title: Tocilizumab is effective for pulmonary hypertension associated with multicentric Castleman's disease.
abstract: A 38-year-old man, diagnosed as having multicentric Castleman's disease (plasma cell type) in 1995, had been treated with melphalan and prednisolone or prednisolone alone, but there was no remarkable response. In 2002, he was admitted to our hospital with a chief complaint of increasing dyspnea on effort. Laboratory data showed high serum IgG (10050 mg/dl), interleukin-6 (37.9 ng/ml), and vascular endothelial growth factor (VEGF 1920 pg/ml) levels. In addition, serum viscosity was very high (6.0 cp). Electrocardiogram, echocardiogram, and cardiac catheterization demonstrated pulmonary hypertension (PH). There were no other demonstrable causes of PH suggesting that PH was due to hyperviscosity syndrome and high VEGF level. He was treated with plasmapheresis, resulting in a transient improvement of dyspnea. Then, he was given humanized anti-interleukin-6 receptor antibody (tocilizumab), which resulted in the dramatic improvement of dyspnea and PH a few weeks later. PH is a rare complication of MCD, and could be successfully treated with tocilizumab.
authors: Division of Hematology and Oncology, Department of Medicine, Kyoto Prefectural University of Medicine, 465 Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto, 606-8566, Japan. namura-k@koto.kpu-m.ac.jp.
authors: Kyoko
authors: K
authors: Taniguchi
authors: Division of Hematology and Oncology, Department of Medicine, Kyoto Prefectural University of Medicine, 465 Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto, 606-8566, Japan.
authors: Chihiro
authors: C
authors: Shimazaki
authors: Division of Hematology and Oncology, Department of Medicine, Kyoto Prefectural University of Medicine, 465 Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto, 606-8566, Japan.
authors: Yoshiko
authors: Y
authors: Fujimoto
authors: Division of Hematology and Oncology, Department of Medicine, Kyoto Prefectural University of Medicine, 465 Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto, 606-8566, Japan.
authors: Kazuho
authors: K
authors: Shimura
authors: Division of Hematology and Oncology, Department of Medicine, Kyoto Prefectural University of Medicine, 465 Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto, 606-8566, Japan.
authors: Hitoji
authors: H
authors: Uchiyama
authors: Division of Hematology and Oncology, Department of Medicine, Kyoto Prefectural University of Medicine, 465 Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto, 606-8566, Japan.
authors: Yosuke
authors: Y
authors: Matsumoto
authors: Division of Hematology and Oncology, Department of Medicine, Kyoto Prefectural University of Medicine, 465 Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto, 606-8566, Japan.
authors: Junya
authors: J
authors: Kuroda
authors: Division of Hematology and Oncology, Department of Medicine, Kyoto Prefectural University of Medicine, 465 Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto, 606-8566, Japan.
authors: Shigeo
authors: S
authors: Horiike
authors: Division of Hematology and Oncology, Department of Medicine, Kyoto Prefectural University of Medicine, 465 Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto, 606-8566, Japan.
authors: Masafumi
authors: M
authors: Taniwaki
mesh_terms: D000328:Adult; D000911:Antibodies, Monoclonal / Q000008:administration & dosage*; D061067:Antibodies, Monoclonal, Humanized; D005871:Castleman Disease / Q000097:blood / Q000150:complications / Q000188:drug therapy*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000097:blood / Q000188:drug therapy* / Q000209:etiology; D007074:Immunoglobulin G / Q000097:blood; D015850:Interleukin-6 / Q000097:blood; D008297:Male; D042461:Vascular Endothelial Growth Factor A / Q000097:blood
chemical_list: D000911:Antibodies, Monoclonal; D061067:Antibodies, Monoclonal, Humanized; C508600:IL6 protein, human; D007074:Immunoglobulin G; D015850:Interleukin-6; C467484:VEGFA protein, human; D042461:Vascular Endothelial Growth Factor A; C502936:tocilizumab