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Japanese Journal of Clinical Oncology Advance Access originally published online on November 21, 2006
Japanese Journal of Clinical Oncology 2007 37(1):66-69; doi:10.1093/jjco/hyl113
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© 2006 Foundation for Promotion of Cancer Research

Successful Treatment of Primitive Neuroectodermal Tumor-associated Microangiopathy with Multiple Bone Metastases

Toru Morishita1, Masanori Matsumoto2, Kanya Honoki3, Atsushi Yoshida3, Yoshinori Takakura3 and Yoshihiro Fujimura2

1 Department of Orthopedic Surgery, National Hospital Organization Nara Medical Center, Nara
2 Department of Blood Transfusion Medicine
3 Department of Orthopedic Surgery, Nara Medical University, Nara, Japan

For reprints and all correspondence: Toru Morishita, Department of Orthopedic Surgery, National Hospital Organization Nara Medical Center, 2-789 Shichijo, Nara, Nara 630-8053; Japan. E-mail: morishit{at}wnara.hosp.go.jp

Received December 28, 2005; accepted April 14, 2006

We report here a 16-year-old male with primitive neuroectodermal tumor (PNET)-associated probable microangiopathy with multiple bone metastases. Laboratory findings excluded the possibility of amegakaryocytic or immune thrombocytopenia and/or disseminated intravascular coagulation. He was first treated with plasma-exchange (PE), followed by platelet transfusions, steroid pulse therapy and combined chemotherapy. PE and steroid pulse therapy reduced his plasma CRP level. Combined chemotherapy drastically increased his platelet count until it had almost normalized without further transfusion. The plasma level of von Willebrand factor-cleaving protease (ADAMTS13) activity measured before PE was not severely deficient (48% of normal) and an unusually large von Willebrand factor multimer (UL-VWFM) was detected. We consider that this therapeutic strategy has the following benefits: (1) reduction of plasma levels of factors that are harmful to both platelet activation and endothelial cell injury; and (2) the safe transfusion of platelet concentrate in thrombotic microangiopathy. This strategy should be confirmed in further cases.

Key Words: PNET • microangiopathy • chemotherapy • ADAMTS13 • UL-VWFM


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