Acquired Amegakaryocytic Thrombocytopenia with Glycoprotein IIb/IIIa Antibody in a Patient with Mantle Cell Lymphoma

• Published in: Internal Medicine Vol. 64; no. 8; pp. 1229 - 1234
• Main Authors: Sato, Keijiro, Ueki, Toshimitsu, Tokutake, Takayoshi, Ueno, Mayumi, Ichikawa, Naoaki, Sumi, Masahiko, Kobayashi, Hikaru
• Format: Journal Article
• Language: English
• Published: Japan The Japanese Society of Internal Medicine 15.04.2025; Japan Science and Technology Agency
• Subjects: Aged; amegakaryocytic thrombocytopenia; anti-glycoprotein IIb/IIIa antibody; Autoantibodies - blood; Autoimmune diseases; Bone Marrow Diseases; Case Report; Chemotherapy; Corticosteroids; Hemopoiesis; Humans; Hypoplasia; Idiopathic thrombocytopenic purpura; immune thrombocytopenia; Lymphoma, Mantle-Cell - complications; Lymphoma, Mantle-Cell - diagnosis; Lymphoma, Mantle-Cell - drug therapy; Lymphoma, Mantle-Cell - immunology; Male; malignant lymphoma; Mantle cell lymphoma; Platelet Glycoprotein GPIIb-IIIa Complex - immunology; Platelets; Purpura, Thrombocytopenic; Purpura, Thrombocytopenic, Idiopathic - diagnosis; Rituximab; Thrombocytopenia - blood; Thrombocytopenia - complications; Thrombocytopenia - diagnosis; Thrombocytopenia - immunology; Thrombopoietin; amegakaryocytic thrombocytopenia; anti-glycoprotein IIb/IIIa antibody; malignant lymphoma; immune thrombocytopenia
• ISSN: 0918-2918; 1349-7235; 1349-7235
• DOI: 10.2169/internalmedicine.4016-24

Acquired amegakaryocytic thrombocytopenia (AAMT) is a rare disorder characterized by thrombocytopenia, marked megakaryocytic hypoplasia, and preserved other-lineage hematopoiesis in the bone marrow. The etiology of AAMT remains poorly understood owing to its rarity. We encountered a diagnostically challenging case involving a 66-year-old man who showed severe thrombocytopenic bleeding with isolated megakaryocytic hypoplasia, elevated serum thrombopoietin levels, glycoprotein IIb/IIIa antibody positivity, and prolonged platelet transfusion refractoriness following mantle cell lymphoma (MCL). Treatment with corticosteroids and intravenous immunoglobulin was ineffective, while a combination of multiagent chemotherapy, including rituximab, was beneficial for both thrombocytopenia and MCL. Ultimately, the patient was diagnosed with AAMT and immune thrombocytopenia (ITP)-like platelet destruction. This case suggests that AAMT and ITP are non-exclusive and sometimes overlap as components of a broad spectrum of platelet-related autoimmune diseases.