Tocilizumab is effective for pulmonary hypertension associated with multicentric Castleman's disease.
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.
Taniguchi
Kyoko
K
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.
Shimazaki
Chihiro
C
Division of Hematology and Oncology, Department of Medicine, Kyoto Prefectural University of Medicine, 465 Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto, 606-8566, Japan.
Fujimoto
Yoshiko
Y
Division of Hematology and Oncology, Department of Medicine, Kyoto Prefectural University of Medicine, 465 Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto, 606-8566, Japan.
Shimura
Kazuho
K
Division of Hematology and Oncology, Department of Medicine, Kyoto Prefectural University of Medicine, 465 Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto, 606-8566, Japan.
Uchiyama
Hitoji
H
Division of Hematology and Oncology, Department of Medicine, Kyoto Prefectural University of Medicine, 465 Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto, 606-8566, Japan.
Matsumoto
Yosuke
Y
Division of Hematology and Oncology, Department of Medicine, Kyoto Prefectural University of Medicine, 465 Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto, 606-8566, Japan.
Kuroda
Junya
J
Division of Hematology and Oncology, Department of Medicine, Kyoto Prefectural University of Medicine, 465 Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto, 606-8566, Japan.
Horiike
Shigeo
S
Division of Hematology and Oncology, Department of Medicine, Kyoto Prefectural University of Medicine, 465 Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto, 606-8566, Japan.
Taniwaki
Masafumi
M
Division of Hematology and Oncology, Department of Medicine, Kyoto Prefectural University of Medicine, 465 Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto, 606-8566, Japan.
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
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