Successful Autologous Peripheral Blood Stem Cell Transplantation Using Thiotepa in a Patient with Systemic Sclerosis and Cardiac Involvement

Case Report

Successful Autologous Peripheral Blood Stem Cell Transplantation Using Thiotepa in a Patient with Systemic Sclerosis and Cardiac Involvement

ATSUSHI KOMATSUDA, YOSHINARI KAWABATA, TAKAHITO HORIUCHI, MUTSUHITO MOTEGI, MASATOYO OZAWA, NAOHITO FUJISHIMA, MASAAKI KUME, MAKOTO HIROKAWA, HIDEKI WAKUI, AKIHIKO YAMAGUCHI¹ and KEN-ICHI SAWADA

Third Department of Internal Medicine, Akita University School of Medicine, Akita, Japan, and ¹Department of Internal Medicine, Senboku General Hospital, Akita, Japan

A 19-year-old man with systemic sclerosis (SSc) was hospitalized for autologous peripheral blood stem cell transplantation (auto-PBSCT) due to progressive scleroderma and cardiac involvement despite conventional treatment. During the administration of cyclophosphamide (60 mg/kg/day for 2 days) for mobilization and collection of CD34⁺ selected peripheral blood stem cells, he developed congestive heart failure. Echocardiogram showed hypokinetic asynergy from the septum to posterior wall, which might indicate underlying cardiac damage. We were also concerned about the risk of high-dose cyclophosphamide-induced cardiotoxicity. Since the dose-limiting toxicity of thiotepa, an alkylating agent, is myelosuppression, and cardiac toxicity due to thiotepa is less common, we used a conditioning regimen consisting of thiotepa (10 mg/kg/day, day −5) and low-dose cyclophosphamide (50 mg/kg/day, days −3 and −2), instead of the conventional high-dose cyclophosphamide (50 mg/kg/day × 4 days/course). The post-transplant course was uneventful, and the modified Rodnan skin thickness score improved from 32 to 15. The present case report demonstrates that thiotepa can be employed as a conditioning regimen for auto-PBSCT in SSc patients with cardiac involvement in order to reduce cyclophosphamide-induced cardiotoxicity.

keywords —— autologous peripheral blood stem cell transplantation (PBSCT); cardiotoxicity; cyclophosphamide; systemic sclerosis; thiotepa

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Tohoku J. Exp. Med., 2006, 209, 61-67

Correspondence: Atsushi Komatsuda, M.D., Third Department of Internal Medicine, Akita University School of Medicine, 1-1-1 Hondo, Akita City, Akita 010-8543, Japan.

e-mail: komatsud@med.akita-u.ac.jp