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

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...

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Bibliographic Details
Published in:Pediatric transplantation 2014-09, Vol.18 (6), p.E208-E211
Main Authors: Koga, Yuhki, Takada, Hidetoshi, Suminoe, Aiko, Ohga, Shouichi, Hara, Toshiro
Format: Article
Language:English
Subjects:
Adolescent
allogeneic bone marrow transplantation
Antibodies, Monoclonal, Murine-Derived - therapeutic use
Antineoplastic Combined Chemotherapy Protocols - therapeutic use
BK virus
Combined Modality Therapy
Cyclophosphamide - therapeutic use
Doxorubicin - therapeutic use
Graft vs Host Disease - drug therapy
Humans
Immunosuppressive Agents - therapeutic use
Lymphoma, Non-Hodgkin - therapy
Male
malignant lymphoma
Prednisone - therapeutic use
reduced‐intensity stem cell transplantation
Stem Cell Transplantation
Varicella-zoster virus
Vincristine - therapeutic use
Wiskott-Aldrich Syndrome - complications
Wiskott-Aldrich Syndrome - therapy
Wiskott–Aldrich syndrome
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Summary: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.
ISSN:1397-3142
1399-3046
DOI:10.1111/petr.12297