Acquired Glanzmann's thrombasthenia associated with Hodgkin's lymphoma : A case report and review of the literature

• Published in: Cancer 1998-05, Vol.82 (9), p.1764-1768
• Main Authors: MALIK, U, DUTCHER, J. P, OLEKSOWICZ, L
• Format: Article
• Language: English
• Subjects: Antibodies, Neoplasm - immunology; Autoantibodies - immunology; Biological and medical sciences; Blood Platelets - physiology; Female; Hematologic and hematopoietic diseases; Hodgkin Disease - blood; Hodgkin Disease - complications; Humans; Immunoglobulin M - immunology; Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis; Medical sciences; Middle Aged; Thrombasthenia - blood; Thrombasthenia - etiology
• ISSN: 0008-543X; 1097-0142
• DOI: 10.1002/(SICI)1097-0142(19980501)82:9<1769::AID-CNCR25>3.0.CO;2-1

Acquired Glanzmann's thrombasthenia is a rare hemorrhagic diathesis resulting from impaired adhesive function of the platelet receptor GPIIb/IIIa (alpha(IIb)beta3). Typically, this disorder develops during adulthood, with patients manifesting fluctuating clinical and laboratory findings. To date, the underlying defect of most if not all cases of acquired Glanzmann's thrombasthenia results from an autoantibody or plasma protein inhibitor directed toward a demonstrably normal GPIIb/IIIa glycoprotein. In this report, a patient with a history of treated Hodgkin's lymphoma presented with a severe hemorrhagic diathesis characterized by mild thrombocytopenia, a prolonged bleeding time, and defective platelet aggregation. Examination of the patient's platelet GPIIb/IIIa by Western blot analysis revealed no abnormality. Mixing studies demonstrated a non-immunoglobulin G plasma inhibitory factor, whereas flow cytometry analysis revealed elevated platelet-associated immunoglobulin (Ig) M. After an emergency colectomy for severe hemorrhage, the patient's qualitative and quantitative platelet parameters significantly improved. Pathology of the resected colonic segment demonstrated atypical lymphoid hyperplastic lesions. To the authors' knowledge, this is the first reported case of acquired Glanzmann's thrombasthenia associated with a putative IgM autoantibody. Furthermore, this report verifies the association of acquired thrombasthenia with lymphoproliferative disease. Although rare, awareness of this hemorrhagic diathesis as a possible sequelae of active or treated lymphoid disorders should encourage clinical vigilance of these patients.