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WHO Classification of Tumours
Overview: Childhood myelodysplastic syndrome


Myelodysplastic syndrome (MDS) is very uncommon in children, accounting for less than 5% of all haematopoietic neoplasms in patients less than 14 years of age (Table 5.06)
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Hasle H, Niemeyer CM, Chessells JM, Baumann I, Bennett JM, Kerndrup G, Head DR (2003)
A pediatric approach to the WHO classification of myelodysplastic and myeloproliferative diseases.
Leukemia 17: 277-82




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Hasle H, Wadsworth LD, Massing BG, McBride M, Schultz KR (1999)
A population-based study of childhood myelodysplastic syndrome in British Columbia, Canada.
Br J Haematol 106: 1027-32




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Niemeyer CM, Baumann I (2008)
Myelodysplastic syndrome in children and adolescents.
Semin Hematol 45: 60-70




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Starý J, Baumann I, Creutzig U, Harbott J, Michalova K, Niemeyer C (2008)
Getting the numbers straight in pediatric MDS: distribution of subtypes after exclusion of down syndrome.
Pediatr Blood Cancer 50: 435-6



. The de novo or primary form of MDS in children should be distinguished from cases of "secondary MDS" that follow congenital or acquired bone marrow (BM) failure syndromes and from therapy- related MDS that follows cytotoxic therapy for a previous neoplastic or non-neoplastic condition. Furthermore, although MDS associated with Down syndrome has been reported to account for 20-25% of cases of childhood MDS in the past
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Starý J, Baumann I, Creutzig U, Harbott J, Michalova K, Niemeyer C (2008)
Getting the numbers straight in pediatric MDS: distribution of subtypes after exclusion of down syndrome.
Pediatr Blood Cancer 50: 435-6



, this disorder is now considered as a unique biologic entity synonymous with myeloid leukaemia associated with Down syndrome and distinct from other cases of childhood MDS.

Many of the morphologic, immunophenotypic and genetic features observed in MDS in adults are also seen in childhood forms of the disease but there are some significant differences reported, particularly in patients who do not have increased blasts in their peripheral blood (PB) or BM. For example, the subtypes of refractory anaemia with ring sideroblasts and MDS associated with isolated del (5q) chromosomal abnormality are exceedingly rare in children

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Niemeyer CM, Baumann I (2008)
Myelodysplastic syndrome in children and adolescents.
Semin Hematol 45: 60-70



. Isolated anaemia, which is the major presenting manifestation of refractory anaemia (RA) in adults, is uncommon in children, who are more likely to present with neutropenia and thrombocytopenia
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Hasle H, Niemeyer CM, Chessells JM, Baumann I, Bennett JM, Kerndrup G, Head DR (2003)
A pediatric approach to the WHO classification of myelodysplastic and myeloproliferative diseases.
Leukemia 17: 277-82




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Kardos G, Baumann I, Passmore SJ, Locatelli F, Hasle H, Schultz KR, Starý J, Schmitt-Graeff A, Fischer A, Harbott J, Chessells JM, Hann I, Fenu S, Rajnoldi AC, Kerndrup G, Van Wering E, Rogge T, Nollke P, Niemeyer CM (2003)
Refractory anemia in childhood: a retrospective analysis of 67 patients with particular reference to monosomy 7.
Blood 102: 1997-2003



. In addition, hypo­cellularity of the BM is more commonly observed in childhood MDS than in older patients. Therefore, some children have findings that do not readily fit into the "low grade" MDS categories. To address these differences, a provisional entity, refractory cytopenia of childhood (RCC) is introduced.

For children with MDS in whom there are 2-19% blasts in the PB or 5-19% blasts in the BM, the same criteria utilized for adults with refractory anemia with excess blasts (RAEB) should be applied. Currently, in contrast to adult MDS, there are no available studies that have investigated the prognostic significance of distinguishing RAEB-1 and RAEB-2 in children, but it is recommended that this distinction be made for future investigation. Children with RAEB generally have relatively stable PB counts for weeks or months. Some cases diagnosed in children as acute myeloid leukaemia (AML) with 20-29% blasts in the PB and/or BM that have myelodysplasia-­related changes, including cases with myelodysplasia-related cytogenetic abnormalities may also have slowly progressive disease. These cases, considered as refractory anaemia with excess blasts in transformation in the French-American-British cooperative classification, may lack the clinical features of acute leukaemia and behave more like MDS than AML

Click to access Pubmed
Hasle H, Niemeyer CM, Chessells JM, Baumann I, Bennett JM, Kerndrup G, Head DR (2003)
A pediatric approach to the WHO classification of myelodysplastic and myeloproliferative diseases.
Leukemia 17: 277-82



, thus follow-up PB and BM studies are often necessary to measure the pace of the disease in such cases. Children who present with a PB and/or BM disorder associated with t(8;21)(q22;q22), inv(16)(p13.1q22) or t(16;16)(p13.1;q22) or t(15;17)(q22;q12) should be considered to have AML regardless of the blast count.







Irith Baumann
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Irith Baumann
Institute of Pathology
Bayreuth Clinic
Bayreuth
GERMANY




Charlotte M. Niemeyer
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Charlotte M. Niemeyer
Department of Pediatrics and Adolescent Medicine
University of Freiburg
Freiburg
Germany




John M. Bennett
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John M. Bennett
Departments of Medicine and Pathology
University of Rochester Medical Center and the James P. Wilmot Cancer Center
Rochester
USA




Kevin Shannon
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Kevin Shannon
University of California at San Francisco
San Francisco
USA