Successful treatment of non-Hodgkin's lymphoma using R-CHOP in a patient with Wiskott-Aldrich syndrome followed by a reduced-intensity stem cell transplant.

[wiskott-aldrich syndrome]

WAS is an X-linked primary immunodeficiency characterized by microthrombocytopenia , eczema , recurrent infections , and increased incidence of autoimmunity and malignancy . HSCT is the only curative treatment for WAS . Herein , we report the case of a 17 -yr -old boy with WAS who received an unrelated HSCT while in complete remission of diffuse large B-cell lymphoma after chemotherapy . Pretransplant conditioning consisted of fludarabine , busulfan , and total body irradiation (4 Â Gy ). GvHD prophylaxis consisted of tacrolimus and short -course methotrexate . Following HSCT , rapid and stable engraftment was observed . Platelet count gradually increased , and the generalized eczema improved . The patient developed grade II acute GvHD and limited chronic GvHD on days 30 and 210 , respectively , which resolved with immunosuppressive treatment . Symptoms caused by the reactivation of human herpes virus-6 , BK virus , and VZV were observed from days 21 , 60 , and 96 , respectively ; they were resolved after conservative treatment and acyclovir administration . No other regimen-related toxicity was observed . Complete donor bone marrow chimerism was achieved one Â month after transplantation . RIST is an effective therapeutic option for older children with WAS accompanied by malignant lymphoma .

Diseases
Validation

Diseases presenting "recurrent infections" symptom

22q11.2 deletion syndrome

junctional epidermolysis bullosa

kabuki syndrome

omenn syndrome

wiskott-aldrich syndrome

This symptom has already been validated