Wiskott-Aldrich syndrome: histopathologic findings in the lymph nodes and spleens of 15 patients.

Seventeen lymph nodes and 13 spleens from 15 patients with the Wiskott-Aldrich syndrome were examined histologically. The material included both biopsy and autopsy specimens. Consistent findings included depletion of small lymphocytes from T cell areas (all cases), prominence of the reticulum cell stroma (all cases), the presence of atypical plasma cells with and without plasmacytosis (16 lymph nodes and 11 spleens), and extramedullary hematopoiesis (13 lymph nodes and 9 spleens). Less frequent features noted were tissue eosinophilia, hemophagocytosis, focal fibrosis, and progressive depletion of germinal centers. One case with a unique abundance of transformed lymphocytes is described.

Lymphoreticular disorders in primary immunodeficiencies: new findings based on an up-to-date histologic classification of 35 cases.

A histologic review was undertaken of 35 lymphoreticular disorders that developed in primary immuno-deficiency patients from the Immunodeficiency Cancer Registry. Twenty-one (60%) of the lesions were non-Hodgkin's lymphomas: these included eight B-immunoblastic sarcomas. Eight (23%) of the lesions were Hodgkin's disease, with a high frequency of lymphocytic depletion type in an unusually young age group. Three lesions (8.5%) represented abnormal proliferative processes, which could not be definitely categorized as either benign or malignant. There were only two acute lymphoblastic leukemias (6%). Differences were found between lymphomas arising in Wiskott-Aldrich syndrome and those occurring in ataxia-telangiectasia; this suggests that different pathogenetic mechanisms might operate in their development. The lymphomas in Wiskott-Aldrich syndrome were all of non-Hodgkin's type, predominantly B-immunoblastic sarcomas, and presented as localized extranodal infiltrates. The lymphomas in ataxia-telangiectasia were either Hodgkin's disease, mostly of lymphocytic depletion type, or non-Hodgkin's lymphomas of the histologic subtypes associated with 14q translocations.

The Wiskott-Aldrich syndrome in the United States and Canada (1892-1979).

Information was collected on 301 cases of the Wiskott-Aldrich syndrome in the United States and Canada Examination of available medical records, death certificates and published case reports on these patients showed that they came from a wide geographic area and many diverse ethnic and racial groups. No significant difference was found in the incidence of cases born between 1947 and 1976; the overall rate was 4.0 per million live male births in the United States. Median survival has increased with time from eight months for patients born before 1935 to 6.5 years for those born after 1964. Seventy-six of the 301 patients (25%) were still alive at last follow-up and ranged in age from 1 to 36 years with a median of 10 years. Causes of death were primarily limited to infections or bleeding, but malignancy represented a significant problem. Twelve percent of the group (36 of 301) developed malignancy, the predominant types being lymphorecticular tumors (23 of 36) and leukemia (7 of 36). The overall relative risk for malignancy was found to be greater than 100 times that of the general population and was found to increase with increasing age.

Primary immunodeficiency and malignancy.

Evidence indicating an essential relationship between immunologic deviation and cancer has accumulated rapidly over the past 15 years. Following a brief review of the enormous body of literature linking experimental immunodeficiency and cancer, this discussion will center on humans with primary immunodeficiency disorders and the inordinate number of malignancies which develop in these patients.