Richter's syndrome is generally accepted that lymphoma or leukemia of low grade
malignancy, during their course, may alter both their morphologic and clinical appearance
and evolve into highly malignant neoplasia. The pathogenesis is not clear, however,
dedifferentiation by the emergence of a new clone of cells of higher maligancy similar to
the blastic transformation in chronic myelocytic leukemia is suggested as possible
mechanism .
A case of Richter's syndrome is described. This 45 year old female had been
diagnosed as chronic lymphocytic leukemia by absolute peripheral lymphocytosis 5
months before, developed sudden severe abdominal pain and was received segmental
resection of ileum under the clinical impression of intestinal perforation with peritonitis.
Histologically, the ulcer margin was diffusely infiltrated by polymorphic cells composed
of large atypical cells having vesicular nuclei, multinucleated giant cells and Reed,
Sternberg like cells admixed with mature lymphocytes. These atypical and
multinucleated cells of the paraffin section showed strong monoclonal immunoreactivity
for IgG and f light chain by PAP method and was interpreted as malignant lymphoma,
diffuse, large cell, immunoblastic, polymorphous.
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