Allogeneic bone marrow transplantation appears to ameliorate IgA nephropathy in a patient with X-linked thrombocytopenia.

[wiskott-aldrich syndrome]

Wiskott-Aldrich syndrome (WAS ) is caused by a mutation in the WAS gene , and it is clinically characterized by the triad of thrombocytopenia , eczema and immunodeficiency . X-linked thrombocytopenia (XLT ), which is a clinically mild form of WAS , is also caused by a WAS gene mutation . Patients with WAS /XLT sometimes also have autoimmune diseases such as IgA nephropathy . Progression of IgA nephropathy may lead to chronic renal failure with a poor prognosis . Here , we describe an XLT patient who also had IgA nephropathy . The patient underwent bone marrow transplantation (BMT ) because of an associated-lymphoproliferative disorder , and clinical and histological improvement in his IgA nephropathy was observed after BMT . The amount of galactose-deficient IgA in the patient 's serum markedly decreased after BMT . Therefore , immunological reconstitution might improve autoimmune diseases in patients with WAS /XLT .