Acquired Glanzmann's thrombasthenia is an uncommon event in association with leukemia. The authors describe a patient with acute lymphoblastic leukemia (ALL) who presented with severe hemorrhagic syndrome, without disseminated intravascular coagulation. The patient's course was complicated by the occurrence of severe hemorrhagic episodes, with a thrombasthenia-like profile, requiring multiple transfusions with packed red cells, platelets, and fresh-frozen plasma. Biological explorations detected anti-GPIIb/IIIa complex antibodies. The patient finally died with refractory disease and persistent bleeding. This case is the first reported of autoantibodies to GPIIb/IIIa in ALL. Such paraneoplastic syndrome is potentially responsible for severe life-threatening hemorrhage.
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Article Synopsis
- rFVIIa is a human recombinant activated coagulation factor VII used for over two decades to treat and prevent bleeding in various bleeding disorders, including congenital and acquired hemophilia, congenital factor VII deficiency, and Glanzmann thrombasthenia.
- Its dosage, administration, and indications vary between the US, Europe, and Japan based on patient needs and regulatory practices.
- Despite the emergence of new therapies like emicizumab for hemophilia A, rFVIIa remains crucial for managing bleeding episodes, especially in surgical situations and when other treatments fail.
Introduction Glanzmann's thrombasthenia (GT) is an autosomal recessive disorder of platelets caused by a deficiency in the glycoprotein IIb-IIIa. Bleeding from the skin, mucous membranes, and ecchymosis are symptoms manifested starting in early childhood. There may also be major bleeding conditions as a result of surgical procedures or trauma.
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