Case Report

Chronic Lymphocytic Leukemia Associated with Myelodysplastic Syndrome with Ring Sideroblasts

Authors: Khalil M. Charafeddine, MD, Georges Y. Ibrahim, MD, Rami A. Mahfouz, MD, Ghazi S. Zaatari, MD, Ziad M. Salem, MD


Diagnosis of myelodysplastic syndrome/acute myeloid leukemia (MDS/AML) and chronic lymphocytic leukemia (CLL) in the same patient is extremely rare. We describe a 69-year-old CLL patient who developed MDS with ring sideroblasts 1 year after diagnosis of CLL and without any previous treatment. Diagnosis was based on flow cytometry, bone marrow aspirate morphology, and iron stain. Our findings indicate that the 2 disorders belong to 2 different hematopoietic clones in this patient. In cases of worsening anemia in CLL patients, it is recommended that an iron stain be performed on bone marrow aspirates to exclude a coexistent malignancy such as refractory anemia with ring sideroblasts.

This content is limited to qualifying members.

Existing members, please login first

If you have an existing account please login now to access this article or view purchase options.

Purchase only this article ($15)

Create a free account, then purchase this article to download or access it online for 24 hours.

Purchase an SMJ online subscription ($75)

Create a free account, then purchase a subscription to get complete access to all articles for a full year.

Purchase a membership plan (fees vary)

Premium members can access all articles plus recieve many more benefits. View all membership plans and benefit packages.


1. McPherson R, Pincus MR. Henry's Clinical Diagnosis and Management by Laboratory Methods (Clinical Diagnosis and Management By Laboratory Methods). Amsterdam, Elsevier Science, 2007.
2. Bastion Y, Thomas X, Felman P, et al. High risk myelodysplastic syndrome coexistent with chronic lymphocytic leukemia for more than 9 years: inhibition of the myeloid clone by the lymphoid clone? Leukemia 1991;5:1006–1009.
3. Bracey AW, Maddox AM, Immken L, et al. Coexistence of myelodysplastic syndrome and untreated chronic lymphocytic leukemia with development of acute myeloid leukemia immediately after treatment of chronic lymphocytic leukemia. Am J Hematol 1989;30:174–180.
4. Catovsky D, Galton DA. Myelomonocytic leukaemia supervening on chronic lymphocytic leukaemia. Lancet 1971;1:478–479.
5. Lawlor E, McCann SR, Whelan A, et al. Acute myeloid leukaemia occurring in untreated chronic lymphatic leukaemia. Br J Haematol 1979;43:369–373.
6. Manoharan A, Catovsky D, Clein P, et al. Simultaneous or spontaneous occurrence of lympho- and myeloproliferative disorders: a report of four cases. Br J Haematol 1981;48:111–116.
7. Roberts PD, Forster PM. Chronic lymphocytic leukemia associated with acute myelomonocytic leukemia. Br J Haematol 1973;25:203–206.
8. Robertson LE, Estey E, Kantarjian H, et al. Therapy-related leukemia and myelodysplastic syndrome in chronic lymphocytic leukemia. Leukemia 1994;8:2047–2051.
9. Stern N, Shemesh J, Ramot B. Chronic lymphatic leukemia terminating in acute myeloid leukemia: review of the literature. Cancer 1981;47:1849–1851.
10. Wallis JP, Joyner MV. Acute myeloid leukemia developing in a patient with longstanding untreated chronic lymphocytic leukaemia. Acta Haematol 1986;75:229–231.
11. Papayannis AG, Stathakis NE, Kyrkoy K, et al. Primary acquired sideroblastic anaemia associated with chronic lymphocytic leukaemia. Br J Haematol 1974;28:125–129.
12. Escribano Mora L, Gasalla Chacon R, Martinez Guerrero ML, et al. Acquired sideroblastic anaemia plus chronic lymphocytic leukemia (author's transl) [in Spanish]. Sangre (Barc)1977;22:639–645.
13.Hull MT, Griep JA. Mixed leukemia, lymphatic and myelomonocytic. Am J Clin Pathol 1980;74:473–475.
14. Kornberg A, Goldfarb A, Shalev O. Pseudo-Pelger-Huet anomaly in chronic lymphocytic leukemia. Acta Haematol 1981;66:127–128.
15. Greenberg BR, Miller C, Cardiff RD, et al. Concurrent development of preleukaemic, lymphoproliferative and plasma cell disorders. Br J Haematol 1983;53:125–133.
16. Copplestone JA, Mufti GJ, Hamblin TJ, et al. Immunological abnormalities in myelodysplastic syndromes. II. Coexistent lymphoid or plasma cell neoplasms: a report of 20 cases unrelated to chemotherapy. Br J Haematol 1986;63:149–159.
17. Ferrara F, Del Vecchio L, Antonolfi I, et al. Chronic lymphocytic leukemia coexisting with chronic myelomonocytic leukemia. Haematologica 1992;77:171–173.
18. Tambone-Reyes M, Di Marco P, Tinnirello D, et al. Successful treatment of two cases of myelodysplastic syndrome coexistent with chronic lymphocytic leukemia with lymphoblastoid interferon. Leuk Res 1992;16:1055–1056.
19. Rupniewska ZM, Rolinski J, Siedlecki JA, et al. Myelodysplastic syndrome associated with clonal proliferation of B lymphocytes [in Polish]. Acta Haematol Pol 1993;24:57–64.
20. Tamul KR, Meyers DC, Bentley SA, et al. Two color flow cytometric analysis of concomitant acute myeloid leukemia and chronic lymphocytic leukemia. Cytometry 1994;18:30–34.
21. McCoy JP Jr, Johnson E, Catalano E, et al. Detection and monitoring of a concomitant atypical myeloproliferative disorder and chronic lymphocytic leukemia by flow-cytometric immunophenotyping. Arch Pathol Lab Med 1995;119:1038–1043.
22. Lima M, Porto B, Rodrigues M, et al. Cytogenetic findings in a patient presenting simultaneously with chronic lymphocytic leukemia and acute myeloid leukemia. Cancer Genet Cytogenet 1996;87:38–40.
23. Silber R, Stahl R. CLL and related disease, in Beutler E, Williams WJ, Erslev AJ, et al (eds):Williams Hematology. New York, McGraw-Hill,1990, pp 245–249.
24. Giles FJ, O'Brien SM, Keating MJ. Chronic lymphocytic leukemia in (Richter's) transformation. Semin Oncol 1998;25:117–125.
25. Bessudo A, Kipps TJ. Origin of high-grade lymphomas in Richter syndrome. Leuk Lymphoma 1995;18:367–372.
26. Foucar K. B cell chronic lymphocytic and prolymphocytic leukemia, in Knowles DM (ed):Neoplastic Hematopathology. Baltimore, Williams & Wilkins,1992, pp 1181–1208.
27. Lam CC, Ma ES, Kwong YL. Therapy-related acute myeloid leukemia after single-agent treatment with fludarabine for chronic lymphocytic leukemia. Am J Hematol 2005;79:288–290.
28. Hatoum HA, Mahfouz RA, Otrock ZK, et al. Acute myeloid leukemia with T-cell receptor gamma gene rearrangement occurring in a patient with chronic lymphocytic leukemia: a case report. Am J Hematol 2007;82:69–72.
29. Miller MK, Strauchen JA, Nichols KT, et al. Concurrent chronic lymphocytic leukemia cutis and acute myelogenous leukemia cutis in a patient with untreated CLL. Am J Dermatopathol 2001;23:334–340.
30. Lu CM, Murata-Collins JL, Wang E, et al. Concurrent acute myeloid leukemia with inv(16)(p13.1q22) and chronic lymphocytic leukemia: molecular evidence of two separate diseases. Am J Hematol 2006;81:963–968.
31. Malani AK, Gupta C, Rangineni R, et al. Concomitant presentation of acute myeloid leukemia with T-cell large granular lymphocytic leukemia. Acta Oncol 2007;46:247–249.
32. Gottardi M, Gattei V, Degan M, et al. Concomitant chronic lymphocytic leukemia and acute myeloid leukemia: evidence of simultaneous expansion of two independent clones. Leuk Lymphoma 2006;47:885–889.
33. Schlaifer D, Riga-Huguet F, Pris J, et al. Secondary neoplasms in two patients treated with purine analogues. Nouv Rev Fr Hematol 1994;36:341.
34. Frewin RJ, Provan D, Smith AG. Myelodysplasia occurring after fludarabine treatment for chronic lymphocytic leukaemia. Clin Lab Haematol 1997;19:151–152.
35. Cheson BD, Vena DA, Barrett J, et al. Second malignancies as a consequence of nucleoside analog therapy for chronic lymphoid leukemias. J Clin Oncol 1999;17:2454–2460.
36. Keating MJ, O'Brien S, Kantarjian H, et al. Long-term follow-up of patients with chronic lymphocytic leukemia treated with fludarabine as a single agent. Blood 1993;81:2878–2884.
37. Sorensen JM, Vena DA, Fallavollita A, et al. Treatment of refractory chronic lymphocytic leukemia with fludarabine phosphate via the group C protocol mechanism of the National Cancer Institute: five-year follow-up report. J Clin Oncol 1997;15:458–465.
38. Florensa L, Vallespi T, Woessner S, et al. Incidence and characteristics of lymphoid malignancies in untreated myelodysplastic syndromes. Leuk Lymphoma 1996;23:609–612.
39. Mitterbauer G, Schwarzmeier J, Mitterbauer M, et al. Myelodysplastic syndrome/acute myeloid leukemia supervening previously untreated chronic B-lymphocytic leukemia: demonstration of the concomitant presence of two different malignant clones by immunologic and molecular analysis.Ann Hematol 1997;74:193–197.
40. Sylvester LS, Nowell PC, Bonner H, et al. Concurrent diagnosis of chronic lymphocytic leukemia and myelodysplastic syndrome. Leuk Res 1997;21:619–621.
41. Lai R, Arber DA, Brynes RK, et al. Untreated chronic lymphocytic leukemia concurrent with or followed by acute myelogenous leukemia or myelodysplastic syndrome. A report of five cases and review of the literature. Am J Clin Pathol 1999;111:373–378.
42. Shanafelt TD, Call TG. Current approach to diagnosis and management of chronic lymphocytic leukemia. Mayo Clin Proc 2004;79:388–398.