A novel conditioning regimen with pre-transplantation immunosuppression reduces the complication rates in hematopoietic stem cell transplantation in transfusion-dependent β-thalassemia.

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.

"Bone marrow-liver-spleen-type diffuse large B-cell lymphoma" presenting with cold autoimmune hemolytic anemia: a case report.

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.

Emergency cholecystectomy: risk factors and impact of delay on electively booked patients, a 5-year experience of a tertiary care center.

Background: Gallstone disease is one of the most resource-intensive surgical conditions. Despite the significant burden of emergency cholecystectomy on healthcare system, there is lack of research assessing the risk factors predisposing scheduled elective cholecystectomy patients to emergency surgery. Characterization of patients with gallstones helps to prioritize delivery of health care to avoid urgent surgery.

Hemolytic disease of the fetus and newborn and Rhesus alloimmunization in Latin American countries: a scoping review.

Background: Hemolytic disease of the fetus and newborn (HDFN) is a condition due to maternal blood group antibodies targeting antigens in fetal red blood cells, with significant prenatal/perinatal morbidity and mortality. Severe HDFN cases are often associated with alloimmunization against Rhesus D (RhD) or Kell antigens. Information about HDFN epidemiology and treatment in Latin American countries is limited.

Cerebral Hemodynamic Responses to Disease-Modifying and Curative Sickle Cell Disease Therapies.

Background And Objectives: Sickle cell disease (SCD) is a hemoglobinopathy resulting in hemoglobin-S production, hemolytic anemia, and elevated stroke risk. Treatments include oral hydroxyurea, blood transfusions, and hematopoietic stem cell transplantation (HSCT). Our objective was to evaluate the neurologic relevance of these therapies by characterizing how treatment-induced changes in hemoglobin (Hb) affect brain health biomarkers.

Living with sickle cell in Uganda: A comprehensive perspective on challenges, coping strategies, and health interventions.

Sickle cell anemia (SCA) is a hereditary blood disorder with profound implications for affected individuals, particularly in resource-limited settings such as Uganda. This review explores the multifaceted aspects of SCA in Uganda, focusing on epidemiology, challenges faced by individuals, coping strategies, healthcare disparities, and community support. The study incorporates a thorough examination of the genetic landscape, prevalence, and the impact of SCA on the quality of life in Uganda.

Red blood cell agglutination caused by ceftriaxone and its effect on erythrocyte parameters: a case report.

Ceftriaxone, a widely used antibiotic, is one of the most common drugs to cause drug-induced immune hemolytic anemia. In this report, we describe the effect of ceftriaxone on red blood cell parameters (low red blood cell count, low hematocrit, and high erythrocyte index values) in two pediatric patients without clinical symptoms of hemolytic anemia. Although automated hematology analyzers have helped to detect incorrect results, a peripheral blood smear examination was necessary for recognizing the erythrocyte agglutinins caused by ceftriaxone.

Scientometrics Analysis of Global Researches on Anemia.

Anemia is a condition in which the number of red blood cells or the hemoglobin concentration within them is lower than normal. This study aims to show the intellectual structure of knowledge regarding anemia and gives a comprehensive and up-to-date image of research in this area. This is a descriptive-analytical study with a scientometric approach.

Hb Monza: A novel extensive HBB duplication with preserved α-β subunit interaction and unstable hemoglobin phenotype.

Background: Unstable hemoglobins are caused by single amino acid substitutions in the HBB gene, often affecting key histidine residues, leading to protein destabilization and hemolytic crises. In contrast, long HBB variants, exceeding 20 bp, are rare and associated with a β-thalassemia phenotype due to disrupted α-β chain interactions. We describe a family wherein four of six members carry a novel 23-amino-acid in-frame duplication of HBB (c.

Respectful care for postpartum women with sickle cell disease: a netnographic study.

Objectives: to analyze principles of respectful maternity care in narratives of postpartum women with sickle cell disease, relating them to Sustainable Development Goals.

Methods: netnographic study, with two videos published in 2020. Deductive iconographic and thematic analysis by Respectful Maternity Care Charter, organized in MAXQDA.

Sequential Use of Prednisolone and Cyclosporine Is Effective in the Management of Immunotherapy-Related Hemolytic Anemia.

Immune checkpoint inhibitors (CPIs) can cause immune-related organ dysfunctions, including nephritis, pneumonitis, thyroiditis, hepatitis, colitis and more rarely hematological toxicities like immune-related autoimmune hemolytic anemia (irAIHA). Very few cases of irAIHA associated with immunotherapy have been reported, and treatment protocols remain unclear. This is partly because not all irAIHA cases are Coomb's test positive.

A novel scoring system for the prediction of disease severity in STEC-HUS.

Background: Shiga toxin-producing Escherichia coli-associated hemolytic uremic syndrome (STEC-HUS) is a life-threatening condition complicated by acute kidney injury, acute respiratory distress syndrome, and central nervous system disorders. The early identification of high-risk patients is required to facilitate timely and appropriate treatment.

Methods: The medical records of patients with STEC-HUS treated at 11 hospitals in Hokkaido, Japan, were reviewed retrospectively.

Parvovirus B19 infection in children: a comprehensive review of clinical manifestations and management.

Parvovirus B19 (B19V) is a significant pathogen responsible for a wide range of clinical manifestations, particularly in children and pregnant women. While B19V is most commonly recognized as the cause of Fifth disease, a mild erythematous illness in children, its clinical impact extends far beyond this condition. B19V can lead to severe complications, including transient aplastic crisis in individuals with chronic hemolytic anemias, arthralgia, and more severe joint diseases.

A rare case report of severe aplastic anaemia caused by long-term use of zidovudine.

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.

Current status and trends in thalassemia burden across South, East and Southeast Asia, 1990-2021 a systematic analysis for the global burden of disease study 2021.

Objective: Thalassemia, an inherited hemoglobin synthesis disorder, imposes a significant health burden in Asian regions with high prevalence. Detailed patterns and trends of the disease across countries and territories within these regions remain underexplored. Our study focuses on the disease burden indices of thalassemia within the four GBD-defined Asian regions and the twenty-five included countries and territories.

Hydroxyurea blunts mitochondrial energy metabolism and osteoblast and osteoclast differentiation exacerbating trabecular bone loss in sickle cell mice.

Sickle cell disease (SCD) is a severe hematological disorder characterized by erythrocyte sickling that causes significant morbidity and mortality. Skeletal complications of SCD include a high incidence of bone loss, especially in vertebrae, leading to fragility fractures that contribute to disease burden. Whether hydroxyurea (HU), a front-line therapy for SCD ameliorates bone disease has not been established.

Etiology and outcomes of kidney-limited and systemic thrombotic microangiopathy.

The syndromes of thrombotic microangiopathy (TMA) are associated with acute kidney injury and end-stage kidney disease (ESKD). TMAs typically present with thrombocytopenia and microangiopathic hemolytic anemia (i.e.

Successful rituximab treatment in a seronegative rheumatoid arthritis patient with concurrent cold agglutinin syndrome and immune thrombocytopenia.

Cold agglutinin disease, a subtype of cold-type autoimmune hemolytic anemia, is referred to as cold agglutinin syndrome (CAS) when it develops secondary to other conditions. Immune thrombocytopenia (ITP) is an autoimmune disorder characterized by the peripheral destruction of platelets. While both CAS and ITP can occur in patients with rheumatoid arthritis (RA), their concurrent manifestation in a single patient has not been reported, leaving the optimal treatment strategy for such a complex case unclear.

Management of hemolytic transfusion reactions in a patient with chronic myelomonocytic leukemia and rare antibodies: A case report.

Delayed hemolytic transfusion reaction (DHTR) poses a significant challenge in patients receiving blood transfusions. This case report highlights the complexities of managing DHTR in a newly diagnosed chronic myelomonocytic leukemia (CMML) patient with clinically significant JKa and little c antibodies during induction chemotherapy. A 46-year-old woman with CMML-2 who presented for induction chemotherapy was found to have hemolytic anemia.

Onasemnogene Abeparvovec is Safe in Hemolytic Disease of the Newborn: A Case Report.

Spinal muscular atrophy (SMA) is a rare autosomal recessive genetic disease caused by Survival Motor Protein 1 () gene mutations. Classically divided into three types, SMA is characterized by hypotonia, weakness, and tongue fasciculation in the first 6 months of life in type 1, inability to walk and limb weakness in type 2, and failure to run with proximal weakness in type 3 SMA. With the advent of newborn screening, treating presymptomatic patients with Onasemnogene abeparvovec (OA) is the treatment of choice in some centers worldwide.

Evaluation of Three Mutations in Codon 385 of Glucose-6-Phosphate Dehydrogenase via Biochemical and In Silico Analysis.

Glucose-6-phosphate dehydrogenase (G6PD) deficiency is an enzymopathy that affects approximately 500 million people worldwide. A great number of mutations in the gene have been described. However, three class A G6PD variants known as G6PD Tomah (C385R), G6PD Kangnam (C385G), and G6PD Madrid (C385W) have been reported to be clinically important due to their associations with severe clinical manifestations such as hemolytic anemia.

Evidence for in vivo resistance against allopurinol in a dog infected with Leishmania infantum by reduction in copy numbers of the S-adenosylmethionine synthetase (METK) gene.

Background: In Europe, canine leishmaniasis is commonly caused by Leishmania infantum. Allopurinol is the main drug for long-term management of the disease, and clinical relapses of L. infantum infection treated with this drug are described.

Risky Remedy: Rasburicase-Induced Methemoglobinemia in Tumor Lysis Syndrome Complicated by G6PD Deficiency.

Tumor lysis syndrome (TLS) is a critical oncologic emergency characterized by metabolic disturbances resulting from rapid cancer cell lysis. Rasburicase, a recombinant urate oxidase, is the primary treatment for hyperuricemia in TLS but poses a risk for methemoglobinemia in patients with glucose 6-phosphate dehydrogenase (G6PD) deficiency. We present the case of a 59-year-old male with diffuse large B-cell lymphoma (DLBCL) who developed spontaneous TLS.

Stay Away From the Cold: An Unusual Case of Cold Agglutinin Disease Presenting as Recurrent Transient Ischemic Attacks.

Cold agglutinin disease (CAD) is a rare autoimmune hemolytic anemia caused by cold-reactive IgM antibodies leading to complement-mediated hemolysis. While CAD-associated venous thromboembolism is recognized, its role in arterial thromboembolic events, particularly ischemic stroke, is poorly defined. We report an 84-year-old woman who developed acute onset upper left extremity weakness following exposure to sub-zero temperatures.