Klumpp T R, Block C C, Caligiuri M A, Rabinowe S N, Soiffer R J, Ritz J
Division of Tumor Immunology, Dana-Farber Cancer Institute, Boston, Massachusetts.
Medicine (Baltimore). 1992 Mar;71(2):73-83. doi: 10.1097/00005792-199203000-00002.
We describe 3 cases of immune-mediated cytopenia occurring after bone marrow transplantation (BMT). In 1 case, only the platelet line was affected, whereas in the other 2 cases more than 1 cell lineage was involved simultaneously. Two of the cases presented with falling peripheral blood counts following apparently normal early engraftment, while in 1 of the cases the affected lineage failed to appear in the peripheral blood despite normal engraftment of the other lineages. In all 3 cases the cytopenia improved following the initiation of treatment with systemic corticosteroids. Immune-mediated cytopenia following bone marrow transplantation may occur via alloimmune or autoimmune mechanisms. Alloimmune cytopenias have arisen in the context of major or minor mismatches in the ABO system, but cases related to mismatches in the Rh system and other erythroid and non-erythroid alloantigen systems may also occur. Alloimmune cytopenias have been reported primarily in the setting of allogeneic BMT, whereas autoimmune cytopenias have been reported following both allogeneic and autologous BMT. Immune-mediated cytopenia may present as early as the day of transplant, or as late as many months afterward. The possibility of immune-mediated cytopenia should always be considered when unexpected peripheral blood cytopenia is present, or when unexpected hemolysis develops, following bone marrow transplantation. The diagnosis is supported by a normal appearance of the affected lineage or lineages in the bone marrow, the absence of other apparent causes for the cytopenia, and the presence of the relevant auto- or allo-antibodies in the serum. However, any of these features may be absent in individual cases. The importance of these syndromes lies in the fact that they may be life-threatening, yet they often respond well to steroids or other standard immunosuppressive measures. It is important to be aware that effective prophylactic measures are available for patients receiving ABO- or Rh-incompatible marrow.
我们描述了3例骨髓移植（BMT）后发生的免疫介导性血细胞减少症。1例仅血小板系受累，而另外2例则同时有1种以上细胞系受累。其中2例在早期植入看似正常后外周血细胞计数下降，而另1例中尽管其他细胞系植入正常，但受累细胞系未出现在外周血中。所有3例在开始使用全身性皮质类固醇治疗后血细胞减少症均有改善。骨髓移植后的免疫介导性血细胞减少症可能通过同种免疫或自身免疫机制发生。同种免疫性血细胞减少症发生于ABO系统主要或次要不匹配的情况下，但与Rh系统及其他红细胞和非红细胞同种抗原系统不匹配相关的病例也可能出现。同种免疫性血细胞减少症主要报道于异基因BMT的情况下，而自身免疫性血细胞减少症在异基因和自体BMT后均有报道。免疫介导性血细胞减少症可早在移植当天出现，或晚至数月后出现。骨髓移植后出现意外的外周血细胞减少或意外的溶血时，应始终考虑免疫介导性血细胞减少症的可能性。骨髓中受累细胞系外观正常、血细胞减少无其他明显原因以及血清中存在相关的自身或同种抗体支持该诊断。然而，个别病例可能不存在这些特征中的任何一项。这些综合征的重要性在于它们可能危及生命，但通常对类固醇或其他标准免疫抑制措施反应良好。重要的是要意识到，对于接受ABO或Rh不相合骨髓的患者，有有效的预防措施。
