Richter's transformation, or Richter's syndrome (RS) is a complication of B cell chronic lymphocytic leukemia (CLL) or hairy cell leukemia (HCL) in which the leukemia changes into a fast-growing diffuse large B cell lymphoma.[1] There is also a less common variant in which the CLL changes into a Hodgkin's lymphoma. Richter's transformation affects about 5% of CLL patients at some point during their lives.[2]
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Symptoms of Richter’s transformation in a CLL patient include fever, loss of weight and muscle mass, and enlarged lymph nodes.[3] While about 8% of all CLL patients will have elevated levels of serum lactate dehydrogenase (LDH), more than 50% of CLL patients with Richter's transformation will have elevated LDH levels.
Richter's can appear suddenly, even in patients who were in remission.
Treatment with conventional immunochemotherapy is usually indicated; in younger patients, allogeneic bone marrow transplantation may be curative.[2]
The prognosis is generally poor. The RS score (Richter syndrome score), which is an estimate of the patient's prognosis, is based on the patient's performance status, LDH, platelet count, the size of the lymphoma tumors, and the number of prior therapies already received.[2] Overall, the median survival is between five to eight months. Untreated, RS is invariably fatal.
The Hodgkin's lymphoma variant of Richter's carries a better prognosis than the predominant diffuse large B-cell lymphoma type, but a worse prognosis than a de novo case of Hodgkin's.
A case of RS may have arisen by one of two different routes: a transformation of the CLL cells into lymphoma, or the appearance of an unrelated lymphoma.[2]