Richter's syndrome with different immunoglobulin light chains and different heavy chain gene rearrangements.
In a patient with Richter's syndrome, the chronic lymphocytic leukemia (CLL) expressed lambda, mu, and delta immunoglobulin (lg) chains and the non-Hodgkin lymphoma (NHL) kappa, mu, and delta lg chains. The difference in lg light chain expression suggests that the CLL and NHL are independent malignancies, or that the oncogenic event occurred in a B cell differentiation stage after the heavy chain gene rearrangements but before the selection of the light chain. Analysis of DNA by Southern blotting revealed that the lg heavy chain genes of the two malignancies were rearranged in a different way. We therefore conclude that in this patient the NHL cannot be regarded as a progression of the CLL but should most likely be considered as an independent B cell malignancy, which arose in a susceptible host.
D000368:Aged; D004262:DNA Restriction Enzymes / Q000378:metabolism; D006801:Humans; D007143:Immunoglobulin Heavy Chains / Q000235:genetics*; D007147:Immunoglobulin Light Chains / Q000235:genetics*; D007621:Karyotyping; D007945:Leukemia, Lymphoid / Q000235:genetics / Q000276:immunology*; D008214:Lymphocytes / Q000276:immunology; D008223:Lymphoma / Q000235:genetics / Q000276:immunology*; D008297:Male; D013577:Syndrome
D007143:Immunoglobulin Heavy Chains; D007147:Immunoglobulin Light Chains; D004262:DNA Restriction Enzymes