Analysis with antiidiotype antibody of a patient with chronic lymphocytic
leukemia and a large cell lymphoma (Richter's syndrome)
LF Bertoli, H Kubagawa, GV Borzillo, M Mayumi, JT Prchal, JF Kearney, JR Durant and MD Cooper
A murine monoclonal antibody made against an idiotypic determinant (Id) of
surface IgM/IgD lambda molecules on chronic lymphocytic leukemia (CLL)
cells of a 71-year-old woman was used for clonal analysis by two- color
immunofluorescence. The anti-Id antibody identified IgM+/IgD+/lambda+ B
cells as the predominant cell type of her CLL clone. In addition,
substantial proportions of the IgG and IgA B cells and most of the IgM
plasma cells in her bone marrow and blood were Id+. Six years after
diagnosis, the patient died of respiratory failure due to infiltration of
lungs by malignant cells. Autopsy revealed a dramatic change in the tumor
cell morphology. The lungs, hilar nodes, and liver were infiltrated by a
diffuse large cell lymphoma admixed with the leukemic cells. By
immunohistologic staining these anaplastic lymphoma cells were
IgM+/IgD-/lambda+ B cells expressing the same Id noted earlier on the CLL
cells. The immunoglobulin gene rearrangement pattern on Southern blot
analysis was also the same in leukemic blood cells and in the tissues
involved by the lymphoma. Thus, the combination of antiidiotype and
immunoglobulin gene analyses in this patient with Richter's syndrome
revealed that a CLL clone, seemingly "frozen" in differentiation, was
actually undergoing isotype switching, differentiation into plasma cells,
and evolution into a rapidly growing and fetal lymphoma.
Volume 70,
Issue 1,
pp. 45-50,
07/01/1987
Copyright © 1987 by The American Society of Hematology