Richter's transformation

Richter's transformation
Other namesRichter's syndrome
SpecialtyHematopathology, Oncology, Surgical oncology
TypesDiffuse large B-cell lymphoma type, Hodgkin's lymphoma type
PrognosisPoor
Frequencyrare

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.[1] 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.[2] CLL along with its SLL variant are grouped together in the term CLL/SLL.[1]

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).[3] There are rare cases of: 1) CLL/SLLs that convert into lymphoblastic lymphoma, hairy cell leukemia, or a high grade T cell lymphoma[4] such as anaplastic large-cell lymphoma or angioimmunoblastic T-cell lymphoma;[5] 2) CLL/SLLs that convert into acute myeloid leukemia;[6] 3) CLL/SLLs that convert into or develop non-hematological malignancies such as lung cancer, brain cancer, melanoma of the eye or skin,[5][7] salivary gland tumors, and Kaposi's sarcomas;[8] and 4) conversion of follicular lymphoma, lymphoblastic lymphoma, or marginal zone lymphoma into other types of hematological malignancies.[9] While some of these conversions have been termed RTs, the World Health Organization[10] and most reviews have defined RT as a conversion of CLL/SLL into a disease with DLBCL or HL histopathology. Here, RTs are considered to be CLL/SLLs which convert into a disease with either DLBCL histopathology (here termed DLBCL-RT) or Hodgkin's lymphoma histopathology (here termed HL-RT).

CLL/SLL is the most common adult leukemia in Western countries, accounting for 1.2% of the new cancers diagnosed each year in the United States. It usually occurs in older adults (median age at diagnosis 70) and follows an indolent course over many years.[11] About 1-10% of CLL/SLLs develop a Richter's transformation at a rate of 0.5–1% per year. In earlier studies, the transformed disease was reported to be far more aggressive than CLL/SLL with overall median survival times (i.e. times in which 50% of cases remain alive) between 1.1 and 16.3 months. Newer therapeutic regimens are improving the prognosis of DLBCL-RT and HL-RT.[12]

  1. ^ a b Sigmund AM, Kittai AS (August 2022). "Richter's Transformation". Current Oncology Reports. 24 (8): 1081–1090. doi:10.1007/s11912-022-01274-4. PMID 35384590. S2CID 247975378.
  2. ^ Musanhu E, Sharma RK, Attygalle A, Wotherspoon A, Chau I, Cunningham D, Dearden C, El-Sharkawi D, Iyengar S, Sharma B (November 2021). "Chronic lymphocytic leukaemia and Richter's transformation: multimodal review and new imaging paradigms". Clinical Radiology. 76 (11): 789–800. doi:10.1016/j.crad.2021.06.001. PMID 34217434. S2CID 235733549.
  3. ^ Rossi D, Gaidano G (March 2009). "Richter syndrome: molecular insights and clinical perspectives". Hematol Oncol. 27 (1): 1–10. doi:10.1002/hon.880. PMID 19206112.
  4. ^ D'Addona M, Giudice V, Pezzullo L, Ciancia G, Baldi C, Gorrese M, Bertolini A, Campana A, Fresolone L, Manzo P, Zeppa P, Serio B, Selleri C (August 2022). "Hodgkin Lymphoma and Hairy Cell Leukemia Arising from Chronic Lymphocytic Leukemia: Case Reports and Literature Review". Journal of Clinical Medicine. 11 (16): 4674. doi:10.3390/jcm11164674. PMC 9410146. PMID 36012912.
  5. ^ a b Trimech M, Letourneau A, Missiaglia E, De Prijck B, Nagy-Hulliger M, Somja J, Vivario M, Gaulard P, Lambert F, Bisig B, de Leval L (June 2021). "Angioimmunoblastic T-Cell Lymphoma and Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma: A Novel Form of Composite Lymphoma Potentially Mimicking Richter Syndrome". The American Journal of Surgical Pathology. 45 (6): 773–786. doi:10.1097/PAS.0000000000001646. hdl:2268/289883. PMID 33739791. S2CID 232301854.
  6. ^ Liu H, Miao Y, Ferrajoli A, Tang G, McDonnell T, Medeiros LJ, Hu S (March 2020). "Leukemic phase of Richter transformation: A mimic of acute myeloid leukemia that responded to Ibrutinib monotherapy". American Journal of Hematology. 95 (10): 1221–1223. doi:10.1002/ajh.25782. PMID 32162729. S2CID 212677249.
  7. ^ Travis LB, Curtis RE, Hankey BF, Fraumeni JF (September 1992). "Second cancers in patients with chronic lymphocytic leukemia". Journal of the National Cancer Institute. 84 (18): 1422–7. doi:10.1093/jnci/84.18.1422. PMID 1512794.
  8. ^ Kumar V, Ailawadhi S, Bojanini L, Mehta A, Biswas S, Sher T, Roy V, Vishnu P, Marin-Acevedo J, Alegria VR, Paulus A, Aulakh S, Iqbal M, Manochakian R, Tan W, Chanan-Khan A, Ailawadhi M (September 2019). "Trends in the risk of second primary malignancies among survivors of chronic lymphocytic leukemia". Blood Cancer Journal. 9 (10): 75. doi:10.1038/s41408-019-0237-1. PMC 6768881. PMID 31570695.
  9. ^ Wąsik-Szczepanek E, Szymczyk A, Szczepanek D, Wszoła-Kleinrok J, Chocholska S, Pluta A, Hus M (December 2018). "Richter syndrome: A rare complication of chronic lymphocytic leukemia or small lymphocytic lymphoma". Advances in Clinical and Experimental Medicine. 27 (12): 1683–1689. doi:10.17219/acem/75903. PMID 30156387. S2CID 52113706.
  10. ^ Soilleux EJ, Wotherspoon A, Eyre TA, Clifford R, Cabes M, Schuh AH (December 2016). "Diagnostic dilemmas of high-grade transformation (Richter's syndrome) of chronic lymphocytic leukaemia: results of the phase II National Cancer Research Institute CHOP-OR clinical trial specialist haemato-pathology central review". Histopathology. 69 (6): 1066–1076. doi:10.1111/his.13024. PMID 27345622. S2CID 205171057.
  11. ^ Chiorazzi N, Chen SS, Rai KR (February 2021). "Chronic Lymphocytic Leukemia". Cold Spring Harbor Perspectives in Medicine. 11 (2): a035220. doi:10.1101/cshperspect.a035220. PMC 7849345. PMID 32229611.
  12. ^ Tadmor T, Levy I (October 2021). "Richter Transformation in Chronic Lymphocytic Leukemia: Update in the Era of Novel Agents". Cancers. 13 (20): 5141. doi:10.3390/cancers13205141. PMC 8533993. PMID 34680290.