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Chronic Myeloid Neoplasms

Myelodysplastic syndromes

Childhood MDS

Reviewer: Nikhil Sangle, M.D., University of Utah & ARUP Laboratories (see Reviewers page)
Revised: 8 August 2011, last major update August 2011
Copyright: (c) 2001-2011, PathologyOutlines.com, Inc.

Clinical features
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● 3-9% of pediatric hematologic malignancies
● Estimated annual incidence in US is 0.5 to 4 cases per million for children compared to 20-40 cases for adults
● Usually diagnosed at ages 6-8 years, although juvenile myelomonocytic leukemia is usually diagnosed at age 2 years
● Difficult to diagnose because (a) less dysplasia at younger ages, (b) dysplasia may be confused with treatment effects of G-CSF, (c) bone marrow often hypocellular, resembling aplastic anemia, (d) resembles HHV6 (Pediatr Blood Cancer 2006;47:543) or parvovirus infections (Rinsho Ketsueki 2001;42:1096, Pediatr Hematol Oncol 2000;17:475)
● Modified WHO classification for childhood MDS: diagnosis requires: (a) sustained and unexplained cytopenia, (b) at least bilineage dysplasia, (c) a clonal cytogenetic abnormality, and (d) at least 5% blasts (Leukemia 2003;17:277); may be most successful system for classification (Arch Pathol Lab Med 2007;131:1110)
● 69% idiopathic, 24% associated with constitutional/inherited disorders (Down’s syndrome, neurofibromatosis, Bloom’s syndrome, Fanconi’s anemia, Atlas of Genetics and Cytogenetics), 7% therapy related (Arch Pathol Lab Med 2007;131:1110)
● Similar prognosis as AML M6 and M7, with poor induction success rate (Pediatr Blood Cancer 2007;49:17)

Molecular
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● Monosomy 7 in 30%

End of Chronic Myeloid Neoplasms > Myelodysplastic syndromes > Childhood MDS

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