OntologiesncitclassesNCIT:C82596   
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Refractory Cytopenia of Childhood
http://purl.obolibrary.org/obo/NCIT_C82596

A myeloid disorder that occurs in childhood and is characterized by persistent cytopenia, dysplastic changes in at least two hematopoietic lineages or in 10% or more of cells in one hematopoietic lineage, less than 5% blasts in bone marrow, and less than 2% blasts in peripheral blood, absence of bone marrow fibrosis, and absence of history of prior cytotoxic chemotherapy or radiation therapy. In a subset of cases, somatic mutations or cytogenetic abnormalities can be identified. Other cases are the result of pre-existing disorders with germline mutations, including Fanconi anemia, Shwachman-Diamond syndrome, and dyskeratosis congenita.

Exact Synonyms
RCC
Refractory Cytopenia of Childhood
Refractory cytopenia of childhood
class Information
ALT_DEFINITION

The most common subtype of the myelodysplastic syndromes affecting children. It is characterized by persistent cytopenia with less than 5% blasts in the bone marrow and less than 2% blasts in the peripheral blood. Most cases are associated with a normal karyotype, however, when abnormal, monosomy 7 is the most frequently seen cytogenetic abnormality.

code

C82596

Contributing_Source
  • GDC
  • NICHD
ICD-O-3_Code

9985/3

Legacy Concept Name

Refractory_Cytopenia_of_Childhood

Maps_To
  • 9985/3
  • Refractory cytopenia of childhood
Neoplastic_Status

Malignant

Preferred_Name

Refractory Cytopenia of Childhood

Semantic_Type

Neoplastic Process

UMLS_CUI

C2826323

class Relations