Tomonari A, Tojo A, Lseki T, Ooi J, Nagayama H, Ogami K, Maekawa T, Shirafuji N, Tani K, Asano S
Department of Hematology/Oncology, Institute of Medical Science, The University of Tokyo, Japan.
Int J Hematol. 2001 Aug;74(2):228-32. doi: 10.1007/BF02982010.
Autoimmune thrombocytopenia (AITP) after bone marrow transplantation (BMT) was suggested to occur by immune dysregulation mainly in association with graft-versus-host disease (GVHD). Here we present a patient who developed severe AITP after BMT. A 40-year-old woman with severe aplastic anemia received a BMT from a partially HLA-matched brother. Despite myeloid and erythroid engraftments, platelet recovery was delayed. All bone marrow cells were 46,XY and were derived from the donor. Grade I acute GVHD involving skin developed from day 34 posttransplantation, but promptly responded to prednisolone in addition to a prophylactic dose of tacrolimus. With the tapering of prednisolone, thrombocytopenia progressed without substantial changes in the white blood cell count, hemoglobin concentration, or reticulocyte count. On day 188, the patient developed chronic GVHD involving skin and liver, which promptly responded to the readministration of prednisolone and increased tacrolimus. However, the patient's platelet count decreased to 9 x 10(9) cells/L on day 222. The platelet-associated immunoglobulin G (PAIgG) values were elevated. Bone marrow examination showed hypercellularity with plentiful megakaryocytes. The number of colony-forming units-megakaryocyte was within the normal range. The elevated PAIgG values and a correlation between thrombocytopenia and the intensity of the immunosuppressive agents strongly suggested a causative role of the autoimmune mechanisms for thrombocytopenia in this patient.
骨髓移植(BMT)后发生的自身免疫性血小板减少症(AITP)被认为主要是由于免疫失调,主要与移植物抗宿主病(GVHD)相关。在此,我们报告一名在BMT后发生严重AITP的患者。一名40岁患有严重再生障碍性贫血的女性接受了来自部分HLA匹配的兄弟的BMT。尽管有髓系和红系植入,但血小板恢复延迟。所有骨髓细胞均为46,XY,且来源于供体。移植后第34天出现了累及皮肤的I级急性GVHD,但除预防性剂量的他克莫司外,对泼尼松龙反应迅速。随着泼尼松龙逐渐减量,血小板减少症进展,而白细胞计数、血红蛋白浓度或网织红细胞计数无明显变化。在第188天,患者出现了累及皮肤和肝脏的慢性GVHD,再次给予泼尼松龙并增加他克莫司剂量后反应迅速。然而,在第222天患者的血小板计数降至9×10⁹/L。血小板相关免疫球蛋白G(PAIgG)值升高。骨髓检查显示细胞增多,有大量巨核细胞。巨核细胞集落形成单位数量在正常范围内。PAIgG值升高以及血小板减少症与免疫抑制剂强度之间的相关性强烈提示自身免疫机制在该患者血小板减少症中起致病作用。