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Myelodysplasia and Myeloid Proliferations

  • Zeba N. SinghEmail author
  • Margaret L. Gulley
Chapter
  • 836 Downloads
Part of the Molecular and Translational Medicine book series (MOLEMED)

Abstract

Myelodysplastic syndrome (MDS) and myeloproliferative disorders are uncommon in the pediatric age group, each constituting less than 5 % of all hematological malignancies. The annual incidence of MDS per million for children up to 14 years of age is 1.8. There are several differences in MDS observed in children and those in adults (discussed later) due to which the classification systems developed for adult MDS (Leukemia 18(12):2008–2014, 2004; Blood 100(7):2292–2302, 2002) are less relevant in children. In the 2008 WHO classification childhood MDS is classified as a separate group. A provisional category of refractory cytopenia of childhood has been introduced to accommodate the significant proportion of children who present with persistent, idiopathic neutropenia or thrombocytopenia in the absence of anemia (Pathology and genetics of tumours of haematopoietic and lymphoid tissues. World Health Organization classification of tumours, 2008). MDS associated with Down syndrome is categorized separately by the WHO as “myeloid proliferations related to Down syndrome.” Philadelphia chromosome positive chronic myelogenous leukemia is rare in children, comprising 1–3 % of all childhood leukemias (Pediatrics 116(1):140–143, 2005). Juvenile myelomonocytic leukemia is a MDS/MPN overlap neoplasm that occurs in children and adolescents with an annual incidence of 1.2 per million children up to 14 years of age.

Keywords

JMML Refractory cytopenia Down syndrome Transient myeloproliferative disorder Acute megakaryoblastic leukemia 

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Copyright information

© Springer Science+Business Media New York 2012

Authors and Affiliations

  1. 1.Department of PathologyUniversity of Arkansas for Medical SciencesLittle RockUSA
  2. 2.Department of Pathology and Laboratory MedicineUniversity of North Carolina at Chapel HillChapel HillUSA

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