Loren Alison Wakoff, Abrams Charles S
Department of Medicine, Division of Hematology/Oncology, University of Pennsylvania Health System, 16 Penn Tower, 3400 Spruce Street, Philadelphia, PA 19104, USA.
Am J Hematol. 2004 Jul;76(3):258-62. doi: 10.1002/ajh.20093.
Post-transfusion purpura (PTP) is a rare form of alloimmune thrombocytopenia that is self-limited but which carries a 10-15% mortality related to fatal hemorrhage. Immunomodulatory therapies such as plasmapheresis and intravenous immunoglobulin G (IVIg) can shorten the duration of thrombocytopenia. However, in a bleeding patient with PTP, more urgent therapy may be required. Textbooks of hematology [1-3] as well as reports in the literature [4,5] suggest that patients do not respond to platelet transfusions. We report a case of PTP in a patient homozygous for HPA-1b who suffered an intracranial hemorrhage. The patient was treated with IVIg and plasmapheresis. Because of her life-threatening bleeding, we also transfused the patient with HPA-1a-negative platelets. These transfusions consistently resulted in transient improvements in her platelet counts and may have limited the degree of intracranial bleeding. Our experience suggests that transfusion of platelets that lack the offending epitope in patients with PTP may be efficacious.
输血后紫癜（PTP）是一种罕见的同种免疫性血小板减少症，具有自限性，但因致命性出血导致的死亡率为10% - 15%。诸如血浆置换和静脉注射免疫球蛋白G（IVIg）等免疫调节疗法可缩短血小板减少的持续时间。然而，对于出血的PTP患者，可能需要更紧急的治疗。血液学教科书[1 - 3]以及文献报道[4,5]表明，患者对血小板输注无反应。我们报告一例HPA - 1b纯合子的PTP患者发生颅内出血的病例。该患者接受了IVIg和血浆置换治疗。由于其出血危及生命，我们还为该患者输注了HPA - 1a阴性血小板。这些输注持续导致其血小板计数短暂改善，且可能限制了颅内出血的程度。我们的经验表明，为PTP患者输注缺乏致病表位的血小板可能有效。
