Kronik lenfositik lösemi- mantle hücreli lenfoma transformasyonu: Bir olgu sunumu
Kronik lenfositik lösemi (KLL) morfolojik olarak B tip lenfositlerin artışıyla karakterize klonal lenfoproliferatif bir hastalıktır. KLL hastalarında ateş, kilo kaybı, sitopeni, klinik kötüleşme ve tedaviye direnç Richter sendromu gelişimini düşündürür. Richter transformasyonu genelde diffüz büyük hücreli lenfomaya dönüşüm olarak izlense de diğer lenfoma tiplerine transformasyonlar daha az sıklıkla gözlenebilmektedir. Sunacağımız vakada; kronik lenfositik lösemi tanısıyla izlenmekte olan bir hastada nadir olan mantle hücreli lenfoma transformasyonu gözlemledik.
Transformation of chronic lymphocytic leukemia to mantle cell iymphoma: A case report
Chronic lymphocytic leukemia (CLL) is a clonal lymphoproliferative disease characterized by clonal expansion of B lymphocytes. Richter Transformation (RT) must be considered if CLL patient had fever, weight loss, cytopenia and resistance to standard therapy. Despite CLL usually transforms to diffuse large cell lymphoma, some rare lymphomas may occur as RT such as Hodgkin variants, lymphoblastic lymphoma nad mantle cell lymphoma. Here, we report a case of Mantle cell lymphoma has become secondary to chronic lymphocytic leukemia due to its being a rare entity.
___
- 1. Redaelli A, Laskin BL, Stephens JM, Botteman MF, Pashos CL. The clinical and epidemiological burden of chronic lymphocytic leukaemia. Eur J Cancer Care 2004;13(3):279-87.
- 2. Cheson BD, Bennett JM, Grever M, et al. National Cancer Institute Sponsored Working Group guidelines for chronic lymphocytic leukemia: revised guidelines for diagnosis and treatment. Blood 1996;87(12):4990-7.
- 3. Guipaud O, Deriano L, Salin H, Vallat L, Sabatier L, Merle-Béral H, Delic J. B-cell chronic lymphocytic leukaemia: a polymorphic family unified by genomic features. Lancet Oncol 2003 ;4(8):505-14.
- 4. 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(12):3141-4.
- 5. Matolcsy A, Inghirami G, Knowles DM. Molecular genetic demonstration of the diverse evolution of Richter’s syndrome. Blood 1994;83(5):1363-72.
- 6. de Boer CJ, Loyson S, Kluin PM, et al. Multiple breakpoints within the BCL-1 locus in B cell lymphoma: rearrangements of the cyclin D1 gene. Cancer Res 1993; 53: 4148–52.
- 7. Robak T. Second malignancies and Richter’s syndrome in patients with chronic lymphocytic leukemia. Hematology 2004;9(5-6):387-400.
- 8. 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(2):202-3.
- 9. Morrison, VA, Rai, K, Peterson, BL, et al. Transformation to Richter’s Syndrome or Prolymphocytic Leukemia (PLL): an Intergroup Study (CALGB 9011). Blood 1999; 94(3):539-42.
- 10. Omoti CE, Omoti AE. Richter syndrome: a review of clinical, ocular, neurological and other manifestations. Br J Haematol 2008;142(5):709-16.
- 11. 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; 24:2343-5.
- 12. Tsimberidou AM, Keating MJ. Richter syndrome: biology, incidence, and therapeutic strategies. Cancer 2005; 103:216-8.
- 13. Robertson LE, Pugh W, O’Brien S, et al. Richter’s syndrome: a report on 39 patients. J Clin Oncol 1993; 11(10):1985-7.
- 14. Tabuteau S, Fernandez J, Garidi R, et al. Richter’s Syndrome in B-CLL: Report of 37 cases (abstract). Blood 1999; 94:306-8.
- 15. 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(12):4415-7.
- 16. Brecher M, Banks PM. Hodgkin’s disease variant of Richter’s syndrome. Report of eight cases. Am J Clin Pathol 1990; 93(2):333-4.
- 17. Lee A, Skelly ME, Kingma DW, Medeiros LJ. B-cell chronic lymphocytic leukemia followed by high grade T-cell lymphoma. An unusual variant of Richter’s syndrome. Am J Clin Pathol 1995; 103(2):348-9.
- 18. Hensel M, Buss EC, Tiemann M, et al. Secondary follicular lymphoma of the bone, transformed into large cell lymphoma, in a patient with chronic lymphocytic leukaemia: an uncommon manifestation of Richter’s syndrome. Eur J Haematol 2004;72(3):217-21.