Bone marrow neoplastic
Bone marrow - neoplastic myeloid
Myelodysplastic syndromes / neoplasms (MDS)
MDS in childhood
Author: Nikhil Sangle, M.D.
Last author update: 1 August 2011
Last staff update: 19 June 2020
Copyright: 2002-2024, PathologyOutlines.com, Inc.
PubMed Search: Childhood MDS
Cite this page: Sangle N. MDS in childhood. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/myeloproliferativechildhoodmds.html. Accessed April 18th, 2024.
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:
- Less dysplasia at younger ages
- Dysplasia may be confused with treatment effects of G-CSF
- Bone marrow often hypocellular, resembling aplastic anemia
- 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:
- Sustained and unexplained cytopenia
- At least bilineage dysplasia
- A clonal cytogenetic abnormality
- At least 5% blasts (Leukemia 2003;17:277); may be most successful system for classification (Arch Pathol Lab Med 2007;131:1110)
- Diagnosis requires:
- 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%
