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What Is Richter's Transformation?

By Karize Uy
Updated Feb 10, 2024
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Richter’s transformation is a condition wherein some types of leukemia, a cancer of the white blood cells, “transforms” and becomes a diffuse, large type of B-cell lymphoma (DLBL), which is an aggressive form of cancer involving the lymphatic system. It can also be called Richter’s syndrome. The types of leukemia that undergo this transformation are the hairy cell leukemia (HCL) and the chronic lymphocytic leukemia (CLL), the latter of which is more common. Statistics show that 5 to 8% of patients with CLL have experienced Richter’s transformation some time after being diagnosed with CLL.

In general, both CLL and DLBL are cancers that affect the body’s immune system, particularly the B cell, a type of white blood cell. CLL involves the overproduction of damaged B cells that eventually outnumber the healthy cells in the blood and bone marrow. If the leukemic cells experience abnormal enlargement, the CLL may have already undergone the Richter’s transformation and have become DLBL. The first person who described the occurrence of the transformation was Dr. Maurice Richter, when, in 1928, one of his male patients who had CLL developed severely swollen lymph nodes and died shortly. The abnormal size of the cell is partly due to a larger nucleus and excessive cytoplasm, the fluid that holds the cell’s organelles.

Some symptoms that the patient can experience in Richter’s transformation are lymphadenopathy or the swelling of 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 the platelet and red blood cells, causing the patient to experience bruising and difficulty in breathing. Other symptoms are sudden weight loss, night sweats, and paleness. Many patients experience Richter’s syndrome without warning and may unexpectedly feel generally ill, even if the CLL is already in remission. A biopsy of the swollen lymph node is often done to accurately diagnose if the CLL has transformed into DLBL.

The cause of Richter’s transformation has yet to be pinpointed, but scientists have hypothesized that the “transformation” of the leukemic cells is a result of some genetic change within the overproduced cells. Studies have shown that the presence of a genetic marker, named “ZAP-70,” increases the chance of transforming the cell. Some scientists have also discovered that the Epstein-Barr virus (EPV), a common virus that causes herpes, may also cause leukemic cells to become lymphoma cells.

Richter’s transformation can be treated with a chemotherapy and radiation process similar to the one used for non-Hodgkin lymphoma. Bone marrow and stem cell transplants can also be included in the treatment options. Patients who are taking immunosuppressants may have to discuss their medication with their doctors, as some studies have shown that the medication, specifically fludarabine, may put a patient more at risk for Richter’s transformation. Treatment is of utmost importance, as prognosis for the condition is not very optimistic: patients are often given around six months to live after being diagnosed.

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