Richter's transformation
Encyclopedia
Richter's transformation, or Richter's syndrome (RS) is a complication of B cell chronic lymphocytic leukemia
(CLL) or hairy cell leukemia
(HCL) in which the leukemia changes into a fast-growing diffuse large B cell lymphoma
. There is also a less common variant in which the CLL changes into a Hodgkin's lymphoma
. Richter's transformation affects about 5% of CLL patients at some point during their lives.
(LDH), more than 50% of CLL patients with Richter's transformation will have elevated LDH levels.
Richter's can appear suddenly, even in patients who were in remission.
may be curative.
, LDH, platelet count, the size of the lymphoma tumors, and the number of prior therapies already received. Overall, the median survival is between five to eight months. Untreated, RS is invariably fatal.
The Hodgkin's lymphoma variant of Richter's carries a better prognosis than the predominant diffuse large B-cell lymphoma type, but a worse prognosis than a de novo case of Hodgkin's.
Chronic lymphocytic leukemia
B-cell chronic lymphocytic leukemia , also known as chronic lymphoid leukemia , is the most common type of leukemia. Leukemias are cancers of the white blood cells . CLL affects B cell lymphocytes. B cells originate in the bone marrow, develop in the lymph nodes, and normally fight infection by...
(CLL) or hairy cell leukemia
Hairy cell leukemia
Hairy cell leukemia is an uncommon hematological malignancy characterized by an accumulation of abnormal B lymphocytes. It is usually classified as a sub-type of chronic lymphoid leukemia...
(HCL) in which the leukemia changes into a fast-growing diffuse large B cell lymphoma
Diffuse large B cell lymphoma
Diffuse large B-cell lymphoma is a type of aggressive non-Hodgkin lymphoma. It accounts for approximately 40% of lymphomas among adults. The median age at diagnosis is 70 years, but it also occurs in children and young adults...
. There is also a less common variant in which the CLL changes into a Hodgkin's lymphoma
Hodgkin's lymphoma
Hodgkin's lymphoma, previously known as Hodgkin's disease, is a type of lymphoma, which is a cancer originating from white blood cells called lymphocytes...
. Richter's transformation affects about 5% of CLL patients at some point during their lives.
Presentation
Symptoms of Richter’s transformation in a CLL patient include fever, loss of weight and muscle mass, and enlarged lymph nodes. While about 8% of all CLL patients will have elevated levels of serum lactate dehydrogenaseLactate dehydrogenase
Lactate dehydrogenase is an enzyme present in a wide variety of organisms, including plants and animals.Lactate dehydrogenases exist in four distinct enzyme classes. Two of them are cytochrome c-dependent enzymes, each acting on either D-lactate or L-lactate...
(LDH), more than 50% of CLL patients with Richter's transformation will have elevated LDH levels.
Richter's can appear suddenly, even in patients who were in remission.
Treatment
Treatment with conventional immunochemotherapy is usually indicated; in younger patients, allogeneic bone marrow transplantationAllogeneic bone marrow transplantation
Allogeneic bone marrow transplantation is a procedure in which a person receives stem cells from a genetically similar, but not identical, donor.- External links :...
may be curative.
Prognosis
The prognosis is generally poor. The RS score (Richter syndrome score), which is an estimate of the patient's prognosis, is based on the patient's performance statusPerformance status
In medicine , performance status is an attempt to quantify cancer patients' general well-being and activities of daily life. This measure is used to determine whether they can receive chemotherapy, whether dose adjustment is necessary, and as a measure for the required intensity of palliative care...
, LDH, platelet count, the size of the lymphoma tumors, and the number of prior therapies already received. Overall, the median survival is between five to eight months. Untreated, RS is invariably fatal.
The Hodgkin's lymphoma variant of Richter's carries a better prognosis than the predominant diffuse large B-cell lymphoma type, but a worse prognosis than a de novo case of Hodgkin's.