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Efficacy of HPA-1a (PlA1)-negative platelets in a patient with post-transfusion purpura
Alison Wakoff Loren1, Charles S Abrams
1Department of Medicine, Division of Hematology/Oncology, University of Pennsylvania Health System, 16 Penn Tower, 3400 Spruce Street, Philadelphia, PA 19104, USA. awakoff@mail.med.upenn.edu
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.
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.
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