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WHO Classification of Tumours
Refractory cytopenia of childhood
Tumours of haematopoietic and lymphoid tissues


Definition

Refractory cytopenia of childhood (RCC) is a myelodysplastic syndrome (MDS) characterized by persistent cytopenia with <5% blasts in the BM and <2% blasts in the PB
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Hasle H, Niemeyer CM, Chessells JM, Baumann I, Bennett JM, Kerndrup G, Head DR (2003)
A pediatric approach to the WHO classification of myelodysplastic and myeloproliferative diseases.
Leukemia 17: 277-82



. Although the presence of dysplasia is required for the diagnosis, the cytological evaluation of dysplasia by itself constitutes only one aspect of the morphological diagnosis of RCC. The evaluation of an adequate BM trephine biopsy specimen is indispensable for the diagnosis of RCC in children. About 75% of children with RCC show considerable hypocellularity of the BM
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Pui CH, Schrappe M, Ribeiro RC, Niemeyer CM (2004)
Childhood and adolescent lymphoid and myeloid leukemia.
Hematology Am Soc Hematol Educ Program : 118-45



. Consequently, it may be very challenging to differentiate hypocellular RCC from other BM failure disorders, especially from acquired aplastic anaemia and inherited BM failure disorders. Myeloid neoplasms associated with Down syndrome are excluded from this diagnosis. The WHO Working Group assigned RCC to a group of provisional entities.