KITAJIMA Kumiko
   Department   School of Medicine(Tokyo Women's Medical University Hospital), School of Medicine
   Position   Assistant Professor
Article types Original article
Language English
Peer review Peer reviewed
Title Successful Long-term Graft Survival of a Renal Transplantation Patient with Wiskott-Aldrich Syndrome
Journal Formal name:Internal medicine (Tokyo, Japan)
Abbreviation:Intern Med
ISSN code:09182918/13497235
Domestic / ForeginDomestic
Volume, Issue, Page 55(13),pp.1761-1763
Author and coauthor KAI KOTARO†, Ogawa Yuichi, MIKI Katsuyuki, IWADOH Kazuhiro, Sannomiya Akihiro, MURAKAMI Toru, Koyama Ichiro, Kitajima Kumiko, Nakajima Ichiro, Fuchinoue Shohei
Publication date 2016
Summary Wiskott-Aldrich syndrome, a rare X-linked hereditary syndrome, is characterized by immunodeficiency, thrombocytopenia, and eczema. The underlying T-cell defect renders renal transplantation and immunosuppressive treatments uncertain. The present case exhibited the mild clinical manifestation, regarded as X-linked thrombocytopenia. He successfully underwent a living-donor ABO-compatible renal transplantation and splenectomy in 2002, and thereafter experiencing no severe rejection, serious infection, or malignancy for more than 10 years. Though IgA nephropathy was detected 8 months after transplantation, the patient's renal function and proteinuria were stable without any treatment. The present case showed a successful long-term graft survival and the importance of splenectomy added to renal transplantation.
DOI 10.2169/internalmedicine.55.6337