What is Richter's transformation?
Richter's transformation is a condition in which some types of leukemia, white blood cell cancer, "transform" and become a diffuse, large type of B-cell lymphoma (DLBL), an aggressive form of cancer involving the lymphatic system. It can also be called Richter's syndrome. The types of leukemia that go through this transformation are leukemia of hairy cells (HC1) and chronic lymphocytic leukemia (CLL), the other of which is more common. Statistics show that 5 to 8% of CLL patients experienced Richter's transformation for some time after CLL was diagnosed. CLL includes overproduction of damaged B cells, which eventually exceed healthy cells in the blood and bone marrow. If leukemic cells are experiencing unusual magnification, CLL may have already undergone Richter's transformation and became DLBL. The first person to describe the occurrence of the transformation was Dr. Maurice Richter, when in 1928 one of his male patients who Cll developed seriously swollen lymph nodes and soon died. Abnormal cell size is a crushTowfully caused by a larger nucleus and excessive cytoplasm, liquid that holds the organelles of cells.
Some symptoms that the patient may experience with Richter's transformation are lymphadenopathy or swelling of the lymph nodes, especially in the stomach area, hepatosplenomegaly or the inflammation of the liver and spleen and inexplicable fever. Blood tests can also show a low number of inserts and red blood cells, causing the patient to experience bruises and difficulty breathing. Other symptoms are sudden weight loss, night sweating and paleness. Many patients experience Richter's syndrome without warning and can unexpectedly feel generally ill is already in remission. Biopsy of swollen lymph nodes is often performed to diagnose precisely if the CLL has turned into a dlbl.
The cause of Richter's transformation must still be determined, but scientists assumed that the "transformation" of leukemic cells is the result of a certain genetican changes in overproduced cells. Studies have shown that the presence of a genetic marker called "ZAP-70" increases the chance of transforming cells. Some scientists have also found that the Epstein-Barr (EPV) virus, a common virus that causes herpes, can also cause leukemic cells to become lymphoma cells.
Richter's transformation can be treated with chemotherapy and a radiation process similar to the one used for non-hodgkin lymphoma. Bone marrow and stem cell transplantation can also be included in treatment options. Patients who use immunosuppressants may have to discuss their medicines with their doctors, as some studies have shown that drugs, namely fludarabin, may expose the patient more risk of Richter's transformation. Treatment is most important because the prognosis of the condition is not very optimistic: patients are often given about six months after diagnosis.