Journal of the American Academy of Dermatology
Volume 60, Issue 1 , Pages 157-161 , January 2009

Primary cutaneous Richter syndrome: Prognostic implications and review of the literature

  • Mika L. Yamazaki, BA

      Affiliations

    • John A. Burns School of Medicine, University of Hawaii at Manoa, Honolulu, Hawaii
    • ,
  • Christopher A. Lum, MD

      Affiliations

    • Departments of Medicine and Pathology at the University of Hawaii, Honolulu, Hawaii
    • ,
  • Allan K. Izumi, MD

      Affiliations

    • Division of Dermatology, Department of Medicine, University of Hawaii, Honolulu, Hawaii
    • Corresponding Author InformationReprint requests: Allan K. Izumi, MD, 1380 Lusitana St, Ste 412, Honolulu, HI 96813.

References 

  1. Miyamura K, Osada H, Yamauchi T, Itoh M, Kodera Y, Suchi T, et al. Single clonal origin of neoplastic B-cells with different immunoglobulin light chains in a patient with Richter's syndrome. Cancer. 1990;66:140–144
  2. Cherepakhin V, Baird SM, Meisenholder GW, Kipps TJ. Common clonal origin of chronic lymphocytic leukemia and high-grade lymphoma of Richter's syndrome. Blood. 1993;82:3141–3147
  3. Cofrancesco E, Baldini L, Ciania A, Neri A, Masini T, Chinaglia D, et al. Evidence of clonal progression in a case of Richter syndrome. Cancer. 1993;71:741–744
  4. Tohda S, Morio T, Suzuki T, Nagata K, Kamiyama T, Imai Y, et al. Richter syndrome with two B cell clones possessing different surface immunoglobulins and immunoglobulin gene rearrangements. Am J Hematol. 1990;35:32–36
  5. Kerim S, Geuna M, Francia di Celle P, Carbone A, Ponti R, Novero D, et al. Heterogeneous immunoglobulin gene rearrangement in a B-chronic lymphocytic leukemia progressing into non-Hodgkin lymphoma (Richter syndrome). Cancer. 1993;71:359–363
  6. Trump DL, Mann RB, Phelps R, Roberts H, Conley CL. Richter's syndrome: diffuse histiocytic lymphoma in patients with chronic lymphocytic leukemia. Am J Med. 1980;68:539–548
  7. Foucar K, Rydell RE. Richter's syndrome in chronic lymphocytic leukemia. Cancer. 1980;46:118–134
  8. Robertson LE, Pugh W, O'Brien S, Kantarjian H, Hirsch-Ginsberg C, Cork A, et al. Richter's syndrome: a report on 39 patients. J Clin Oncol. 1993;11:1985–1989
  9. Brousse N, Solal-Celigny P, Herrera A, Breil P, Molas G, Flejou JF, et al. Gastrointestinal Richter's syndrome. Hum Pathol. 1985;16:854–857
  10. Ott MM, Ott G, Roblick U, Linke B, Kneba M, de Leon F, et al. Localized gastric non-Hodgkin's lymphoma of high-grade malignancy in patients with pre-existing chronic lymphocytic leukemia or immunocytoma. Leukemia. 1996;9:609–614
  11. Hattenhauer MG, Pach JM. Ocular lymphoma in a patient with chronic lymphocytic leukemia. Am J Ophthalmol. 1996;122:266–268
  12. Houdelette P, Dumotier J, Hauteville D, Pierre C, Berthod N. Richter's syndrome with testicular localization. J Urol (Paris). 1989;95:507–508
  13. Mahé B, Moreau P, Bonnemain B, Letortorec S, Menegali D, Bourdin S, et al. Isolated Richter's syndrome of the brain: two recent cases. Nouv Rev Fr Hematol. 1994;36:383–385
  14. Bayliss KM, Kueck BD, Hanson CA, Matthaeus WG, Almagro UA. Richter's syndrome presenting as primary central nervous system lymphoma. Transformation of an identical clone. Am J Clin Pathol. 1990;93:117–123
  15. O'Neill BP, Habermann TM, Banks PM, O'Fallon JR, Earle JD. Primary central nervous system lymphoma as a variant of Richter's syndrome in two patients with chronic lymphocytic leukemia. Cancer. 1989;64:1296–1300
  16. Novice RM, Makhail GR, Maeda K, Hawley RG. Richter's syndrome presenting as a solitary cutaneous nodule. Int J Dermatol. 1989;28:36–37
  17. Zarco C, Lahuerta-Palacios JJ, Borrego L, Toscano R, Gil R, Iglesias L. Centroblastic transformation of chronic lymphocytic leukaemia with primary skin involvement—cutaneous presentation of Richter's syndrome. Clin Exp Dermatol. 1993;18:263–267
  18. Fraitag S, Bodemer C, Rousselot P, Hermine O, MacIntyre E, De Prost Y, et al. Cutaneous transformation of chronic lymphoid leukemia into immunoblastic lymphoma. Cutaneous manifestation of Richter syndrome. [in French] Ann Dermatol Venereol. 1995;122:530–533
  19. Cerroni L, Zenahlik P, Hofler G, Kaddu S, Smolle J, Kerl H. Specific cutaneous infiltrates of B-cell chronic lymphocytic leukemia: a clinicopathologic and prognostic study of 42 patients. Am J Surg Pathol. 1996;20:1000–1010
  20. Ratnavel RC, Dunn-Walters DK, Boursier L, Frazer-Andrews E, Orchard G, Russell-Jones R, et al. B-cell lymphoma associated with chronic lymphatic leukaemia: two cases with contrasting aggressive and indolent behavior. Br J Dermatol. 1999;140:708–714
  21. Robak E, Góra-Tybor J, Kordek R, Wawrzyniak E, Bartkowiak J, Bednarek A, et al. Richter syndrome first manifesting as cutaneous B-cell lymphoma clonally distinct from primary B-cell chronic lymphocytic leukaemia. Br J Dermatol. 2005;153:833–837
  22. Richter MN. Generalized reticular cell sarcoma of lymph nodes associated with lymphatic leukemia. Am J Pathol. 1928;4:285–292
  23. Lortholary P, Boiron M, Ripault P, Levy JP, Manus A, Bernard J. Chronic lymphoid leukemia secondarily associated with a malignant reticulopathy: Richter's syndrome. Nouv Rev Fr Hematol. 1964;78:621–644
  24. Kahn G, Norton AJ, Slavin G. Epstein-Barr virus in Reed Sternberg–like cells in non-Hodgkin's lymphomas. J Pathol. 1993;169:9–14
  25. Foon KA, Thiruvengada R, Saven A, Bernstein ZP, Gale RP. Genetic relatedness of lymphoid malignancies. Transformation of chronic lymphocytic leukemia as a model. Ann Intern Med. 1993;119:63–73
  26. Brynes RK, McCourty A, Sun NC, Koo CH. Trisomy 12 in Richter's transformation of chronic lymphocytic leukemia. Am J Clin Path. 1995;104:199–203
  27. Han T, Sadamori N, Ozer H, Gajera R, Gomez GA, Henderson ES, et al. Cytogenetic studies in 77 patients with chronic lymphocytic leukemia: correlations with clinical, immunologic, and phenotypic data. J Clin Oncol. 1984;2:1121–1132
  28. Cobo F, Martínez A, Pinyol M, Hernández L, Gómez M, Beá S, et al. Multiple cell cycle regulator alterations in Richter's transformation of chronic lymphocytic leukemia. Leukemia. 2002;16:1028–1034
  29. Foon K, Thiruvengadam R, Saven A, Bernstein ZP, Gale RP. Genetic relatedness of lymphoid malignancies: transformation of chronic lymphocytic leukemia as a model. Ann Intern Med. 1993;119:63–73
  30. Bekkenk MW, Geelen FA, van Voorst Vader PC, Heule F, Geerts ML, van Vloten WA, et al. Primary and secondary cutaneous CD30(+) lymphoproliferative disorders: a report from the Dutch Cutaneous Lymphoma Group on the long-term follow-up data of 219 patients and guidelines for diagnosis and treatment. Blood. 2000;95:3653–3661
  31. Liu HL, Hoppe RT, Kohler S, Harvell JD, Reddy S, Kim YH. CD30+ cutaneous lymphoproliferative disorders: the Stanford experience in lymphomatoid papulosis and primary cutaneous anaplasitc large cell lymphoma. J Am Acad Dermatol. 2003;49:1049–1058
  32. Grange F, Bekkenk MW, Wechsler J, Meijer CJ, Cerroni L, Bernengo M, et al. Prognostic factors in primary cutaneous large B-cell lymphomas: a European multicenter study. J Clin Oncol. 2001;19:3602–3610
  33. Robak T. Second malignancies and Richter's syndrome in patients with chronic lymphocytic leukemia. Hematology. 2004;9:387–400
  34. Tsimberidou AM, O'Brien SM, Cortes JE, Faderl S, Andreeff M, Kantarjian HM, et al. Phase II study of fludarabine, cytarabine (Ara-C), cyclophosphamide, cisplatin and GM-CSF (FACPGM) in patients with Richter's syndrome or refractory lymphoproliferative disorders. Leuk Lymphoma. 2002;43:767–772
  35. Willemze R, Jaffe ES, Burg G, Cerroni L, Berti E, Serdlow SH, et al. WHO-EORTC classification for cutaneous lymphomas. Blood. 2005;105:3768–3785

 Funding sources: None.

 Conflicts of interest: None declared.

PII: S0190-9622(08)00908-0

doi: 10.1016/j.jaad.2008.07.017

Journal of the American Academy of Dermatology
Volume 60, Issue 1 , Pages 157-161 , January 2009

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