Myelodysplastic syndrome with myelofibrosis in a 12-year-old patient – A case report

Open access

Abstract

Myelodysplastic syndromes are a heterogeneous group of clonal disorders characterized by peripheral blood cytopenia and normal or hypercellular bone marrow with dysplasia in more than one blood cell lineage, unfavorable prognosis, and lack of response to treatment. We present the case of a 12-year-old male patient who was referred to the Hematology and Oncology Department of Pediatric Clinic I Târgu- Mures in May 2016, with splenomegaly and pancytopenia. The osteomedullary biopsy revealed myelofibrosis, discrete dysplasia of the myeloid series and megakaryocytes, blasts CD34+ approximately 10%, which led to the diagnosis of myelodysplastic syndrome with myelofibrosis. The myeloid precursors indicated a high risk of transformation into acute myeloid leukemia, so chemotherapy associated with corticosteroids was started, leading to slight improvements. Although myelodysplastic syndrome associated with myelofibrosis is rare at this age, despite the treatment and favorable progression in the case presented, in the absence of hematopoietic stem cell transplantation the prognosis remains unfavorable.

1. Elghetany TM, Bhatla T. Myelodysplastic Syndromes and Myeloproliferative Disorders. Lanzkowsky P. Hofmann I. Jeffrey M. Lipton Lanzkowsky’s Manual of Pediatric Hematology and Oncology. Sixth Edition Elsevier. 2016, London, 348-351.

2. Hasle H, Niemeyer CM, Chessells JM. A pediatric approach to the WHO classification of myelodysplastic and myeloproliferative diseases. Leukemia. 2003;17(2):277-282. DOI: 10.1038/sj.leu.2402765

3. Disperati P, Ichim CV, Tkachuk D. Progression of myelodysplasia to acute lymphoblastic leukaemia: implications for disease biology. Leuk Res. 2006;30:233-239. DOI: 10.1016/j.leukres.2005.06.011

4. Vardiman JW, Thiele J, Arber DA, Brunning RD, Borowitz MJ, Porwit A, et al. The 2008 revision of the World Health Organization (WHO) classification of myeloid neoplasms and acute leukemia: rationale and important changes. Blood. 2009;1145(5):937-51. DOI: 10.1182/blood-2009-03-209262

5. Tefferi A. Pathogenesis of myelofibrosis with myeloid metaplasia. J Clin Oncol. 2005;23(33):8520-30. DOI: 10.1200/JCO.2004.00.9316

6. Das P, Deepali J, Das R, Garewal G. Hyperfibrotic myelodysplastic syndrome: a report of three cases from north India. Turk J Hematol. 2009;26:93-6.

7. Bowen D, Culligan D, Jowitt S, Kelsey S, Mufti G, Oscier D, et al. Guidelines for the diagnosis and therapy of adult myelodysplastic syndromes. Br J Haematol. 2003;120(2):187-200. DOI: 10.1046/j.1365-2141.2003.03907.x

8. Porwit A, Saft L. The AML-MDS interface-leukemic transformation in myelodysplastic syndromes. J Hematopathol. 2011;4:69-79. DOI: 10.1007/s12308-011-0088-6

9. Diem H. Myelodysplasia. Tehml H, Diem H, Haferlach T. Color Atlas of Hematology - Practical Microscopic and Clinical Diagnosis. 2nd revised edition 2004. Munich, 106-9.

10. Glaubach T, Robinson L.J, Corey S.J Pediatric Myelodysplastic Syndromes: They Do Exist! J Pediatr Hematol Oncol. 2014;36:1-7. DOI: 10.1097/MPH.0000000000000046

11. Elghetany TM. Myelodysplastic syndromes in children: a critical review of issues in the diagnosis and classification of 887 cases from 13 published series. Arch Pathol Lab Med. 2007;131:1110-6.

12. Cîrstea M, Coliță A, Ionescu B, Ghiaur A, Vasilescu D, et al. Therapy-related myelodysplastic syndrome after successful treatment of acute promyelocytic leukemia: case report and literature review. Rev Romana Med Lab. 2017;25(2):165-79. DOI: 10.1515/rrlm-2017-0013

13. Camitta BM. Diseases of blood: Splenomegaly. Kliegman RM. Behrman RE, Jenson HB, Waldo E, Nelson Textbook of Pediatrics. 18th edition, Philadelphia, Elsevier, 2008, 2091-2.

14. Leguit RJ, Van Den Tweel J.G. The pathology of bone marrow failure. Histopathology. 2010;57(5):655-670. DOI: 10.1111/j.1365-2559.2010.03612.x

15. Bănescu C, Benedek I, Duicu C, Demian S, Voidăzan S. Cytogenetic findings and their prognostic impact in myelodysplastic syndrome patients. Rev Romana MedLab. 2011;19(2):139-47.

16. Della Porta MG, Malcovati L. Myelodysplastic syndromes with bone marrow fibrosis, Haematol Hematol J. 2011;96:180-3. DOI: 10.3324/haematol.2010.039875

17. Geddes AD, Bowen DT, Jacobs A. Clonal karyotype abnormalities and clinical progress in the myelodysplastic syndrome, Br J Haematol. 1990;76(2):194-202. DOI: 10.1111/j.1365-2141.1990.tb07871.x

18. Bernasconi P, Klersy C, Boni M. Does cytogenetic evolution have may any prognostic relevance in myelodysplastic syndromes? Ann Hematol. 2010;89:545-551. DOI: 10.1007/s00277-010-0927-z

19. Koppel A, Schiller G. Myelodysplastic syndrome: an update on diagnosis and therapy. Curr Oncol Rep. 2008 10(5):372-378. DOI: 10.1007/s11912-008-0058-z

20. Xiaojun KS, Xu Min L. Hypocellular myelodysplastic syndrome with myelofibrosis in acute myeloid leukemia transformation: A case report. Oncol Lett. 2015;10:422-4.

Revista Romana de Medicina de Laborator

Romanian Journal of Laboratory Medicine

Journal Information


IMPACT FACTOR 2017: 0.400
5-year IMPACT FACTOR: 0.320



CiteScore 2017: 0.31

SCImago Journal Rank (SJR) 2017: 0.144
Source Normalized Impact per Paper (SNIP) 2017: 0.195

Metrics

All Time Past Year Past 30 Days
Abstract Views 0 0 0
Full Text Views 220 220 15
PDF Downloads 91 91 7