Richter's transformation
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| Richter's transformation | |
|---|---|
| Reed-Sternberg cell | |
| Synonyms | Richter's syndrome |
| Pronounce | N/A |
| Specialty | N/A |
| Symptoms | Lymphadenopathy, fever, night sweats, weight loss |
| Complications | Tumor lysis syndrome, infections |
| Onset | Typically in patients with chronic lymphocytic leukemia |
| Duration | Progressive |
| Types | N/A |
| Causes | Transformation of chronic lymphocytic leukemia into a more aggressive form |
| Risks | Chronic lymphocytic leukemia, immunosuppression |
| Diagnosis | Biopsy, immunophenotyping, genetic testing |
| Differential diagnosis | Diffuse large B-cell lymphoma, Hodgkin lymphoma |
| Prevention | N/A |
| Treatment | Chemotherapy, immunotherapy, stem cell transplant |
| Medication | N/A |
| Prognosis | Generally poor, varies with treatment response |
| Frequency | Occurs in approximately 2-10% of chronic lymphocytic leukemia cases |
| Deaths | N/A |
Richter's transformation (RT), also known as Richter's syndrome, is a complication of chronic lymphocytic leukemia (CLL) and small lymphocytic lymphoma (SLL). It is characterized by the transformation of these diseases into a more aggressive form of lymphoma, typically diffuse large B-cell lymphoma (DLBCL).
Epidemiology[edit]
Richter's transformation occurs in approximately 2-10% of patients with CLL/SLL. The risk of transformation increases with the duration of CLL/SLL.
Pathophysiology[edit]
The exact mechanism of Richter's transformation is not fully understood. It is believed to involve genetic mutations and changes in the tumor microenvironment.
Clinical presentation[edit]
Patients with Richter's transformation often present with rapidly enlarging lymph nodes, B symptoms (fever, night sweats, weight loss), and elevated lactate dehydrogenase levels.
Diagnosis[edit]
The diagnosis of Richter's transformation is made by biopsy of an enlarged lymph node or other involved tissue. Immunohistochemistry and flow cytometry can be used to confirm the diagnosis.
Treatment[edit]
The treatment of Richter's transformation is challenging and often involves aggressive chemotherapy regimens. Stem cell transplantation may be considered in eligible patients.
Prognosis[edit]
The prognosis of Richter's transformation is generally poor, with a median survival of less than one year.
See also[edit]
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