Primary Malignant Reticulosis of the Brain in Wiskott-Aldrich Syndrome

In 1937 Wiskott originally described the combination of eczema, thrombocytopenia, and increased susceptibility to infection in 3 brothers. Aldrich, Steinberg, and Campbell (1954) subsequently characterized this syndrome as a sex-linked recessive disorder, describing a family which included 40 males, 16 of whom died in infancy, and 10 of whom were known to have had eczema, otitis, and bloody diarrhoea. Pathological features of this syndrome include haemorrhage involving the gastro-intestinal tract, skin, adrenal glands, or brain; purulent otitis, pneumonitis, and meningitis; lymphadenopathy, splenomegaly, and thymic atrophy, or fibrosis. Histologically, there is a characteristic depletion of lymphocytes in the follicles of the lymph nodes and the Malpighian corpuscles of the spleen, often accompanied by prominent reticuloendothelial hyperplasia, and infiltration of the visceral organs and brain by reticulum cells, plasma cells, and histiocytes (Coleman, Leikin, and Guin, 1961; Huntley and Dees, 1957; Kildeberg, 1961; Krivit and Good, 1959; ten Bensel, Stadlan, and Krivit, 1966; Wolff, 1967). The development of malignant systemic reticuloendotheliosis and lymphoma in children with Wiskott-Aldrich syndrome has been described (Chaptal et al., 1966; Diamond, 1966; Kildeberg, 1963; Pearson et al., 1965; ten Bensel et al., 1966). It is the purpose of this report to record an example of this syndrome with malignant reticulosis limited to the central nervous system.