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13 - Myelodysplastic/myeloproliferative neoplasms

from Section 2 - Neoplastic hematopathology

Published online by Cambridge University Press:  03 May 2011

By
John Kim Choi
Edited by
Maria A. Proytcheva
John Kim Choi
Affiliation:
University of Pennsylvania School of Medicine
Maria A. Proytcheva
Affiliation:
Northwestern University Medical School, Illinois
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Summary

Introduction

Myelodysplastic/myeloproliferative neoplasms are hematopoietic diseases with features of both myelodysplastic syndrome (MDS) and myeloproliferative neoplasm (MPN). In the past, these disorders were grouped as MDS, but they are now grouped as a distinct subentity in the latest classification scheme of hematopoietic neoplasms by the World Health Organization (WHO) [1, 2]. The MDS/MPN category includes chronic myelomonocytic leukemia (CMML), atypical chronic myeloid leukemia (aCML), juvenile myelomonocytic leukemia (JMML), and myelodysplastic/myeloproliferative neoplasm, unclassifiable. This chapter will focus on JMML, which is unique to the pediatric population. CMML in children is considered a secondary or therapy-related MDS [3], and aCML does not occur in the pediatric population [4].

Juvenile myelomonocytic leukemia (JMML)

Synonyms

Juvenile chronic myelomonocytic leukemia, juvenile chronic myeloid leukemia, juvenile granulocytic leukemia, monosomy 7 syndrome, infantile monosomy 7 syndrome.

Epidemiology

JMML is a rare disorder with an incidence of 0.6 per 1 million children, and represents approximately 1.6% of childhood leukemias [5, 6]. The age of presentation ranges from 0 to 11 years, with 40%, 90%, and 98% of the cases presenting at under 1, 4, and 10 years of age, respectively [7, 8]. Boys are affected more frequently, with a male to female ratio of 2 : 1.

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Diagnostic Pediatric Hematopathology , pp. 245 - 252
Publisher: Cambridge University Press
Print publication year: 2011

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