Richter’s transformation is defined as the development of diffuse
large cell lymphoma in patients with an underlying diagnosis of CLL.
The reported incidence of the development of NHL, Hodgkin’s lymphoma,
and multiple myeloma are 3.0%, 0.5%, and 0.1%, respectively.
Richter’s syndrome is often heralded by a rapid clinical deterioration
in the presence of multiple systemic symptoms, such as drenching night
sweats, high spiking temperatures, and weight loss.2In
addition, patients may manifest transformation with increased
lymphadenopathy, splenomegaly, and hepatomegaly.3–5 All
of these features were present in our patient. The histology of
lymphoma is noted to be a diffuse large cell type or an immunoblastic
variant. Once Richter’s transformation occurs, it is generally
believed to be a poor prognostic indicator, as median survival is 6
months. Therapy for Richter’s syndrome is directed at the large cell
lymphoma. The response to chemotherapy is poor, but there have been
cases of complete remission reported.