A small percent (3-5%) of patients with a well-differentiated lymphoproliferative disorder may develop a high grade lymphoma or Hodgkin's disease. The new lesion may be a transformation of the same malignant clone as the original tumor, or it may represent a second neoplasm. The pattern of disease progression and subsequent clinical course is termed Richter's syndrome.
Features of Richter's syndrome:
(1) history of chronic lymphocytic leukemia (CLL), or less often a small lymphocytic lymphoma (WDLL)
(2) onset of clinical deterioration with fever and weight loss
(3) demonstration of a diffuse large cell lymphoma or Hodgkin's disease
(4) a poor prognosis, with the new tumor relatively resistant to therapy
The new tumor may involve:
(1) predominantly lymph nodes, with hepatosplenomegaly
(2) the gastrointestinal tract, which may have a better prognosis than the nodal form
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