Richter's transformation
Richter's transformation (RT), also known as Richter's syndrome, is a complication of chronic lymphocytic leukemia (CLL) and small lymphocytic lymphoma (SLL). It is characterized by the transformation of these diseases into a more aggressive form of lymphoma, typically diffuse large B-cell lymphoma (DLBCL).
Epidemiology[edit | edit source]
Richter's transformation occurs in approximately 2-10% of patients with CLL/SLL. The risk of transformation increases with the duration of CLL/SLL.
Pathophysiology[edit | edit source]
The exact mechanism of Richter's transformation is not fully understood. It is believed to involve genetic mutations and changes in the tumor microenvironment.
Clinical presentation[edit | edit source]
Patients with Richter's transformation often present with rapidly enlarging lymph nodes, B symptoms (fever, night sweats, weight loss), and elevated lactate dehydrogenase levels.
Diagnosis[edit | edit source]
The diagnosis of Richter's transformation is made by biopsy of an enlarged lymph node or other involved tissue. Immunohistochemistry and flow cytometry can be used to confirm the diagnosis.
Treatment[edit | edit source]
The treatment of Richter's transformation is challenging and often involves aggressive chemotherapy regimens. Stem cell transplantation may be considered in eligible patients.
Prognosis[edit | edit source]
The prognosis of Richter's transformation is generally poor, with a median survival of less than one year.
See also[edit | edit source]
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Contributors: Prab R. Tumpati, MD
