Definition:
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.
Diagnosis:
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.
Treatment:
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.
Symptoms and Signs:
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.
Causes:
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.
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