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Review

Richter syndrome in chronic lymphocytic leukemia: updates on biology, clinical features and therapy

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Pages 1949-1958 | Received 20 May 2014, Accepted 15 Oct 2014, Published online: 21 Jan 2015

References

  • Richter MN. Generalized reticular cell sarcoma of lymph nodes associated with lymphatic leukemia. Am J Pathol 1928;4:285–292.
  • Lortholary P, Boiron M, Ripault P, et al. [Chronic lymphoid leukemia secondarily associated with a malignant reticulopathy: Richter's syndrome]. Nouv Rev Fr Hematol 1964;4:621–644.
  • Tsimberidou AM, Keating MJ. Richter syndrome: biology, incidence, and therapeutic strategies. Cancer 2005;103:216–228.
  • Robak T, Szmigielska-Kapłon A, Smolewski P, et al. Hodgkin's type of Richter's syndrome in familial chronic lymphocytic leukemia treated with cladribine and cyclophosphamide. Leuk Lymphoma 2003;44: 859–866.
  • Tsimberidou AM, O’Brien S, Kantarjian HM, et al. Hodgkin transformation of chronic lymphocytic leukemia: the M. D. Anderson Cancer Center experience. Cancer 2006;107:1294–1302.
  • Bockorny B, Codreanu I, Dasanu CA. Hodgkin lymphoma as Richter transformation in chronic lymphocytic leukaemia: a retrospective analysis of world literature. Br J Haematol 2012;156:50–66.
  • Swerdlow SH, Campo E, Harris NL, et al. WHO classification of tumours of haematopoietic and lymphoid tissues. 4th ed. Lyon: IARC; 2008.
  • Jaffe ES, Pittaluga S. A review of new and old entities in the WHO classification. Hematology Am Soc Hematol Educ Program 2011: 506–514
  • Aboulafia DM, Lee RM, Hafermann M, et al. Extramedullary facial plasmacytomas with anaplastic features: a diagnostic dilemma with implications for treatment. Am J Clin Oncol 1998;21:401–405.
  • Nakamura KT, Hashimoto Y, Wakasa H, et al. Richter transformation of a T cell phenotype with p53 gene mutation. Eur J Haematol 1997;59:331–332.
  • Sun T, Grupka N, Klein C. Transformation of hairy cell leukemia to highgrade lymphoma: a case report and review of the literature. Hum Pathol 2004;35:1423–1426.
  • Reiniger L, Bodor C, Bognar A, et al. Richter's and prolymphocytic transformation of chronic lymphocytic leukemia are associated with high mRNA expression of activation-induced cytidine deaminase and aberrant somatic hypermutation. Leukemia 2006;20:1089–1095.
  • Harousseau JL, Flandrin G, Tricot G, et al. Malignant lymphoma supervening in chronic lymphocytic leukemia and related disorders. Richter's syndrome: a study of 25 cases. Cancer 1981;48:1302–1308.
  • McDonnell JM, Beschorner WE, Staal SP, et al. Richter's syndrome with two different B-cell clones. Cancer 1986;58:2031–2037.
  • Rossi D, Cerri M, Capello D, et al. Biological and clinical risk factors of chronic lymphocytic leukaemia transformation to Richter syndrome. Br J Haematol 2008;142:202–215.
  • Mao Z, Quintanilla-Martinez L, Raffeld M, et al. 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;31:1605–1614.
  • Rossi D, Spina V, Forconi F, et al. Molecular history of Richter syndrome: origin from a cell already present at the time of chronic lymphocytic leukemia diagnosis. Int J Cancer 2012;130:3006–3010.
  • Chigrinova E, Rinaldi A, Kwee I, et al. Two main genetic pathways lead to the transformation of chronic lymphocytic leukemia to Richter Syndrome. Blood 2013;122:2673–2682.
  • Wong E, Dickinson M. Transformation in follicular lymphoma:biology, prognosis, and therapeutic options. Curr Oncol Rep 2012;14:424–432.
  • Parikh SA, Rabe KG, Call TG, et al. Diffuse large B-cell lymphoma (Richter syndrome) in patients with chronic lymphocytic leukaemia (CLL): a cohort study of newly diagnosed patients. Br J Haematol 2013;162:774–782.
  • Jamroziak K, Grzybowska-Izydorczyk O, Jesionek-Kupnicka D, et al. Poor prognosis of Hodgkin variant of Richter transformation in chronic lymphocytic leukemia treated with cladribine. Br J Haematol 2012;158:286–288.
  • Tadmor T, Shvidel L, Bairey O, et al. Richter transformation to diffuse large B-cell lymphoma: a retrospective study reporting clinical data, outcome and the benefit of adding rituximab to chemotherapy, from the Israeli CLL Study Group. Am J Hematol 2014 Aug 1. [Epub ahead of print]
  • Tadmor T, Shvidel L, Goldschmidt N, et al. Hodgkin's variant of Richter transformation in chronic lymphocytic leukemia; a retrospective study from the Israeli CLL Study Group. Anticancer Res 2014;34:785–790.
  • Hallek M, Fischer K, Fingerle-Rowson G, et al. Addition of rituximab to fludarabine and cyclophosphamide in patients with chronic lymphocytic leukaemia: a randomised, open-label, phase 3 trial. Lancet 2010;376:1164–1174.
  • Maddocks-Christianson K, Slager SL, Zent CS, et al. Risk factors for development of a second lymphoid malignancy in patients with chronic lymphocytic leukaemia. Br J Haematol 2007;139:398–404.
  • Solh M, Rai KR, Peterson BL, et al. The impact of initial fludarabine therapy on transformation to Richter syndrome or prolymphocytic leukemia in patients with chronic lymphocytic leukemia:analysis of an intergroup trial (CALGB 9011). Leuk Lymphoma 2013;54:252–254.
  • de Leval L, Vivario M, De Prijck B, et al. Distinct clonal origin in two cases of Hodgkin's lymphoma variant of Richter's syndrome associated with EBV infection. Am J Surg Pathol 2004;28:679–686.
  • Mao ZR, Rosenwald A, Zhang SJ, et al. Clonality analysis and mutational status of IgVH gene in Hodgkin variant of Richter syndrome. Chin J Pathol 2008;37:523–528.
  • Kyasa MJ, Hazlett L, Parrish RS, et al. Veterans with chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) have a markedly increased rate of second malignancy, which is the most common cause of death. Leuk Lymphoma 2004;45:507–513.
  • Robak T, Blonski JZ, Gora-Tybor J, et al. Second malignancies and Richter's syndrome in patients with chronic lymphocytic leukaemia treated with cladribine. Eur J Cancer 2004;40:383–389.
  • Landgren O, Gridley G, Check D, et al. Acquired immune-related and inflammatory conditions and subsequent chronic lymphocytic leukaemia. Br J Haematol 2007;139:791–798.
  • Rasi S, Spina V, Bruscaggin A, et al. A variant of the LRP4 gene affects the risk of chronic lymphocytic leukaemia transformation to Richter syndrome. Br J Haematol 2011;152:284–294.
  • Aydin S, Rossi D, Bergui L, et al. CD38 gene polymorphism and chronic lymphocytic leukemia:a role in transformation to Richter syndrome? Blood 2008;111:5646–5653.
  • Jamroziak K, Szemraj Z, Grzybowska-Izydorczyk O, et al. CD38 gene polymorphisms contribute to genetic susceptibility to B-cell chronic lymphocytic leukemia: evidence from two case-control studies in Polish Caucasians. Cancer Epidemiol Biom Prev 2009;18:945–953.
  • Rossi D, Spina V, Cerri M, et al. Stereotyped B-cell receptor is an independent risk factor of chronic lymphocytic leukemia transformation to Richter syndrome. Clin Cancer Res 2009;15:4415–4422.
  • Rossi D, Rasi S, Spina V, et al. Different impact of NOTCH1 and SF3B1 mutations on the risk of chronic lymphocytic leukemia transformation to Richter syndrome. Br J Haematol 2012;158:426–429.
  • Seymour J, Campbell J. Richter's syndrome. Basic Clin Oncol 2001;26:459–484.
  • Rossi D, Khiabanian H, Spina V, et al. Clinical impact of small TP53 mutated subclones in chronic lymphocytic leukemia. Blood 2014;123:2139–2147.
  • Fabbri G, Khiabanian H, Holmes AB, et al. Genetic lesions associated with chronic lymphocytic leukemia transformation to Richter syndrome. J Exp Med 2013;210:2273–2288.
  • Rinaldi A, Mensah AA, Kwee I, et al. Promoter methylation patterns in Richter syndrome affect stem-cell maintenance and cell cycle regulation and differ from de novo diffuse large B-cell lymphoma. Br J Haematol 2013;163:194–204.
  • Omoti CE, Omoti AE. Richter syndrome: a review of clinical, ocular, neurological and other manifestations. Br J Haematol 2008;142:709–716.
  • Briones J, Cervantes F, Montserrat E, et al. Hypercalcemia in a patient with chronic lymphocytic leukemia evolving into Richter's syndrome. Leuk Lymphoma 1996;21:521–523.
  • Papajik T, Myslivecek M, Urbanova R, et al. 2-[18F]fluoro-2-deoxy-d-glucose positron emission tomography/computed tomography examination in patients with chronic lymphocytic leukemia may reveal Richter transformation. Leuk Lymphoma 2014;55:314–319.
  • Niemann CU, Polliack A, Hutchings M. Suspected Richter transformation: positron emission tomography/computed tomography tells us who should have a biopsy and where. Leuk Lymphoma 2014;55:233–234.
  • Travis LB, Curtis RE, Hankey BF, et al. Second cancers in patients with chronic lymphocytic leukemia. J Natl Cancer Inst 1992;84:1422–1427.
  • Jenke P, Eichhorst B, Busch R, et al. Cyclophosphamide, adriamycin, vincristine and prednisone plus rituximab (CHOP-R) in fludarabine (F) refractory chronic lymphocytic leukemia (CLL) or CLL with autoimmune cytopenia (AIC) or Richter's transformation (RT): final analysis of a phase II study of the German CLL Study Group. Blood 2011;118(Suppl. 1): Abstract 2860.
  • Coiffier B, Lepage E, Briere J, et al. CHOP chemotherapy plus rituximab compared with CHOP alone in elderly patients with diffuse large-B-cell lymphoma. N Engl J Med 2002;346:235–242.
  • Dabaja BS, O’Brien SM, Kantarjian HM, et al. Fractionated cyclophosphamide, vincristine, liposomal daunorubicin (daunoXome), and dexamethasone (hyperCVXD) regimen in Richter's syndrome. Leuk Lymphoma 2001;42:329–337.
  • Tsimberidou AM, Wierda WG, Plunkett W, et al. Phase I-II study ofoxaliplatin, fludarabine, cytarabine, and rituximab combination therapy in patients with Richter's syndrome or fludarabine-refractory chronic lymphocytic leukemia. J Clin Oncol 2008;26:196–203.
  • Tsimberidou AM, Wierda WG, Wen S, et al. Phase I-II clinical trial of oxaliplatin, fludarabine, cytarabine, and rituximab therapy in aggressive relapsed/refractory chronic lymphocytic leukemia or Richter syndrome. Clin Lymphoma Myeloma Leuk 2013;13:568–574.
  • Tsimberidou AM, Murray JL, O’Brien S, et al. Yttrium-90 ibritumomab tiuxetan radioimmunotherapy in Richter syndrome. Cancer 2004;100:2195–2200.
  • Parikh SA, Kay NE, Shanafelt TD. How we treat Richter syndrome. Blood 2014;123:1647–1657.
  • Robak T, Góra-Tybor J, Tybor K, et al. Richter's syndrome in the brain first manifested as an ischaemic stroke. Leuk Lymphoma 2004;45:1261–1267.
  • Robertson LE, Pugh W, O’Brien S, et al. Richter's syndrome: a report on 39 patients. J Clin Oncol 1993;11:1985–1989.
  • Rodriguez J, Keating MJ, O’Brien S, et al. Allogeneic haematopoietic transplantation for Richter's syndrome. Br J Haematol 2000;110:897–899.
  • Cwynarski K, van Biezen A, de Wreede L, et al. Autologous and allogeneic stem-cell transplantation for transformed chronic lymphocytic leukemia (Richter's syndrome): a retrospective analysis from the chronic lymphocytic leukemia subcommittee of the chronic leukemia working party and lymphoma working party of the European Group for Blood and Marrow Transplantation. J Clin Oncol 2012;30:2211–2217.
  • Espanol I, Buchler T, Ferra C, et al. Richter's syndrome after allogeneic stem cell transplantation for chronic lymphocytic leukaemia successfully treated by withdrawal of immunosuppression, and donor lymphocyte infusion. Bone Marrow Transplant 2003;31:215–218.
  • Rossi D, Spina V, Deambrogi C, et al. The genetics of Richter syndrome reveals disease heterogeneity and predicts survival after transformation. Blood 2011;117:3391–401.
  • Younes A, Bartlett NL, Leonard JP, et al. Brentuximab vedotin (SGN-35) for relapsed CD30-positive lymphomas. N Engl J Med 2010;363:1812–1821.
  • Tsimberidou AM, Kantarjian HM, Cortes J, et al. Fractionated cyclophosphamide, vincristine, liposomal daunorubicin, and dexamethasone plus rituximab and granulocyte-macrophage-colony stimulating factor (GM-CSF) alternating with methotrexate and cytarabine plus rituximab and GM-CSF in patients with Richter syndrome or fludarabine-refractory chronic lymphocytic leukemia. Cancer 2003;97:1711–1720.
  • Tsimberidou AM, O’Brien S, Khouri I, et al. Clinical outcomes and prognostic factors in patients with Richter's syndrome treated with chemotherapy or chemoimmunotherapy with or without stem-cell transplantation. J Clin Oncol 2006;24:2343–2351.

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