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Successful treatment of amegakaryocytic thrombocytopenia with anti-CD20 antibody (rituximab) in a patient with systemic lupus erythematosus

Division of Hematology and Immunology, Department of Internal Medicine, Kanazawa Medical University, Kahoku-gun, Ishikawa, Japan

L Dong

Division of Hematology and Immunology, Department of Internal Medicine, Kanazawa Medical University, Kahoku-gun, Ishikawa, Japan; Department of Hematology and Immunology, Tongji Hospital, Huazhong University of Science and Technology, Wuhan, Hubei, China

T Sakai

Division of Hematology and Immunology, Department of Internal Medicine, Kanazawa Medical University, Kahoku-gun, Ishikawa, Japan

T Sawaki

Division of Hematology and Immunology, Department of Internal Medicine, Kanazawa Medical University, Kahoku-gun, Ishikawa, Japan

M Miki

Division of Hematology and Immunology, Department of Internal Medicine, Kanazawa Medical University, Kahoku-gun, Ishikawa, Japan

M Tanaka

Division of Hematology and Immunology, Department of Internal Medicine, Kanazawa Medical University, Kahoku-gun, Ishikawa, Japan

Y Masaki

Division of Hematology and Immunology, Department of Internal Medicine, Kanazawa Medical University, Kahoku-gun, Ishikawa, Japan

Y Hirose

Division of Hematology and Immunology, Department of Internal Medicine, Kanazawa Medical University, Kahoku-gun, Ishikawa, Japan

M Kuwana

Department of Internal Medicine, Keio University School of Medicine, Shijuku-ku, Tokyo, Japan

H Umehara

Division of Hematology and Immunology, Department of Internal Medicine, Kanazawa Medical University, Kahoku-gun, Ishikawa, Japan

Abstract

Amegakaryocytic thrombocytopenia is an extremely rare disorder in systemic lupus erythematosus, and its mechanism and treatment are still largely unknown. We describe a 42-year-old woman with systemic lupus erythematosus who presented various clinical manifestations of life-threatening amegakaryocytic thrombocytopenia (10,000 platelets/mm³ with a marked decrease of megakaryocytes in the bone marrow), proteinuria, psychosis, refractory chylothorax, ascites, and type II diabetes caused by the anti-insulin receptor autoantibody. She was initially treated with prednisolone (25–50�mg/day) and cyclosporine A (200�mg/day) without any improvement in severe thrombocytopenia. However, her clinical symptoms, including platelet counts, dramatically improved, with a concurrent decrease in the anti-c-Mpl antibody, an autoantibody against the thrombopoietin receptor, after a subsequent treatment with rituximab (375�mg/m² intravenously, weekly, for two consecutive weeks). Our case suggested that amegakaryocytic thrombocytopenia in patients with systemic lupus erythematosus might be mediated by the anti-c-Mpl antibody and could be treated with rituximab through elimination of pathogenic B cells producing autoimmune antibodies.

Key Words: amegakaryocytic thrombocytopenia • rituximab (anti-CD20 mAb) • SLE

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