Malignant lymphoma in patients with the Wiskott-Aldrich syndrome.

The type and incidence of malignant lymphoma developing in patients with the Wiskott-Aldrich syndrome being followed at the National Cancer Institute (NCI) between the years 1966 and 1982 was evaluated. Histologic material from lymphoid tissue was available for review on 24 of the 50 Wiskott-Aldrich patients followed by the Metabolism Branch of the NCI. In 17 patients, specimens were obtained by biopsy performed for diagnosis of lymphoid mass lesions, and in 16 patients autopsy specimens were reviewed. In 9 of the 24 patients a diagnosis of malignant lymphoma was made. A distinct preponderance of non-Hodgkin's lymphoma (NHL) over Hodgkin's disease (HD) with a ratio 8:1 was observed, and the overall incidence of malignant lymphoma in all 50 patients was 18%. The most common histologic subtype of NHL was large cell immunoblastic. In all but one patient the diagnosis of lymphoma was made antemortem, most often presenting in extranodal sites or the brain. Involvement of peripheral lymph nodes was conspicuous by its absence. Immunoperoxidase staining for kappa and lambda chain immunoglobulin and lysozyme was negative in the four cases studied, failing to provide supportive evidence for a B-cell or true histiocytic origin for the tumor cells. Histologic subtypes of lymphoma commonly observed in childhood, such as Burkitt's lymphoma and lymphoblastic lymphoma, were not observed. Despite treatment with combination chemotherapy in some patients, there were no long-term remissions and median survival was less than one year following the diagnosis of lymphoma.