Autoimmune hemolytic anemia (AIHA) is a rare complication of ABO-matched hematopoietic stem cell transplantation (HSCT). Post-HSCT AIHA is diagnosed by a positive hemolysis laboratory work-up and positive direct antiglobulin testing with usually a pan-reactive antibody in indirect antiglobulin testing. In this case report, we describe the diagnosis of post-HSCT AIHA in a 62-year-old male patient and its management with combined immunosuppressive therapy with steroids, rituximab, and intravenous immunoglobulin to achieve transfusion independence and disease remission. Post-HSCT AIHA is generally refractory to treatment but this case highlights the role of combination immunosuppressive therapies to maximize the likelihood of transfusion independence.
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Case Report of an ABO-Matched Post-Hematopoietic Stem Cell Transplant-Associated Autoimmune Hemolytic Anemia: A Diagnostic and Therapeutic Challenge.
Transplant Proc
February 2025
Division of Hematology, Department of Medicine, McGill University Health Centre, McGill University, Montreal, QC.
Autoimmune hemolytic anemia (AIHA) is a rare complication of ABO-matched hematopoietic stem cell transplantation (HSCT). Post-HSCT AIHA is diagnosed by a positive hemolysis laboratory work-up and positive direct antiglobulin testing with usually a pan-reactive antibody in indirect antiglobulin testing. In this case report, we describe the diagnosis of post-HSCT AIHA in a 62-year-old male patient and its management with combined immunosuppressive therapy with steroids, rituximab, and intravenous immunoglobulin to achieve transfusion independence and disease remission.
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.
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Sezione di Ematologia, Dipartimento di Scienze Radiologiche ed Ematologiche, Università Cattolica del Sacro Cuore, Rome, Italy.
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.
View Article and Find Full Text PDFPersistent Hypogammaglobulinemia after Receiving Rituximab Post-HSCT Is Not Caused by an Intrinsic B Cell Defect.
Int J Mol Sci
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Laboratory for Pediatric Immunology, Willem-Alexander Children's Hospital, Leiden University Medical Center, 2333 ZA Leiden, The Netherlands.
In the setting of hematopoietic stem cell transplantation (HSCT), Rituximab (RTX) is used for the treatment and prevention of EBV-associated post-transplantation lymphoproliferative disease or autoimmune phenomena such as autoimmune hemolytic anemia (AIHA). Persistent hypogammaglobulinemia and immunoglobulin substitution dependence has been observed in several patients after RTX treatment despite the normalization of total B cell numbers. We aimed to study whether this is a B cell intrinsic phenomenon.
View Article and Find Full Text PDFRecent studies have identified autoimmune haemolytic anaemia (AIHA) as a haematopoietic stem cell transplant (HSCT) complication that represents a significant cause of morbidity and mortality for these patients. In order to understand this autoimmune phenomenon, emerging research has focused on the prognostic factors associated with the development of the disorder. These studies have identified numerous possible associations with often contrasting and conflicting results.
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