Richter syndrome is an extremely rare disease characterized by the transformation of chronic lymphocytic leukemia (CLL) into a rapidly progressive form of lymphoma. Also known as Richter transformation, this disorder is a type of high grade non Hodgkin’s lymphoma affecting patients with chronic lymphocytic leukemia. To date, only 1 in 20 documented cases of CLL leads to Richter syndrome.

To diagnose Richter syndrome, a lymph node biopsy followed by substantial blood tests may be done. Other possible diagnostic steps include a bone marrow biopsy and a CT scan.

Standard treatment for chronic lymphocytic leukemia (CLL) will continue even if the affected patients have developed Richter syndrome. Treatment is usually a combination of chemotherapy, radiation therapy, monoclonal antibodies and potentially stem cell implantation.

Richter syndrome commonly presents with weight loss, muscle mass loss, fever, and other related health problems. It is important to note that Richter syndrome is a fast-growing cancer, and affected patients can become quite suddenly ill.

Currently, the specific cause of Richter syndrome remains unknown. However, some researches suspect the Epstein Barr virus as the primary trigger. In addition, some theories point to a link between the disease and genetics. It has been proposed that some genes predispose certain individuals to Richter syndrome.