Shtalrid Mordechai, Shvidel Lev, Vorst Eljakim, Weinmann Eran E, Berrebi Alain, Sigler Erica
Hematology Institute, Kaplan Medical Center, Rehovot, Israel.
Isr Med Assoc J. 2006 Oct;8(10):672-4.
Post-transfusion purpura is a rare syndrome characterized by severe thrombocytopenia and bleeding caused by alloimunization to human platelet specific antigens following a blood component transfusion. The suggested incidence is 1:50,000-100,000 transfusions, most often occurring in multiparous women. The diagnosis is not easy because these patients, who are often critically ill or post-surgery, have alternative explanations for thrombocytopenia such as infection, drugs, etc.
To describe patients with initially misdiagnosed PTP and to emphasize the diagnostic pitfalls of this disorder.
During a period of 11 years we diagnosed six patients with PTP, four women and two men. The incidence of PTP was approximately 1:24,000 blood components transfused. We present the detailed clinical course of three of the six patients in whom the diagnosis was particularly challenging. The patients were initially misdiagnosed as having heparin-induced thrombocytopenia, systemic lupus erythematosus complicated by autoimmune thrombocytopenia, and disseminated intravascular coagulation. A history of recent blood transfusion raised the suspicion of PTP and the diagnosis was confirmed by appropriate laboratory workup.
PTP seems to be more frequent than previously described. The diagnosis should be considered in the evaluation of life-threatening thrombocytopenia in both men and women with a recent history of blood transfusion.
输血后紫癜是一种罕见的综合征,其特征为严重血小板减少和出血,这是在输注血液成分后针对人类血小板特异性抗原发生同种免疫所致。建议的发病率为每50,000 - 100,000次输血中发生1例,最常发生于经产妇。诊断并不容易,因为这些患者通常病情危重或处于术后状态,对于血小板减少有诸如感染、药物等其他解释。
描述最初被误诊为输血后紫癜的患者,并强调该疾病的诊断陷阱。
在11年期间,我们诊断出6例输血后紫癜患者,4名女性和2名男性。输血后紫癜的发病率约为每24,000次输注血液成分中发生1例。我们呈现了6例患者中3例的详细临床病程,这3例患者的诊断尤其具有挑战性。这些患者最初被误诊为肝素诱导的血小板减少症、系统性红斑狼疮合并自身免疫性血小板减少症以及弥散性血管内凝血。近期输血史引发了对输血后紫癜的怀疑,通过适当的实验室检查确诊。
输血后紫癜似乎比先前描述的更为常见。在评估近期有输血史的男性和女性危及生命的血小板减少症时应考虑该诊断。