Acquired Glanzmann's Thrombasthenia Associated with Hodgkin Lymphoma: Rapid Reversal of Functional Platelet Defect with Chemotherapy Despite Persistence of a Glycoprotein Iib/Iiia Auto-Antibody

• Published in: Blood Vol. 120; no. 21; p. 4656
• Main Authors: Raman, Vinod, Quillen, Karen, Sloan, John Mark
• Format: Journal Article
• Language: English
• Published: Elsevier Inc 16-11-2012
• ISSN: 0006-4971; 1528-0020
• DOI: 10.1182/blood.V120.21.4656.4656

Abstract 4656
Acquired Glanzmann's Thrombasthenia (GT) is a rare hemorrhagic disorder characterized by the impairment of platelet function caused by inhibition of GpIIb/IIIa signaling, often by an autoantibody. This disorder has been associated with lymphoproliferative and autoimmune disease.
A previously healthy 44 year old male presents with severe spontaneous nasal, gingival, and gastrointestinal bleeding with mildly elevated INR and aPTT and normal platelet count in the setting of newly diagnosed stage IVB classical Hodgkin lymphoma, Nodular Sclerosis type.
At presentation, platelet aggregation studies showed no aggregation with ADP, epinepherine, and arachidonic acid, with preserved ristocetin aggregation. An anti-platelet antibody was detected. Bone marrow aspiration showed megakaryocytes that were increased in number with occasional atypical forms. Patient was started on ABVD chemotherapy for Hodgkin lymphoma with immediate resolution of bleeding diathesis. Repeat platelet aggregation after cycle 2 of ABVD showed normalization of platelet aggregation. Despite the clinical resolution of bleeding tendency and functional platelet defect, a persistent anti-platelet antibody directed against GPIIb/IIIa receptor was detected.
Acquired Glanzmann thrombasthenia is a rare disorder of hemostasis associated with lymphoma and autoimmune conditions. The prompt reversal of the functional defect with chemotherapy in this patient, as well as the persistence of the GpIIb/IIIa antibody, suggest that there may be additional additional lymphoma-specific factors that modify the effect of the autoantibody.
No relevant conflicts of interest to declare.