A new look at an old syndrome.

In 1928, a pathologist at Bellevue Hospital in New York City named Maurice Richter performed an autopsy on a 46-year-old man. The patient had a 2-month history of abdominal pain and weight loss and was admitted to the hospital, where he died. He was found to have lymphatic leukemia and reticulum cell sarcoma.[1] In 1964, the term “Richter's syndrome” (or “Richter's transformation”) was introduced into the literature.[2] Subsequently, “Richter's transformation” has been used to describe not only the appearance of high-grade B-cell lymphoma in patients with chronic lymphocytic leukemia, but also transformation of any low-grade B-cell malignancy into a more aggressive disease. The phenomenon appears to occur most frequently in low-grade follicular lymphoma, but it can be seen in any small B-cell malignancy. Transformation does not have to be to diffuse large B-cell lymphoma; indeed, cases of the transformation of chronic lymphocytic leukemia to Hodgkin lymphoma, or to acute leukemia, are also reported using the phrase “Richter's transformation.”[3,4]