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Richter syndrome

Monday 26 April 2004

Definition: Occurrence of an aggressive lymphoma in patients with chronic lymphocytic leukemia (CLL). The two tumors are of distinct clonal origin (15105659).

Approximately 5% of B-cell chronic lymphocytic leukemia (B-CLL) patients develop a secondary aggressive lymphoma, usually of diffuse large B-cell type (DLBCL), termed Richter’s transformation (RT). Rarely, classic Hodgkin lymphoma (HL) is observed. Tumor cells in DLBCL and HL can be clonally identical to the B-CLL clone or arise as an independent, secondary lymphoma.

Localization

- slin (cutaneous Richter syndrome) (20145381)

References

- Cutaneous Richter’s syndrome, prognosis, and clinical, histological and immunohistological patterns: report of four cases and review of the literature. Duong T, Grange F, Auffret N, Aractingi S, Bodemer C, Brousse N, Hermine O, Fraitag S. Dermatology. 2010;220(3):226-33. PMID: 20145381

- Mao Z, Quintanilla-Martinez L, Raffeld M, Richter M, Krugmann J, Burek C, Hartmann E, Rudiger T, Jaffe ES, Muller-Hermelink HK, Ott G, Fend F, Rosenwald A. IgVH Mutational Status and Clonality Analysis of Richter’s Transformation: Diffuse Large B-cell Lymphoma and Hodgkin Lymphoma in Association With B-cell Chronic Lymphocytic Leukemia (B-CLL) Represent 2 Different Pathways of Disease Evolution. Am J Surg Pathol. 2007 Oct;31(10):1605-1614. PMID: 17895764