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Myelodysplasia

 

 

Classification (MDS)

Three main groups of myelodysplastic and myeloproliferative disorders in children:

 

Myelodysplastic and myeloproliferative neoplasms in children:

Myelodysplastic and myeloproliferative neoplasms Juvenile Myelomonocytic Leukemia (JMML)

Down Syndrome Disease (DS)

Transient Abnormal Myelopoiesis of Down Syndrome (TAM-DS)

 

Myeloid Leukemia of DS

 

Myelodyspastic Syndrome/Neoplasm(MDS)

Refractory Cytopenia of Childhood (RCC):

  • Also called Refractory anemia
  • Peripheral blood (PB) blasts <2% and
  • Bone Marrow (BM) blasts <5% 
  • Usually hypocellular bone marrow

 

Refractory Anemia with excess blasts (RAEB):

  • PB blasts 2 - 19% or
  • BM blasts 5 - 19%

 

 

Myeloid leukemia in children with Down Syndrome is a unique and a separate entity.

 

Primary vs Secondary MDS

  • MDS arising in a previously healthy child is called primary or "de novo".
  • MDS arising in a child with a predisposing condition is called secondary.

 

Differential Diagnosis

Disorders which may present with similar morphological features include:

  • Viral infections (eg. CMV, HHV, parvovirus)
  • Vitamin deficiencies (B12, folate, E)
  • PNH
  • Other disorders:
    • Rheumatological
    • Metabolic
    • Autoimmune

 

 

 

 

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