Kroll H, Kiefel V, Mueller-Eckhardt C
Institut für Klinische Immunologie und Transfusionsmedizin, Justus-Liebig-Universität Giessen.
Infusionsther Transfusionsmed. 1993 Oct;20(5):198-204.
Posttransfusion purpura (PTP) is characterized by severe thrombocytopenia and hemorrhagic diathesis about 1 week following blood transfusion. Only few studies exceed the description of single cases.
Clinical data from 38 patients were analyzed. Platelet antibodies were studied by complement binding, immunofluorescence, MAIPA assay and acid elution techniques.
All patients were female. The mean age at onset was 60.7 years (35-78 years). 4 patients received whole blood, 28 were transfused with packed RBC. 11 of 13 patients (85%) had febrile, non hemolytic adverse reactions during the transfusion. The interval between transfusion and onset of purpura extended from 2 to 14 days, with a peak at 7 and 8 days. Hemorrhagic symptoms lasted 10.1 days. The minimal platelet count was 7.0 x 10(3)/microliters. The platelet count increased to over 50 x 10(3)/microliters after 13.9 days (n = 26), and to over 100 x 10(3)/microliters after 17.0 days (n = 22). 2 patients died of hemorrhagic complications. 24 patients were treated with glucocorticoids, 20 with intravenous immunoglobulins (ivIG), 17 of these received both therapies. 14 of 19 patients (74%) responded well to ivIG. In contrast, platelet transfusions produced no adequate increment, in 6 cases they provoked febrile reactions. 35 sera (92.1%) contained anti-Zwa, either alone or together with anti-HLA or in 1 case with anti-Bra. Anti-Baka and anti-Bakb were found in 1 case each. In 5 patients, anti-Zwa could be eluted from autologous Zwa-negative platelets.
The broad clinical spectrum of PTP could be shown. Since 5% of the patients succumb to bleeding complications, the application of ivIG as therapy of choice is recommended. The phenomenon of elution of alloantibodies from autologous platelets is interpreted as a consequence of 'pseudo-specificity' which may play a role in the pathogenesis of PTP.
输血后紫癜(PTP)的特点是在输血后约1周出现严重血小板减少和出血倾向。仅有少数研究超出了对单个病例的描述。
分析了38例患者的临床资料。通过补体结合、免疫荧光、单克隆抗体特异性血小板抗原固定试验(MAIPA)和酸洗脱技术研究血小板抗体。
所有患者均为女性。发病时的平均年龄为60.7岁(35 - 78岁)。4例患者输注全血,28例输注红细胞悬液。13例患者中有11例(85%)在输血期间出现发热性非溶血性不良反应。输血与紫癜发作的间隔时间为2至14天,高峰在第7天和第8天。出血症状持续10.1天。血小板最低计数为7.0×10³/微升。13.9天后26例患者的血小板计数升至50×10³/微升以上,17.0天后22例患者的血小板计数升至100×10³/微升以上。2例患者死于出血并发症。24例患者接受糖皮质激素治疗,20例接受静脉注射免疫球蛋白(ivIG)治疗,其中17例同时接受两种治疗。19例患者中有14例(74%)对ivIG反应良好。相比之下,血小板输注未能使血小板计数充分增加,6例患者出现发热反应。35份血清(92.1%)含有抗Zwa,单独存在或与抗HLA同时存在,1例同时含有抗Bra。各有1例发现抗Baka和抗Bakb。5例患者的自身Zwa阴性血小板可洗脱抗Zwa。
展示了PTP广泛的临床谱。由于5%的患者死于出血并发症,建议应用ivIG作为首选治疗方法。同种抗体从自身血小板洗脱的现象被解释为“假特异性”的结果,这可能在PTP的发病机制中起作用。