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

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

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

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

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

The treatment of Richter's transformation is challenging and often involves aggressive chemotherapy regimens. Stem cell transplantation may be considered in eligible patients.

Prognosis

The prognosis of Richter's transformation is generally poor, with a median survival of less than one year.

See also

• Chronic lymphocytic leukemia
• Diffuse large B-cell lymphoma
• Lymphoma

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