Kroll H, Kiefel V, Mueller-Eckhardt C
Institut für Klinische Immunologie und Transfusionsmedizin, Justus-Liebig-Universität Giessen, BRD.
Beitr Infusionsther. 1992;30:403-7.
Posttransfusion purpura (PTP) is a rare transfusion reaction characterized by a severe bleeding tendency and thrombocytopenia which occurs approximately 1 week after transfusion of platelet-containing blood components in patients previously immunized against platelet alloantigens. 34 cases of PTP were studied. Our patients were all female with a mean age of 60.8 years (35-78 years, n = 32). The inciting blood components were whole blood (4) or red cell concentrates (28). The interval between transfusion and onset of purpura ranged from 2 to 14 days with a clear maximum at 7 and 8 days. In 11 of 13 patients (85%) transfusion was accompanied by febrile, nonhemolytic transfusion reactions. Hemorrhagic symptoms lasted 9.4 days (3-37 days, n = 16). The mean minimal platelet count was 7.1 x 10(3)/microliters [(0-28) x 10(3)/microliters, n = 29]. The platelet count rose to over 50 x 10(3)/microliters after 13.9 days (2-61 days, n = 26), over 100 x 10(3)/microliters after 17.0 days (3-75 days, n = 22). In 1 patient, PTP led to death due to intracranial hemorrhage. 22 patients were treated with corticosteroids, 20 patients with intravenous immunoglobulins (IVIG), 17 of these patients received both. Therapy with IVIG was successful in 14 of 19 patients, whereas platelet transfusions (n = 18) were not able to evaluate the platelet count. Serological analysis showed that antibodies against the HPA-1a antigen either alone (18), in combination with HLA antibodies (12) or with anti-HPA-5b (1) were responsible in 91.2%, while antibodies against other platelet antigens were rarely implicated. In elution experiments HPA-1a antibodies could be eluted from the autologous HPA-1a-negative platelets. We suppose that these antibodies had a pseudo-specificity and were involved in the destruction of the patients' own platelets.
输血后紫癜(PTP)是一种罕见的输血反应,其特征为严重的出血倾向和血小板减少,发生于先前已对血小板同种抗原致敏的患者输注含血小板血液成分后约1周。对34例输血后紫癜患者进行了研究。我们的患者均为女性,平均年龄60.8岁(35 - 78岁,n = 32)。引发紫癜的血液成分是全血(4例)或红细胞浓缩液(28例)。输血与紫癜发作之间的间隔时间为2至14天,在第7天和第8天出现明显峰值。13例患者中有11例(85%)输血时伴有发热性非溶血性输血反应。出血症状持续9.4天(3 - 37天,n = 16)。平均最低血小板计数为7.1×10³/微升[(0 - 28)×10³/微升,n = 29]。血小板计数在13.9天后(2 - 61天,n = 26)升至50×10³/微升以上,在17.0天后(3 - 75天,n = 22)升至100×10³/微升以上。1例患者因颅内出血导致输血后紫癜死亡。22例患者接受了皮质类固醇治疗,20例患者接受了静脉注射免疫球蛋白(IVIG)治疗,其中17例患者两者均接受了治疗。IVIG治疗在19例患者中的14例取得成功,而血小板输注(n = 18)未能使血小板计数升高。血清学分析表明,单独针对HPA - 1a抗原的抗体(18例)、与HLA抗体联合(12例)或与抗HPA - 5b联合(1例)导致了91.2%的病例,而针对其他血小板抗原的抗体很少涉及。在洗脱实验中,HPA - 1a抗体可从自身HPA - 1a阴性血小板中洗脱出来。我们推测这些抗体具有假特异性,并参与了患者自身血小板的破坏。