Familial chronic lymphocytic leukemia.
Chronic lymphocytic leukemia (CLL) was previously documented in a father and 4 of his 5 offspring. Follow-up studies revealed spontaneous regression of the disease in 1 patient and shifts in the clinical patterns in the other patients; the unaffected sibling developed lung adenocarcinoma. Cell surface analysis showed that 2 of these patients shared a common surface immunoglobulin profile with mu- and delta-type heavy chains and kappa-type light chains, whereas a 3d sibling with CLL had elevated mu- and kappa-chains. The patient with spontaneous disease remission had a perturbation in the percentage of cells bearing these same markers, consistent with a subclinical persistence of her lympho-proliferative process. Immunogenetic markers were associated with the occurrence of CLL, but these B-cell alloantigens were not linked to HLA. Two patients had abnormalities of chromosome 12 in B- but not T-cells: One had trisomy 12; the other had a mixture of dicentrics and translocations involving the same chromosome.
D000368:Aged; D000911:Antibodies, Monoclonal / Q000032:analysis; D005260:Female; D005500:Follow-Up Studies; D006680:HLA Antigens / Q000032:analysis; D000949:Histocompatibility Antigens Class II / Q000032:analysis; D006801:Humans; D007143:Immunoglobulin Heavy Chains / Q000032:analysis; D007147:Immunoglobulin Light Chains / Q000032:analysis; D007621:Karyotyping; D007945:Leukemia, Lymphoid / Q000235:genetics* / Q000276:immunology; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D011947:Receptors, Antigen, B-Cell / Q000032:analysis
D000911:Antibodies, Monoclonal; D006680:HLA Antigens; D000949:Histocompatibility Antigens Class II; D007143:Immunoglobulin Heavy Chains; D007147:Immunoglobulin Light Chains; D011947:Receptors, Antigen, B-Cell