Pathology, The George Washington University School of Medicine and Health Sciences, Washington, District of Columbia, USA.
Department of Pathology, Brody School of Medicine, Greenville, NC, USA.
BMJ Case Rep. 2024 Feb 28;17(2):e251638. doi: 10.1136/bcr-2022-251638.
Cold agglutinins produced in the setting of B cell neoplasms, such as lymphoplasmacytic lymphoma and plasma cell myeloma, can mediate autoimmune haemolytic anemia. Transfusion of these patients can exacerbate cold agglutinin-mediated haemolysis. Moreover, the workup for these reactions represents a diagnostic challenge due in part to false negative direct antiglobulin tests (DATs). Here, we report an anaemic patient who after a red blood cell (RBC) transfusion performed without blood warming, experienced a DAT-negative haemolytic transfusion reaction, and was later diagnosed with IgA-multiple myeloma, which showed an uncommon granular pattern by CD138 immunohistochemistry. Extensive workup excluded other diagnostic possibilities, including the presence of Donath-Landsteiner antibodies and cryoglobulins. Successful treatment with CyBorD (cyclophosphamide, bortezomib and dexamethasone) achieved complete remission, and additional RBC transfusions using warmers were completed uneventfully.
冷自身抗体在 B 细胞肿瘤（如淋巴浆细胞淋巴瘤和浆细胞瘤骨髓瘤）的情况下产生，可介导自身免疫性溶血性贫血。这些患者的输血可能会加重冷自身抗体介导的溶血。此外，这些反应的检查代表了一个诊断挑战，部分原因是直接抗球蛋白试验（DAT）的假阴性。在这里，我们报告了一名贫血患者，他在未进行血液加温的情况下接受了红细胞（RBC）输血，随后发生了 DAT 阴性的溶血性输血反应，并随后被诊断为 IgA-多发性骨髓瘤，该疾病在 CD138 免疫组化中表现出不常见的颗粒状模式。广泛的检查排除了其他诊断可能性，包括存在 Donath-Landsteiner 抗体和冷球蛋白。用 CyBorD（环磷酰胺、硼替佐米和地塞米松）成功治疗后达到完全缓解，并且使用热合器的额外 RBC 输血也顺利完成。
