[Primary immunologic deficiencies and cancer. 5 anatomo-clinical case reports].
Five patients with primary immunodeficiency and cancer are presented. Two children with ataxia-telangiectasia developed acute lymphoblastic leukemia and malignant lymphoma of B-like origin with chromosome damage and unusual prevalence of antibodies to E.B.V. early antigen. A bone sarcoma occurred in a patient with common variable hypogammaglobulinemia. At least two infants who died with severe combined immunodeficiency had at autopsy congenital myelomonocytic leukemia and malignant lymphoma. These cases indicate the high risk for development of cancer in patients with primary abnormalities of the immune system and suggest the heterogeneity and complexity of pathogenic mechanisms.
D000361:Agammaglobulinemia / Q000150:complications; D001260:Ataxia Telangiectasia / Q000150:complications; D001859:Bone Neoplasms / Q000209:etiology; D002648:Child; D002675:Child, Preschool; D005260:Female; D005770:Gastrointestinal Neoplasms / Q000209:etiology; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000150:complications*; D007223:Infant; D007945:Leukemia, Lymphoid / Q000209:etiology; D007951:Leukemia, Myeloid / Q000209:etiology; D008228:Lymphoma, Non-Hodgkin / Q000209:etiology; D008297:Male; D009369:Neoplasms / Q000209:etiology*; D012516:Osteosarcoma / Q000209:etiology
Déficits immunitaires primitifs et cancers. Cinq observations anatomo-cliniques.