Richter transformation
MONDO:0002083
Transformation of chronic lymphocytic leukemia (CLL) or small lymphocytic lymphoma (SLL) into a more aggressive type of lymphoma, usually diffuse large B-cell lymphoma (immunoblastic or centroblastic variant). Occasional cases of transformation to Hodgkin's lymphoma have also been described, particularly in patients treated with purine nucleotide analogs. Molecular genetic studies suggest that in approximately half of the cases, the lymphoma is clonally related to the underlying CLL/SLL, whereas in the remaining cases the lymphoma probably represents a secondary, unrelated neoplasm.
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Richter's transformation
Richter's transformation (RT), also known as Richter's syndrome, is the conversion of chronic lymphocytic leukemia (CLL) or its variant, small lymphocytic lymphoma (SLL), into a new and more aggressively malignant disease. CLL is the circulation of malignant B lymphocytes with or without the infiltration of these cells into lymphatic or other tissues while SLL is the infiltration of these malignant B lymphocytes into lymphatic and/or other tissues with little or no circulation of these cells in the blood. CLL along with its SLL variant are grouped together in the term CLL/SLL. RT is diagnosed in individuals who have CLL/SLL that converts to a malignancy with the microscopic histopathology of diffuse large B-cell lymphoma (DLBCL) or, less commonly, Hodgkin's lymphoma (HL). There are rare cases of: CLL/SLLs that convert into lymphoblastic lymphoma, hairy cell leukemia, or a high grade T cell lymphoma such as anaplastic large-cell lymphoma, angioimmunoblastic T-cell lymphoma, or plasmablastic lymphoma; CLL/SLLs that convert into acute myeloid leukemia; CLL/SLLs that convert into or develop non-hematological malignancies such as lung cancer, brain cancer, melanoma of the eye or skin, salivary gland tumors, and Kaposi's sarcomas; conversion of follicular lymphoma, lymphoblastic lymphoma, or marginal zone lymphoma into other types of hematological malignancies.. While some of these conversions have been termed RTs, the World Health Organization and most reviews have defined RT as a conversion of CLL/SLL into a disease with DLBCL or HL histopathology. Here, RTs are considered t...
Background only. Canonical identity remains MONDO; clinical-trial relationships come from ClinicalTrials.gov.Drugs being studied
Disease identity and terminology: MONDO. Trial relationships: ClinicalTrials.gov.