Hodgkin's disease occurring in a patient with the Wiskott-Aldrich syndrome.
Hodgkin's disease, nodular sclerosing, developed in a 16-year-old man with the Wiskott-Aldrich syndrome. Two brothers and two nephews had documented Wiskott-Aldrich syndrome and had died of infectious complications in childhood. While the patient reported here had lifelong thrombocytopenia and recurrent upper respiratory infections, he had no severe infection prior to the development of Hodgkin's disease. Skin test sensitization with dinitrochlorobenzene was unsuccessful. No antibodies were found after immunization with pneumococcal polysaccharides. Platelet aggregation studies were abnormal in the patient, his mother, and one of his nephews. A complete response of short duration occurred after treatment with nitrogen mustard, vincristine, procarbazine, and prednisone. On recurrence, he proved unresponsive to further chemotherapy or radiation therapy. Infection with four different fungi was found at autopsy. This patient is the third recorded case of Hodgkin's disease associated with the Wiskott-Aldrich syndrome.
D000293:Adolescent; D000328:Adult; D001792:Blood Platelets / Q000648:ultrastructure; D005260:Female; D006689:Hodgkin Disease / Q000097:blood / Q000150:complications* / Q000276:immunology; D006801:Humans; D007109:Immunity; D008297:Male; D010375:Pedigree; D010974:Platelet Aggregation; D014923:Wiskott-Aldrich Syndrome / Q000097:blood / Q000150:complications* / Q000209:etiology