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T细胞去除的异基因骨髓移植后发生的自身免疫性溶血性贫血。

Autoimmune hemolytic anemia following T cell-depleted allogeneic bone marrow transplantation.

作者信息

Drobyski W R, Potluri J, Sauer D, Gottschall J L

机构信息

Department of Medicine, Medical College of Wisconsin Milwaukee, USA.

出版信息

Bone Marrow Transplant. 1996 Jun;17(6):1093-9.

PMID:8807120
Abstract

The development of immune-mediated hemolytic anemia is a well-recognized complication after allogeneic bone marrow transplantation (BMT). The majority of reported cases, however, have been alloimmune in origin due to ABO or minor red blood cell antigen incompatibilities between the donor and recipient. In this study, we report seven adult patients who developed autoimmune hemolytic anemia (AIHA) between June 1985 and January 1993. These patients were identified from a total of 236 adult patients who received T cell-depleted (TCD) grafts as graft-versus-host disease (GVHD) prophylaxis. The onset of AIHA was at a median of 10 months (range 7-25 months) post-transplant and occurred in 5% of all patients transplanted with TCD grafts who survived at least 6 months. Six patients had a warm reacting autoantibody, while one patient had a cold-reacting antibody with a thermal amplitude up to 30 degrees C. All were receiving immunosuppressive treatment for GVHD at the time of diagnosis. Initial treatment in all patients consisted of steroids. Three of the seven had a partial response while the four remaining patients failed to respond to corticosteroids. Splenectomy was performed in three patients with two partial responses. Four patients were treated with additional therapeutic interventions, including plasmapheresis, immunoglobulin infusions, staphylococcus protein A column, or other immunosuppressive agents. In five cases, erythropoietin was administered as adjunctive treatment to maintain adequate hematocrit levels. Two patients are presently in complete remission after prolonged courses of steroids, while a third patient has compensated hemolysis requiring low-dose steroids. Four patients died due to either infectious complications or disseminated intravascular coagulation secondary to cold agglutinin disease. These data indicate that AIHA is a clinically significant and not infrequent complication in allogeneic marrow transplant recipients. The response to conventional treatment is generally unsatisfactory as even patients who ultimately remit require prolonged courses of immunosuppressive therapy.

摘要

免疫介导的溶血性贫血是异基因骨髓移植(BMT)后一种公认的并发症。然而,大多数报道的病例起源于同种免疫,原因是供体和受体之间存在ABO血型或次要红细胞抗原不相容。在本研究中,我们报告了1985年6月至1993年1月期间发生自身免疫性溶血性贫血(AIHA)的7例成年患者。这些患者是从总共236例接受去除T细胞(TCD)移植物以预防移植物抗宿主病(GVHD)的成年患者中识别出来的。AIHA的发病中位时间为移植后10个月(范围7 - 25个月),发生在所有接受TCD移植物且存活至少6个月的患者中的5%。6例患者有温反应自身抗体,而1例患者有冷反应抗体,热幅度高达30℃。所有患者在诊断时均因GVHD接受免疫抑制治疗。所有患者的初始治疗均为使用类固醇。7例患者中有3例部分缓解,其余4例对皮质类固醇治疗无效。3例患者接受了脾切除术,其中2例部分缓解。4例患者接受了额外的治疗干预,包括血浆置换、免疫球蛋白输注、葡萄球菌蛋白A柱或其他免疫抑制剂。5例患者使用促红细胞生成素作为辅助治疗以维持足够的血细胞比容水平。2例患者在长时间使用类固醇治疗后目前完全缓解,而第3例患者有代偿性溶血,需要低剂量类固醇治疗。4例患者死于感染性并发症或冷凝集素病继发的弥散性血管内凝血。这些数据表明,AIHA在异基因骨髓移植受者中是一种具有临床意义且并非罕见的并发症。对传统治疗的反应通常不令人满意,因为即使最终缓解的患者也需要长时间的免疫抑制治疗疗程。

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