Richter's transformation

Richter's syndrome (RS), also known as Richter's transformation, is a transformation of B cell chronic lymphocytic leukemia (CLL)[1] or hairy cell leukemia into a fast-growing diffuse large B cell lymphoma, a variety of non-Hodgkin lymphoma which is refractory to treatment and carries a bad prognosis.[2] There is also a less common variant in which the CLL changes into a Hodgkin's lymphoma. Rarely, transformations to a form of myeloid leukemia have been observed. These extraordinarily rare transformations carry a very poor prognosis.[3] Richter's transformation affects about 5% of CLL patients at some point during their lives.[4]

Signs and symptoms

Symptoms of Richter’s transformation in a CLL patient include fever (without infection), elevated serum levels of lactate dehydrogenase, and rapidly enlarging lymph nodes.[5] While about 8% of all CLL patients will have elevated levels of serum lactate dehydrogenase (LDH), more than 50% of CLL patients with Richter's transformation will have elevated LDH levels.

Richter's can appear suddenly, even in patients who were in remission.

Cause

A case of RS may have arisen by one of two different routes: a transformation of the CLL cells into lymphoma, or the appearance of an unrelated lymphoma.[4]

It is thought that genetic defects may introduce the additional abnormalities necessary to transform CLL cells into Richter's syndrome cells.[5]

Treatment

Treatment with conventional immunochemotherapy is usually indicated; in younger patients, allogeneic bone marrow transplantation may be curative.[4]

Prognosis

The prognosis is generally poor. The RS score (Richter syndrome score), which is an estimate of the patient's prognosis, is based on the patient's performance status, LDH, platelet count, the size of the lymphoma tumors, and the number of prior therapies already received.[4] Overall, the median survival is between five and eight months.[6] Untreated, RS is invariably fatal.

The Hodgkin's lymphoma variant of Richter's carries a better prognosis than the predominant diffuse large B-cell lymphoma type, but a worse prognosis than a de novo case of Hodgkin's.

Epidemiology

A SEER-based analysis estimated the risk of Richter's transformation in Chronic Lymphocytic Leukemia (CLL) patients in the United States to be about 0.72%. The majority of transformations (85%) were Diffuse Large B-Cell Lymphoma (DLBCL), while transformation to Hodgkin occurred in 15% of cases. Most of those transformation events were nodal (74%). The most commonly involved extra-nodal sites were the GI tract (25%), skeletal system (19%), and the brain/CNS (12%). The median time from the diagnosis of CLL to the development of Richter's transformation was about 4 years, with no significant difference in the time interval based on histology of the transformation event comparing DLBCL and Hodgkin's. The median age at the time of Richter's transformation was 66 years. [7]

References

  1. ↑ Jain, P; O'Brien, S (Dec 2012). "Richter's transformation in chronic lymphocytic leukemia". Oncology (Williston Park, N.Y.). 26 (12): 1146–52. PMID 23413591.
  2. ↑ "NCI Dictionary of Cancer Terms: R". National Cancer Institute. NIH. 2011-02-02. Retrieved 4 January 2013.
  3. ↑ Hatoum, Hassan A.; Mahfouz, Rami A.R.; Otrock, Zaher K.; Hudaib, Abdel-Rahman; Taher, Ali T.; Shamseddine, Ali I. (2007). "Acute myeloid leukemia with T-cell receptor gamma gene rearrangement occurring in a patient with chronic lymphocytic leukemia: A case report". American Journal of Hematology. 82 (1): 69–72. doi:10.1002/ajh.20762. ISSN 0361-8609. PMID 16947317.
  4. 1 2 3 4 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.
  5. 1 2 Tsimberidou, Apostolia-Maria; Keating, Michael J.; Wierda, William G. (October 2007). "Richter's transformation in chronic lymphocytic leukemia". Current Hematologic Malignancy Reports. 2 (4): 265–271. doi:10.1007/s11899-007-0036-9. PMID 20425379. S2CID 39363430.
  6. ↑ Swords, R; Bruzzi, J; Giles, F (2007). "Recent advances in the diagnosis and therapy of Richter's syndrome". Medical Oncology (Northwood, London, England). 24 (1): 17–32. doi:10.1007/BF02685899. PMID 17673808. S2CID 41455076.
  7. ↑ Elnair, Radowan; Ellithi, Moataz; Kallam, Avyakta; Shostrom, Valerie; Bociek, Robert G. (1 October 2021). "Outcomes of Richter's transformation of chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL): an analysis of the SEER database". Annals of Hematology. 100 (10): 2513–2519. doi:10.1007/s00277-021-04603-y. PMID 34279675. S2CID 236036443.

Further reading

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