title: 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.
abstract: 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 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 month after transplantation. RIST is an effective therapeutic option for older children with WAS accompanied by malignant lymphoma.
authors.affiliation: Department of Pediatrics, Graduate school of Medical Sciences, Kyushu University, Fukuoka, Japan.
authors.forename: Yuhki
authors.initials: Y
authors.lastname: Koga
authors.forename: Hidetoshi
authors.initials: H
authors.lastname: Takada
authors.forename: Aiko
authors.initials: A
authors.lastname: Suminoe
authors.forename: Shouichi
authors.initials: S
authors.lastname: Ohga
authors.forename: Toshiro
authors.initials: T
authors.lastname: Hara
mesh_terms: D000293:Adolescent; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D003131:Combined Modality Therapy; D003520:Cyclophosphamide / Q000627:therapeutic use; D004317:Doxorubicin / Q000627:therapeutic use; D006086:Graft vs Host Disease / Q000188:drug therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008228:Lymphoma, Non-Hodgkin / Q000628:therapy*; D008297:Male; D011241:Prednisone / Q000627:therapeutic use; D000069283:Rituximab; D033581:Stem Cell Transplantation; D014750:Vincristine / Q000627:therapeutic use; D014923:Wiskott-Aldrich Syndrome / Q000150:complications* / Q000628:therapy*
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D007166:Immunosuppressive Agents; C571759:R-CHOP protocol; D000069283:Rituximab; D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; D011241:Prednisone
keywords: Wiskott-Aldrich syndrome; allogeneic bone marrow transplantation; malignant lymphoma; reduced-intensity stem cell transplantation