Refractory cytopenia of childhood

Refractory cytopenia of childhood (RCC) is a subgroup of myelodysplastic syndrome (MDS), having been added to the World Health Organization classification in 2008. Before then, RCC cases were classified as childhood aplastic anemia.[1][2] RCC is the most common form of MDS in children.[3]

Clinical features

Symptoms result from underproduction of red blood cells (weakness, pallor, failure to thrive, pica), white blood cells (recurrent or overwhelming infection), and/or platelets (bleeding).

Bone marrow transplant is the only known curative treatment.

Histopathologic features

The bone marrow of patients with RCC contains islands of erythroid precursors and spare granulocytes.

References

  1. ↑ Niemeyer, C. M.; Baumann, I (2011). "Classification of childhood aplastic anemia and myelodysplastic syndrome". Hematology 2011: 84–9. doi:10.1182/asheducation-2011.1.84. PMID 22160017.
  2. ↑ "Medscape Pediatric Myelodysplasia". Retrieved 16 June 2015.
  3. ↑ Niemeyer, C. M.; Baumann, I (2008). "Myelodysplastic syndrome in children and adolescents". Seminars in Hematology 45 (1): 60–70. doi:10.1053/j.seminhematol.2007.10.006. PMID 18179970.
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