Autoimmune cytopenias (AIC) following allogeneic hematopoietic stem cell transplantation (HSCT) may cause significant morbidity and mortality and are often challenging to treat. We present a case of a pediatric patient with primary myelofibrosis of infancy caused by VPS45 protein deficiency, who developed severe refractory hemolytic anemia and immune-mediated thrombocytopenia 3.5 months following HSCT. After the failure of several treatments, he received daratumumab, an anti-CD38 specific antibody, and demonstrated fast and sustained response. The only side effect was delayed recovery of humoral immunity. Daratumumab, by targeting antibody-producing plasma cells, may be a valid treatment option for refractory post-HSCT AIC.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1002/pbc.28010 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Severe childhood SLE with refractory autoimmune hemolytic anemia-a therapeutic challenge.
BMJ Case Rep
January 2025
Dermatology, Venereology and Leprosy, AIIMS Rishikesh, Rishikesh, Uttarakhand, India
Paediatric Systemic lupus erythematosus (SLE) constitutes 10 to 20% of cases of SLE with more severe disease and higher mortality. We report a case of an adolescent girl with SLE with multisystem involvement who was started on hydroxychloroquine and oral prednisolone. However, due to persistent worsening of skin lesions and falling cell counts, pulsed dexamethasone was initiated which showed improvement in the skin lesions, cell counts, proteinuria and pleural effusion but there was a persistent fall in the haemoglobin.
View Article and Find Full Text PDFA retrospective analysis of autoimmune hemolytic anemia after allogeneic hematopoietic stem cell transplantation for transfusion-dependent β-thalassemia: focus on T and B lymphocyte reconstitution.
Ann Hematol
January 2025
Department of Hematology, The First Affiliated Hospital of Wenzhou Medical University, Wenzhou, 325035, China.
Background: Autoimmune hemolytic anemia (AIHA) following allogeneic hematopoietic stem cell transplantation (allo-HSCT) is often refractory and relapsing, leading to increased mortality post-HSCT.
Methods: We retrospectively analyzed the cases of patients with transfusion-dependent β-thalassemia (TDT) who underwent allo-HSCT to study their clinical features, the occurrence of AIHA post-HSCT, and treatment response and to explore the possible pathogenesis of AIHA.
Result: A total of 113 patients were registered in the study, out of whom 14 developed AIHA following allo-HSCT, resulting in a cumulative incidence of 12.
Successful Management of Severe and Refractory Autoimmune Hemolytic Anemia (AIHA) in a Sickle Cell Disease Patient With Bortezomib.
Cureus
November 2024
Hematology and Medical Oncology, Al-Zahraa Medical College, Basrah, IRQ.
Autoimmune hemolytic anemia (AIHA) is a multifactorial disease that causes immune-mediated red blood cell destruction, resulting in anemia and hemolysis symptoms. Despite a significant understanding of its pathogenesis, the precise causes of AIHA remain largely unclear and are thought to be multifactorial. In this paper, we presented a case of sickle cell anemia who developed severe AIHA that failed to maintain response to multiple treatment lines, including steroids, intravenous immunoglobulin, rituximab, and immune suppressive medications.
View Article and Find Full Text PDFRituximab Plus Bortezomib for Relapsed and Refractory Warm Autoimmune Hemolytic Anemia: A Prospective Phase 2 Trial.
Am J Hematol
February 2025
Department of Hematology, Peking Union Medical College Hospital and Chinese Academy of Medical Sciences, Beijing, China.
Article Synopsis
- Bacterial infections like V. cholerae are a growing public health issue, particularly in areas with poor sanitation and unsafe drinking water, leading to serious complications like septicemic shock.
- A recent case in Bangladesh involved a 30-year-old man with a history of beta-thalassemia who died from severe complications linked to V. cholerae bacteremia, highlighting the pathogen's deadly potential beyond cholera.
- This case is significant as it provides the first detailed molecular analysis of fatal V. cholerae bacteremia in Bangladesh, emphasizing the urgent need for better investigations into bacterial septicemia to avoid future fatalities.
Want AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!