Richter transformation is defined as a diffuse large cell lymphoma, occurring by transformation of chronic lymphocytic leukemia (CLL).
It may be observed is approximately 5-10% of patient's with chronic lymphocytic leukaemia 5.
Patient's may present with an aggressive disease course with rapidly enlarging lymph nodes, hepatosplenomegaly, and elevated serum lactate dehydrogenase (LDH) levels.
The nodal disease can potentially occur anywhere in the body. The disease can also present as distinct mass like lesions 1.
Epstein Barr virus infection:
- has been shown to associated in smal proportion of patients.
History and etymology
It was first described by M N Richter in 1928 4.
On pathology the biopsy results can mimic small lymphocytic lymphoma
- 1. Ghofrani M, Tantiwongkosi B, Smith AS et-al. Richter transformation of chronic lymphocytic leukemia presenting as a dural-based non-hodgkin lymphoma mass. AJNR Am J Neuroradiol. 2007;28 (2): 318-20. Pubmed citation
- 2. Fiz F, Marini C, Piva R et-al. Adult advanced chronic lymphocytic leukemia: computational analysis of whole-body CT documents a bone structure alteration. Radiology. 2014;271 (3): 805-13. doi:10.1148/radiol.14131944 - Pubmed citation
- 3. Bruzzi JF, Macapinlac H, Tsimberidou AM et-al. Detection of Richter's transformation of chronic lymphocytic leukemia by PET/CT. J. Nucl. Med. 2006;47 (8): 1267-73. Pubmed citation
- 4. Richter MN. Generalized Reticular Cell Sarcoma of Lymph Nodes Associated with Lymphatic Leukemia. Am. J. Pathol. 2010;4 (4): 285-292.7. Free text at pubmed - Pubmed citation
- 5. Jain P, O'Brien S. Richter's transformation in chronic lymphocytic leukemia. Oncology (Williston Park, N.Y.). 2013;26 (12): 1146-52. Pubmed citation
- 6. Yee KW, O'Brien SM, Giles FJ. Richter's syndrome: biology and therapy. Cancer J. 2005;11 (3): 161-74. Pubmed citation
- 7. Rubin D, Hudnall SD, Aisenberg A et-al. Richter's transformation of chronic lymphocytic leukemia with Hodgkin's-like cells is associated with Epstein-Barr virus infection. Mod. Pathol. 1994;7 (1): 91-8. Pubmed citation