Meral TÜRKER, Berna ATABAY, Haldun ÖZİZ, HASAN KILIÇ, Çiğdem ALTAY

Eritroid hipoplazi gelişen bir pediatrik myelodisplastik sendrom olgusu: Olgu sunumu

Myelodisplastik sendrom (MDS), displastik ve inefektif kan hücre üretimi ile giden, akut lösemiye dönüşüm riski yüksek multipotent kök hücre bozukluğu ile karakterize bir hastalık grubudur. Çocukluk çağında nadirdir ve sitogenetik anomaliler tanı ve prognoz açısından önem taşımaktadır. Kliniğimizde refrakter anemi ile giden MDS (MDS-RA) tanısı alan 5 yaşındaki kız olgu, 45 XO sitogenetik anomalisi ve izlemde gelişen eritroid hipoplazisi nedeni ile sunulmuştur.

Anahtar Kelimeler:

Tanı, ayırıcı, Miyelodisplastik sendromlar, Kromozom sapmaları, Çocuk, okul öncesi, Anemi, tedaviye dirençli

Pediatric myelodyspalstic syndrome with erythroid hypoplasia: Case report

The myelodysplastic syndromes (MDS) are heterogeneous group of stem cell malignancies characterized by dysplastic and ineffective blood cell production and with a variable risk of transformation to acute leukemias. In children, these diseases are rare and cytogenetic abnormalities have been a cornerstone for characterization of MDS. We report the first pediatric with MDS-RA who developed progressive red cell hypoplasia and had XO cytogenetic abnormalities.

Keywords:

Diagnosis, Differential, Myelodysplastic Syndromes, Chromosome Aberrations, Child, Preschool, Anemia, Refractory,

___

1. Grier HE, Ovın CI. Myeloid Leukemias, Myelodysplasia, and Myeloproliferative Disease in Children. In Nathan DG, Oski FA, eds. Hematology of Infancy and Childhood, 5 th Edition: WB Saunders 1998;1286-1313.
2. Hasle H, Wadsworth LD, Massing BG, et al. A population based study of childhood myelodisplastic syndrome in British Columbia, Canada. Br J Hematol 1999;106:1027-1032.
3. Tuncer MA, Pagliuca A, Hiçsönmez G, et al. Primary myelodysplastic syndrome in children: the clinical experience in 33 cases. Br J Hematol 1992;8:347-353.
4. Sueiu S, Ruse R, Web. HJ, et al. Results of chromosome studies and their relation to morphology, course and prognosis in 120 patients with de novo myelodysplastic syndrome. Cancer Cytogenet 1990;44:15-26.
5. Nichols K, Parson SK, Guinan E. Long term follow-up 12 pediatric patients with primary myelodysplastic syndrome treated with HLA- identical sibling donor bone marrow transplantation. Blood 1996;87:4020-4022.
6. Kardos G, Baumann I, Fenu S, et al. Refractory anemia in childhood: a study of the European working group of MDS in childhood (EWOG-MDS). Leukemia 2000; 14:967a.
7. Park S, Merlat A, Guesnu M, et al. Pure red cell aplasia associated with myelodysplastic syndromes. Leukemia 2000;14:1709-1710.
8. Saurez JG, Pascual T, Munoz MA, et al. Myelodysplastic syndrome with erythroid hypoplasia/ aplasia: a case report and review of the literature. Am J Haematol 1998;58:319-325.
9. Novitzky N, Prindull G. Myelodysplastic syndromes in children. A critical review of the clinical manifestations and management. Am J Haematol 2000;63:212-222.
10. Williamson PJ, Oscier DG, Bell AJ, et al. Red cell aplasia in myelodysplastic syndrome. J Clin Pathol 1991;44:431-432.
11. Passmore SJ, Hann IM, Stiller CA, et al. Pediatric myelodysplasia: a study of 68 children and a new prognostic scoring system. Blood 1995;85:1742-1750.
12. Bader-Meunier B. Mielot F, Tchernia G, et al. Myelodysplastic syndromes in chilhood: report of 49 patients from a French multicentre study. Br J Haematol 1996;92:344-350.
13. Emanuel PD. Review. Myelodysplasia and myeloproliferative disorders in chilhood: an update. Br J Haematol 1999;105:852-863.
14. Luna-Fineman S, Shannon KM, Atwater SK, et al. Myelodysplastic and myeloproliferative disorders of childhood: a study of 167 patients. Blood;93:459-466.
15. Abruzzese E, Rao PN, Slatkoff M, et al. Monosomy X as a recurring sole cytogenetic abnormality associated with mylodysplastic disease. Cancer Genet Cytogenet 1997;93:140-146.
16. Foster LA, Abboud MR, Taylor AB, et al. Myelodysplasia associated with Turner syndrome. J Pediatr Hematol Oncol 1996;! 8: 299-301.