Richter’s transformation is when leukemia transforms into a diffuse type of B-cell lymphoma, affecting the lymphatic system. Hairy cell leukemia and chronic lymphocytic leukemia are the types that undergo this transformation. Symptoms include swollen lymph nodes, inflammation of the liver and spleen, and unexplained fever. The cause is unknown, but it can be treated with chemotherapy and radiation. Patients are often given about six months to live after diagnosis.
Richter’s transformation is a condition in which some type of leukemia, a cancer of the white blood cells, “transforms” into a large, diffuse type of B-cell lymphoma (DLBL), which is an aggressive form of cancer that involves the lymphatic system. It can also be called Richter’s syndrome. The types of leukemia that undergo this transformation are hairy cell leukemia (HCL) and chronic lymphocytic leukemia (CLL), the latter of which is more common. Statistics show that 5 to 8% of CLL patients experience Richter’s transformation sometime after being diagnosed with CLL.
In general, both CLL and DLBL are cancers that affect the body’s immune system, especially the B lymphocyte, a type of white blood cell. CLL involves the overproduction of damaged B cells that eventually outnumber healthy cells in the blood and bone marrow. If leukemia cells become abnormally enlarged, CLL may have already undergone Richter’s transformation and become DLBL. The first person to describe the transformation occurring was Dr. Maurice Richter, when, in 1928, one of his male CLL patients developed severely enlarged lymph nodes and died soon after. The abnormal size of the cell is partly due to a larger nucleus and excessive cytoplasm, the fluid that contains the cell’s organelles.
Some symptoms that the patient may experience in Richter’s transformation are lymphadenopathy or swollen lymph nodes, especially in the stomach area, hepatosplenomegaly or inflammation of the liver and spleen, and unexplained fever. Blood tests may also show low counts of both platelets and red blood cells, causing the patient to bruise and have difficulty breathing. Other symptoms are sudden weight loss, night sweats and pale skin. Many patients experience Richter syndrome without warning and may unexpectedly feel generally unwell, even if the CLL is already in remission. A biopsy of the enlarged lymph node is often done to accurately diagnose whether CLL has turned into DLBL.
The cause of Richter’s transformation has yet to be pinpointed, but scientists have speculated that the ‘transformation’ of leukemia cells is the result of some genetic change within the excess cells. Studies have shown that the presence of a genetic marker, called “ZAP-70”, increases the possibility of transforming the cell. Some scientists have also discovered that the Epstein-Barr virus (EPV), a common virus that causes herpes, can also cause leukemia cells to become lymphoma cells.
Richter’s transformation can be treated with a process of chemotherapy and radiation similar to that used for non-Hodgkin’s lymphoma. Bone marrow and stem cell transplants may also be included in treatment options. Patients taking immunosuppressants may need to discuss their medications with their doctors, as some studies have shown that medication, especially fludarabine, can put a patient more at risk for Richter’s transformation. Treatment is of the utmost importance, as the prognosis for the condition is not very optimistic – patients are often given about six months to live after diagnosis.
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