ABSTRACT Background Haploidentical hematopoietic stem cell transplantation (haplo‐HSCT) is a curative therapeutic modality for hematological disorders, yet cold agglutinin syndrome (CAS) following haplo‐HSCT is exceedingly rare in the literature, leading to great diagnostic and therapeutic challenges in clinical practice. This study aimed to characterize the clinical manifestations, laboratory features, treatment strategies, and outcomes of post‐haplo‐HSCT CAS, and to provide clinical evidence for its early diagnosis and management. Methods We analyzed the data of all four patients with post‐haplo‐HSCT CAS at Peking University People's Hospital (2019–2024). Clinical data, including demographics, baseline characteristics, clinical manifestations, laboratory findings, therapeutic interventions, and follow‐up outcomes, were systematically collected and analyzed from electronic medical records. Results All four patients with CAS following haplo‐HSCT were male. CAS occurred at a median of 3.65 months post‐haplo‐HSCT (range: 1.9–9.7 months). All patients presented with severe hemolytic anemia (median Hb: 49.5 g/L, range: 22–59 g/L) and typical hemolytic laboratory findings, with no cold‐induced symptoms (acrocyanosis/Raynaud's phenomenon) observed. Three patients had concurrent infections, and two had underlying disease relapse/progression. Rituximab‐based regimens combined with supportive care achieved treatment response in three patients, with one patient showing no response. Two patients died from severe infections, and no CAS relapse was observed in all the survivors. Conclusions Post‐haplo‐HSCT CAS presents atypically without cold symptoms, is often triggered by infections or disease relapse amid immune dysregulation, and responds to rituximab‐based therapies. Clinicians should suspect CAS in haplo‐HSCT recipients with unexplained hemolytic anemia for prompt intervention. Trial Registration : The authors have confirmed clinical trial registration is not needed for this submission
An et al. (Fri,) studied this question.
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