Chronic Myeloid Neoplasms
Myelodysplastic syndromes
Childhood MDS

Author: Nikhil Sangle, M.D. (see Authors page)

Revised: 1 March 2017, last major update August 2011

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PubMed Search: Childhood MDS

Cite this page: Childhood MDS. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/myeloproliferativechildhoodmds.html. Accessed July 26th, 2017.
Clinical features
  • 3 - 9% of pediatric hematologic malignancies
  • Estimated annual incidence in U.S. 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:
    1. Less dysplasia at younger ages
    2. Dysplasia may be confused with treatment effects of G-CSF
    3. Bone marrow often hypocellular, resembling aplastic anemia
    4. 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
  • 69% idiopathic, 24% associated with constitutional / inherited disorders (Down syndrome, neurofibromatosis, Bloom syndrome, Fanconi 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 / cytogenetics description
  • Monosomy 7 in 30%