-associated hepatotoxicity is a previously unreported adverse event in NMOSD patients. Therefore, patients should be monitored for liver function tests during eculizumab treatment, and care should be taken for hepatotoxicity. If hepatotoxicity is detected while under eculizumab treatment, patients should be investigated for other drug use, complementary food supplementation, or possible autoimmune hepatitis, and other potential causes should be excluded. [ABSTRACT FROM AUTHOR]

The article discusses a case of a young girl with a rare immune disorder called autoimmune lymphoproliferative syndrome (ALPS) due to a biallelic FASL gene mutation. The girl developed Hodgkin lymphoma and underwent a successful hematopoietic stem cell transplant as part of her treatment. The case highlights the challenges and successful management of ALPS-FASL and associated malignancies. The study emphasizes the importance of genetic analysis, regular monitoring, and appropriate treatment in such cases. [Extracted from the article]

Cytotoxic T-lymphocyte associated antigen-4 (CTLA-4) is a costimulatory receptor exhibiting a potent inhibitory signal on antigen-activated immune responses. A soluble form, sCTLA-4, has been identified and was found to be increased in several autoimmune diseases. We aimed to evaluate serum levels of sCTLA-4 in different immune cytopenias, and to determine its possible relation to the disease activity. We measured serum levels of sCTLA-4 in 47 patients with immune cytopenias and compared them to 47 age- and sex-matched healthy controls. sCTLA-4 levels were significantly higher in patients with immune cytopenias compared to healthy controls (p < 0.001), however, levels were comparable between different groups of immune cytopenias (p = 0.084). Serum sCTLA-4 inversely correlated with age at diagnosis and hemoglobin level (p = 0.048, and p = 0.039 respectively), while it directly correlated with disease duration (p = 0.023) as well as markers of hemolysis including reticulocyte count, serum LDH and indirect bilirubin (p = 0.025; p = 0.019; p = 0.004 respectively). In the AIHA group, serum sCTLA-4 levels were significantly lower in patients in remission compared to patients with active disease (p = 0.026). Children with immune cytopenia exhibit significantly higher levels of circulating sCTLA-4 which correlated with disease activity, yet the prognostic significance and its use to tailor treatment regimen require additional studies. [ABSTRACT FROM AUTHOR]

Introduction: Splenectomy has been used as a diagnostic and therapeutic tool in the management of hematological diseases for many years. However, the emergence of new medical therapies has modified guidelines for many hematological diseases for which splenectomy was previously considered. We aimed to evaluate the evidence of a decrease in the hematological indications for splenectomy and the reasons and justifications for this change. Material and methods: We conducted a single-center, retrospective analysis of patients who underwent laparoscopic splenectomy for hematological disease between January 2010 and December 2023. Patients were classified into four groups: 1 autoimmune and hemolytic diseases (HAD), (2) lymphomas, (3) myeloproliferative diseases (MPN), and (4) other splenic diseases. We recorded the annual incidence of splenectomy and the ratio of new medical cases, demographic and clinical data and surgical outcomes. Results: During the study period, 98 patients were referred for splenectomy. There was a significant progressive decrease in this surgical indication, particularly regarding HAD (p < 0.001). The indication for splenectomy for immune thrombocytopenic purpura (ITP) declined to zero despite an increase in the number of patients diagnosed with this disorder (p < 0.001). The pattern of decrease in AHAI and Evans syndrome was similar to that in ITP. The group of splenectomies due to lymphoma persisted consistently during the study period, as did the indication for splenectomy in the context of lymphoma treatment. Splenectomy due to massive splenomegaly secondary to MPN was indicated only in one patient. Splenectomies due to other causes were similarly distributed over the years. Conclusions: Our findings confirm a significant decrease in the indication for elective surgery for hematological diseases, mainly regarding autoimmune disease. The surgical community and surgical departments should be aware of this situation yet maintain the skills to adopt this technique both safely and efficiently. [ABSTRACT FROM AUTHOR]

Copyright of African Journal of Reproductive Health is the property of Women's Health & Action Research Centre and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

INTRODUCTION: Autoimmune hemolytic anemia (AIHA) is a condition in which there is decreased survival of red blood cells (RBC) due to the destruction of RBC by autoantibodies. AIHA is classified into warm, cold, and mixed according to temperature sensitivity. The antibodies may be immunoglobulin G, immunoglobulin M, immunoglobulin A, or complement proteins, and hemolysis may be intravascular or extravascular. The present study was done to find out serological characteristics of AIHA patients in our population. MATERIALS AND METHODS: During the study, a total of 112 patients’ samples were analyzed. All immunohematology workup, including blood grouping, direct Coombs test (DCT), indirect Coombs test (ICT), monospecific DCT, and alloantibody identification, were done. RESULTS: A maximum number of patients were in the age group of 16–45 years (69.6%). Primary AIHA (56.25%) is more common than secondary AIHA. Females (73.2%) were more affected than men, mostly due to the prevalence of autoimmune disease more in females. Warm AIHA (58%) was more common, followed by mixed (33%) and cold (9%). Grouping discrepancy was seen in 30 (26.8%) cases. CONCLUSION: Warm AIHA is more common in our population followed by mixed and cold types. Blood group discrepancy seen in good proportion of AIHA patients, therefore meticulous immunohematological work up plays an essential role in these patients.

Background: Autoimmune hemolytic anemia (AIHA) is a rare immune disorder which occurs when antibodies are directed against self red blood cells (RBCs) leading to hemolysis. AIHA is widely classified as warm autoimmune hemolytic anemia, cold agglutinin syndrome, mixed AIHA, paroxysmal cold hemoglobinuria and rarely drug induced AIHA. The pathogenesis of AIHA is complex interplay between genetic predisposition, immune dysregulation and enviornmental triggers. A direct antiglobulin test can be used to assess the immunological origin of the hemolysis in order to diagnose AIHA after identifying laboratory and clinical symptoms of hemolysis. Objective: The objective is to understand underlying mechanism in AIHAs, and usage of targeted therapies to modulate specific components of the immune response. Materials and Methods: We are hereby presenting a case series of 11 clinically suspected cases of AIHA in collaboration with their clinical features, immuno-hematological and other laboratory parameters, Flow cytometric analysis of lymphocyte subset in relevant cases, underlying etiology as well as serological subtype are also included. Results: Majority of the patients were categorized as secondary AIHA (7/11, 63.63%). Out of 11 cases 7 were serologically subtyped as warm AIHA (7/11, 63.63%) ,2 cases were DaaT negative AIHA (2/11;18.18%), 2 cases were characterized as mixed AIHA subtype (2/11, 18.18%). Conclusion: Accurate subtyping of AIHA requires a systematic immunohematological approach coupled with comprehensive evaluations of clinical, hematological, and biochemical parameters.

Background: Evans syndrome is an autoimmune condition that manifests as two or more autoimmune cytopenia. We present a case of Evans syndrome diagnosed with an underlying skeletal dysplasia; Schimke immune-osseous dysplasia (SIOD). The condition is characterized by spondyloepiphyseal dysplasia, progressive nephropathy, and T-cell immunodeficiency with cytopenia. Notably, our patient did not exhibit any signs of renal involvement. Case report: A five-and-a-half-year-old female child presented with a short history of bleeding manifestations along with a history of poor weight and height gain. She was subsequently diagnosed with Evans syndrome secondary to an underlying SMARCAL1 mutation, which could be well controlled with oral steroids. Conclusion: SIOD is a rare multisystem disorder affecting the skeletal, renal, immune, and vascular systems. Autoimmune cytopenia was the chief presentation of our patient. Such entities should be suspected in patients with growth failure, bone deformities, and hematological involvement. Early genetic testing will help to reduce disease-related comorbidities and aid in rationalizing treatment strategies.

Objective: Infantile giant cell hepatitis with autoimmune haemolytic anaemia (GCH-AHA) is a rare disease characterised by giant cell and autoimmune haemolysis. The pathogenic mechanisms involve several factors, including genetic and immunological components, particularly those related to the lectin pathway of the complement system. In this study, we aimed to identify possible germline variations in patients with GCH-AHA. Material and Method: Whole-exome sequencing (WES) was performed on a 6-month-old boy who was diagnosed with GCHAHA. An in-house data analysis pipeline was applied to determine familial segregation using Sanger sequencing. ELISA was used for MASP2 protein detection. Result: WES revealed a likely pathogenic heterozygous missense variant (p.(Cys618Tyr)) in the mannose-binding lectin (MBL)-associated serine protease-2 (MASP-2) gene. The MASP2 variant identified in the serine protease domain was predicted to disrupt disulphide bonds. In vitro assays showed decreased MASP2 levels in the patient and mother compared with controls, supporting the potential pathogenicity of the variant. Objective: Infantile giant cell hepatitis with autoimmune haemolytic anaemia (GCH-AHA) is a rare disease characterised by giant cell and autoimmune haemolysis. The pathogenic mechanisms involve several factors, including genetic and immunological components, particularly those related to the lectin pathway of the complement system. In this study, we aimed to identify possible germline variations in patients with GCH-AHA. Material and Method: Whole-exome sequencing (WES) was performed on a 6-month-old boy who was diagnosed with GCHAHA. An in-house data analysis pipeline was applied to determine familial segregation using Sanger sequencing. ELISA was used for MASP2 protein detection. Result: WES revealed a likely pathogenic heterozygous missense variant (p.(Cys618Tyr)) in the mannose-binding lectin (MBL)-associated serine protease-2 (MASP-2) gene. The MASP2 variant identified in the serine protease domain was predicted to disrupt disulphide bonds. In vitro assays showed decreased MASP2 levels in the patient and mother compared with controls, supporting the potential pathogenicity of the variant. [ABSTRACT FROM AUTHOR]

Autoimmune haemolytic anaemia (AIHA) is characterized by an increased destruction of red blood cells due to immune dysfunction and auto‐antibody production. Clinical manifestations are mainly related to anaemia, which can become life‐threatening in case of acute haemolysis. Aiming at counterbalancing severe anaemia, supportive treatments for these patients frequently include transfusions. Unfortunately, free serum auto‐antibodies greatly interfere in pre‐transfusion testing, and the identification of compatible red blood cell units for AIHA patients can be challenging or even impossible. Problems faced in pre‐transfusion testing often lead to delay or abandonment of transfusions for AIHA patients. In this review, we discuss publications concerning global transfusion management in AIHA, with a focus on pre‐transfusion testing, and practical clues to manage the selection of transfusion units for these patients. Depending on the degree of transfusion emergency, we propose an algorithm for the selection and laboratory testing of units to be transfused to AIHA patients. [ABSTRACT FROM AUTHOR]

The most frequent cause of nephritic syndrome in the pediatric population is acute post-infectious glomerulonephritis (PIGN). A rare complication is posterior reversible encephalopathy syndrome (PRES), characterized by subcortical vasogenic cerebral edema associated with variable neurological symptoms. The development of autoimmune hemolytic anemia is an atypical clinical presentation. Objective: To report the coexistence of two unusual and serious extrarenal complications of PIGN and to discuss potential mechanisms involved in their development. Clinical Case: A 4-year-old male patient, with a 5-day history of hematuria and edema, headache, nausea, and vomiting. He was admitted in convulsive status and hypertensive crisis, laboratory showed C3 hypocomplementemia and high titers of Antistreptolysin O, which was interpreted as PIGN. Due to the presence of encephalopathy, PRES secondary to hypertensive emergency was suspected, which was confirmed by brain MRI. He also presented autoimmune hemolytic anemia, with hemoglobin up to 5 g/dL. The antihytreatment was based on antihypertensive therapy, neuroprotective measures, and steroid treatment. He was discharged 31 days after hospitalization and remained asymptomatic 6 months after discharge. Conclusions: There must be a high suspicion index of PRES before the appearance of nonspecific neurological symptoms during the evolution of a PIGN. The existing anemia on occasion can be autoimmune. [ABSTRACT FROM AUTHOR]

Background: Autoimmune hemolytic anemia (AIHA) is a rare disease due to increased destruction of erythrocytes by autoantibodies, with or without complement activation. Summary: AIHA is usually classified in warm AIHA (wAIHA) and cold agglutinin disease (CAD), based on isotype and thermal amplitude of the autoantibody. The direct antiglobulin test (DAT) or Coombs test is the cornerstone of AIHA diagnosis, as it is positive with anti-IgG in wAIHA, and with anti-C3d/IgM antisera plus high titer cold agglutinins in CAD. Therapy is quite different, as steroids and rituximab are effective in the former, but have a lower response rate and duration in the latter. Splenectomy, which is still a good option for young/fit wAIHA, is contraindicated in CAD, and classic immunosuppressants are moving to further lines. Several new drugs are increasingly used or are in trials for relapsed/refractory AIHAs, including B-cell (parsaclisib, ibrutinib, rilzabrutinib), and plasma cell target therapies (bortezomib, daratumumab), bispecific agents (ianalumab, obexelimab, povetacicept), neonatal Fc receptor blockers (nipocalimab), and complement inhibitors (sutimlimab, riliprubart, pegcetacoplan, iptacopan). Clinically, AIHAs are highly heterogeneous, from mild/compensated to life-threatening/fulminant, and may be primary or associated with infections, neoplasms, autoimmune diseases, transplants, immunodeficiencies, and drugs. Along with all these variables, there are rare forms like mixed (wAIHA plus CAD), atypical (IgA or warm IgM driven), and DAT negative, where the diagnosis and clinical management are particularly challenging. Key Messages: This article covers the classic clinical features, diagnosis, and therapy of wAIHA and CAD, and focuses, with the support of clinical vignettes, on difficult diagnosis and refractory/relapsing cases requiring novel therapies. [ABSTRACT FROM AUTHOR]

Introduction: Autoimmune hemolytic anemia (AIHA) occurs in 0.7–5.6% of patients undergoing hematopoietic stem cell transplantation, especially from unrelated or haploidentical donor or after lympho-depleted transplant; the majority of cases are represented by warm AIHA, occurring in a full donor chimerism setting. Standard treatments (corticosteroids, intravenous immunoglobulin, splenectomy, rituximab, cyclophosphamide, plasma exchange) lead to lower response rates than those reported in primary AIHA. Daratumumab use has been proposed in many autoimmune conditions (immune thrombocytopenic purpura, aplastic anemia, thrombotic thrombocytopenic purpura, systemic lupus erythematosus, multiple sclerosis), but only few reports have been published on its use for post-HSCT AIHA, mainly in pediatric patients. Case Presentation: We report the successful use of daratumumab in a 68-year-old patient, suffering from post-HSCT AIHA. Five months after Rh-mismatched HSCT, the patient was diagnosed with anti-D AIHA. After first-line treatment (oral prednisone, rituximab, and plasma exchange) failure, being still transfusion-dependent with symptomatic anemia, he underwent treatment with daratumumab, achieving both clinical and laboratory responses. Discussion: Daratumumab may represent a safe and effective alternative to conventional immunosuppressive therapy, and it deserves further investigations. [ABSTRACT FROM AUTHOR]

This article presents a case study of a Japanese female patient with autosomal dominant TCF3 deficiency, a genetic disorder that affects B-cell development and leads to immunodeficiency. The patient had a unique immunoglobulin profile compared to previously reported cases, with elevated serum IgA levels and decreased serum IgG, IgM, and IgE levels. The patient also experienced episodes of anemia and pure red cell aplasia. Treatment with immunoglobulin replacement therapy was initiated to manage the immunodeficiency. The article suggests that the atypical immunoglobulin profile may be due to a small number of aberrant B-cells. The article also discusses the potential involvement of TCF3 in T-cell differentiation and the need for continuous evaluation of the patient's cellular immunity. [Extracted from the article]

A 48-year-old female presented to the emergency department with severe fatigue. Admission laboratory test results were hemoglobin 6.6 g/dL, platelet count 287,000/μL, and white blood cell count 25,200/μL. Lactate dehydrogenase was elevated at 898 U/L, haptoglobin was markedly decreased (< 31 mg/dL), indirect bilirubin was elevated (5.3 mg/dL), and the absolute reticulocyte count was low at 0.0050/μL. A sample was sent to the immunohematology reference laboratory. The direct antiglobulin test immunoglobulin G was negative; C3 was 1+. All cells were reactive at immediate spin phase, indirect antiglobulin testing (IAT) with polyethylene glycol, with low ionic strength saline, neat, prewarm, and in the solid phase. All cells were nonreactive at IAT-ficin. Additional testing included a cold antibody titer that was 1:4096 and thermal amplitude studies demonstrating reactivity of 2+ at 37°C. These results were consistent with a clinically significant anti-Pr and cold agglutinin disease (CAD). Although rituximab is effective in autoimmune hemolytic anemia, this may take weeks. The patient was treated with pegcetacoplan, a pegylated peptide that targets C3 inhibiting hemolysis. The patient was discharged on day 29 with a hemoglobin of 8 g/dL. This is a report of one of the first patients successfully treated with pegcetacoplan for CAD. [ABSTRACT FROM AUTHOR]

The article analyses data on the occurrence of hematological abnormalities in severe acute respiratory syndrome 2 infection. Among these hematological abnormalities, the majority of patients develop a hypercoagulable state associated with thromboembolic complications and poor prognosis. Approximately one-third of patients with severe acute respiratory syndrome 2 infection are diagnosed with mild to severe thrombocytopenia. Another hematological autoimmune disease observed in patients with coronavirus disease 2019 is autoimmune hemolytic anemia. A clinical case with the development of autoimmune hemolytic anemia in the setting of coronavirus infection was described. The diagnosis was based on the presence of anemia, reticulocytosis, a significant decrease in haptoglobin levels, and a positive antiglobulin test (Coombs test). Given the comorbidity, the risks of adverse effects of severe coronavirus disease were high, despite this, it was possible to achieve clinical and hematological remission of autoimmune hemolytic anemia by prescribing pathogenetic therapy with anti-CD-20 monoclonal antibody (rituximab), recombinant erythropoietin and glucocorticoid hormones. This clinical case demonstrates the possibility of successful treatment of patients with severe hemolytic anemia. Special attention should be paid to the discrepancy between the severity of the condition and objective data. This case demonstrates the need for a more in-depth approach to each patient with anemia associated with coronavirus disease infection, namely, in the presence of anemic syndrome, it is imperative to include a full range of laboratory tests. [ABSTRACT FROM AUTHOR]

Epstein-Barr virus-associated smooth muscle tumor (EBV-SMT) is rare in adults. The presence of intratumoral T lymphocytes and primitive rounded cells characterized this neoplasm. We report a 24-year-old Chinese man who developed EBV-SMT in the right adrenal gland with hepatitis B infection and autoimmune hemolytic anemia without a history of HIV infection, primary immune deficiency, organ transplantation, or malignant tumor. This patient had an unknown immunodeficient state. EBV-SMTs are commonly located in the liver, lung, and gastrointestinal tract but rarely in the adrenal gland. We reviewed 10 reported literature on EBV-SMT in the adrenal gland. It is imperative to distinguish EBV-SMT from conventional somatic smooth muscle tumors. The discovery of EBV-SMT forces the clinician to conduct a thorough evaluation of immune function and immune status surveillance, and these patients are vulnerable to subsequent malignant tumors. [ABSTRACT FROM AUTHOR]

Evans syndrome (ES) is a spectrum of diseases in which the combination of autoimmune hemolytic anemia and immune thrombocytopenia or sometimes neutropenia. ES has been accepted usually as an idiopathic condition, but it may be secondary. The coexistence of autoimmune cytopenias and Hodgkin lymphoma (HL) is rarely observed and the rate of ES in HL patients is not clear. Here we describe a 56-year-old male patient who presented with ES and was diagnosed with HL. After corticosteroids, intravenous immunoglobulin (IVIG) and ABVD (doxorubicin, bleomycin, vinblastine, dacarbazine) treatment, immune cytopenias were completely resolved. The literature is also reviewed and we found 16 cases in which HL and ES coexist. Although AIHA and immune thrombocytopenia usually develop simultaneously, they rarely occur at different times. Many aspects of the pathogenesis are unknown, but it is thought to be a complex immunological background. Corticosteroids and/or IVIG are the most commonly used first-choice drugs in the initial treatment of ES. Response rates to treatment are variable and response to treatment may be poor, particularly with underlying conditions. If detected, the underlying lymphoma should be treated. [ABSTRACT FROM AUTHOR]

Background: Systemic lupus erythematosus (SLE) is a chronic autoimmune disorder characterized by a variety of both signs and symptoms; it mainly affects women of childbearing age, with an estimated prevalence of 24/ 100,000 people in Europe and North America. SLE is often described as an antibodies-driven disease as its clinical manifestations are usually associated with the presence or the absence of specific antibodies. Objectives: To evaluate clinical manifestations in patients with SLE and to assess the relationship with the presence of specific antibodies by using real-world data. Methods: A retrospective study was performed; the 2019 EULAR/ACR Classification Criteria for Systemic Lupus Erythematosus were used to classify patients with SLE. Data concerning serological profiles (which included Antinuclear antibodies -- ANA, anti dsDNA, anti-Ro/SS-A, anti-La/SS-B, anti- Smith) were gathered along with medical records of clinical manifestations. Complement levels were also tested for possible clinical correlations. c² or Fisher's exact tests were utilized to establish associations between autoantibodies and symptoms. The odds ratios (OR) and their 95% confidence intervals (CI) were computed. No correction was made for multiple testing; only a p-value 0.01 ≤ was considered significant. Results: One-hundred and twenty-seven patients (n=127, mean age 53.43 ± 14.02) were enrolled in this study. Anti-dsDNA antibodies were found to be statistically significant for both malar rash and proteinuria; anti-Ro/SSA antibodies showed an association with photosensitivity and pericarditis; furthermore, a strong association was found between anti-Ro antibodies and proteinuria, but only if anti-dsDNA antibodies were present as well. Patients who tested positive for anti-La/SSB antibodies correlated with a threefold increase in the risk of developing pericarditis. Lastly, anti-Smith appeared to be associated with NPSLE as well as an increased risk for both autoimmune hemolytic anemia and thrombocytopenia. [ABSTRACT FROM AUTHOR]

Anti-programmed cell death protein 1 (PD-1) therapy has demonstrated notable efficacy in treating patients with deficient mismatch repair/high microsatellite instability (dMMR/MSI-H) metastatic colorectal cancer (mCRC). However, its clinical application is fraught with challenges and can lead to significant immune-related adverse events (ir-AEs). In this report, we present a complicated case of an mCRC patient with MSI-H and mutations in b2M and LRP1B proteins, complicated by concurrent bacteremia and liver fluke infection, who received first-line anti-PD1 therapy. The patient exhibited a positive response to anti-PD1 treatment, even in the presence of concomitant antibiotic and anti-parasitic interventions. Additionally, the patient experienced immunotherapy-related autoimmune hemolytic anemia (ir-AIHA), a rare hematological ir-AE, which was effectively treated later on. Immunotherapy represents a pivotal and highly effective approach to tumor treatment. Baseline assessment of the MMR and MSI status is a crucial step before initiating immunotherapy, and regular ongoing assessments during the treatment course can facilitate early recognition of any secondary complications, enabling prompt intervention and ensuring optimal therapeutic outcomes. Overall, a multidisciplinary diagnostic and therapeutic algorithm can help maximize the therapeutic benefits of immunotherapy. [ABSTRACT FROM AUTHOR]

Objective: In this study, we aimed to retrospectively evaluate the results of patients with autoimmune hemolytic anemia (AIHA) who had undergone splenectomy. Methods: The records of 25 patients with AIHA refractory to steroids and who underwent splenectomy between January 1, 2000, and October 31, 2017, and were followed up at Ondokuz Mayıs University were retrospectively reviewed. Patient demographics, AIHA type and secondary causes, direct Coombs subtypes, hemoglobin and lactate dehydrogenase levels at diagnosis and at postsplenectomy, bone marrow biopsy and splenectomy pathologies, liver and spleen size, splenectomy complications, treatments given presplenectomy, status after splenectomy, and relapse treatments were examined. Results: Twelve of 25 patients (48%) had primary AIHA and 13 (52%) had secondary AIHA. All patients received methylprednisolone before splenectomy. After splenectomy, 14 (56%) of the cases were in remission, while 11 (44%) relapsed. Of the patients who relapsed, steroids were used in 54.54%, rituximab in 18.18%, cyclosporine in 36.36%, intravenous immunoglobulin in 9.09%, and mycophenolate mofetil in 9.09%. One patient underwent plasmapheresis. At the end of all treatments, 6 (54.54%) patients who relapsed after splenectomy were in remission, while 3 (27.27%) relapsed and 2 (18.18%) were refractory. Four patients died. As a complication of splenectomy, sepsis developed in 4 patients (16%) and thrombotic events occured in 6 patients (24%). Conclusion: Some of the patients with AIHA did not respond to splenectomy. We think that the number of patients who need to undergo splenectomy will decrease with randomized trials using new generation drugs. [ABSTRACT FROM AUTHOR]

TEN like Lupus erythematosus is an uncommon life-threatening variant of Lupus erythematosus. It is usually associated with flares of systemic lupus erythematosus and also because of widespread skin erosions, it can cause acute skin failure. It is often confused with drug induced TEN, however the management of both the diseases is different and hence correct diagnosis becomes crucial. In this study we aimed to assess the clinical characteristics and outcome of TEN like LE in the Indian population. All patients satisfying ACR/EULAR 2019 criteria for SLE and clinically diagnosed with TEN like LE were retrospectively reviewed. A total of 6 patients were identified. All patients were female. Except 1 patient who presented de-novo, the others had pre-existing symptoms of connective tissue disease. Half of the patients had palmoplantar involvement. Mucosal involvement was only mild. Majority had systemic involvement in the form of nephritis followed by arthralgia, autoimmune hepatitis and autoimmune hemolytic anemia. [ABSTRACT FROM AUTHOR]

This article provides information on the characteristics and diagnosis of Wiskott-Aldrich syndrome (WAS), a primary immunodeficiency disorder. It highlights that while low mean platelet volume (MPV) is typical for WAS, normal MPV values can be observed in some patients at the time of diagnosis. The article presents case reports of infants with thrombocytopenia and normal MPV values, emphasizing the importance of accurate diagnosis and distinguishing WAS from other conditions. It also discusses the potential misdiagnosis of WAS in infants with rectal bleeding and eczema, particularly when cow's milk protein allergy (CMPA) is considered. The article concludes by emphasizing the need for further research and awareness of WAS to improve diagnosis and treatment outcomes. [Extracted from the article]

Autoimmune cytopenias are a heterogeneous group of disorders characterized by immune‐mediated destruction of haematopoietic cell lines. Effective and well‐tolerated treatment options for relapsed‐refractory immune cytopenias are limited. In this study, the aim was to evaluate the efficacy and safety of sirolimus in this disease group within the paediatric age group. The study enrolled patients in the paediatric age group who used sirolimus with a diagnosis of immune cytopenia between December 2010 and December 2020, followed at six centres in Turkey. Of the 17 patients, five (29.4%) were treated for autoimmune haemolytic anaemia (AIHA), six (35.2%) for immune thrombocytopenic purpura (ITP) and six (35.2%) for Evans syndrome (ES). The mean response time was 2.7 months (range, 0–9 months). Complete response (CR) and partial response (PR) were obtained in 13 of 17 patients (76.4%) and nonresponse (NR) in four patients (23.5%). Among the 13 patients who achieved CR, three of them were NR in the follow‐up and two of them had remission with low‐dose steroid and sirolimus. Thus, overall response rate (ORR) was achieved in 12 of 17 patients (70.5%). In conclusion, sirolimus may be an effective and safe option in paediatric patients with relapsed‐refractory immune cytopenia. [ABSTRACT FROM AUTHOR]

This case report is to help raise awareness of a rare autoimmune disorder, cold agglutinin disease (CAD), which is characterised by the premature destruction of red blood cells (haemolysis) (National Organisation for Rare Disorders [NORD], (2020). It can have a severe physical and psychological impact on those suffering with it. Caring for patients with CAD can also be extremely challenging and even traumatic for clinicians. The importance of good multidisciplinary team (MDT) working both within hospital and when discharging a patient to a community healthcare setting is also highlighted (NORD, 2020), as well as the importance of clear communication for the patient from medical staff on the expectations of treatment and prognosis. Approximately one person in 300,000 gets CAD, which is more prevalent in those aged 60 years and older. Women are also more likely to get it than men (Smith, 2021). [ABSTRACT FROM AUTHOR]

Various autoimmune diseases have been reported to develop as a result of a coronavirus disease 19 (COVID-19) infection. There have been some reports of COVID-19-triggered autoimmune hepatitis and autoimmune hemolytic anemia infection, but none have reported simultaneous onset of these diseases. A 15-year-old girl was admitted to our hospital with severe liver injury and anemia. Three weeks before admission, her father was diagnosed with COVID-19, after which she became aware of a sore throat. Two weeks later, she visited her doctor for malaise. She was referred to our hospital due to severe anemia, elevated hepatobiliary enzymes, and jaundice. A COVID-19 polymerase chain reaction test was positive at the time of referral. She was diagnosed with autoimmune hemolytic anemia based on decreased hemoglobin and haptoglobin, positive direct Coombs test, and increased urinary urobilinogen. Blood tests were positive for antinuclear antibodies, and a liver biopsy revealed interface hepatitis and plasma cell infiltration, consistent with autoimmune hepatitis. Based on these findings, a diagnosis of autoimmune hepatitis and autoimmune hemolytic anemia triggered by COVID-19 infection was made. Steroid therapy was initiated, which resulted in rapid improvement of blood markers and symptoms. [ABSTRACT FROM AUTHOR]