9985/3 Refractory cytopenia with multilineage dysplasia


Definitions

Tumours of haematopoietic and lymphoid tissues
ICD-O-3 topography code: C42 and C77
ICD10: D46.5
           

Refractory cytopenia with multilineage dysplasia (RCMD) is a type of myelodysplastic syndrome (MDS) with one or more cytopenias and dysplastic changes in two or more of the myeloid lineages: erythroid, granulocytic, megakaryocytic 1
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Rosati S, Mick R, Xu F, Stonys E, Le Beau MM, Larson R, Vardiman JW (1996)
Refractory cytopenia with multilineage dysplasia: further characterization of an 'unclassifiable' myelodysplastic syndrome.
Leukemia 10: 20-6



. There are <1% blasts in the peripheral blood (PB) and <5% in the bone marrow (BM); Auer rods are not present and the monocytes in the PB are less than 1x109/L. The recommended levels for defining cytopenias are haemoglobin <10g/dL, absolute neutrophil count <1.8x109/L and platelet count <100x109/L 2
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Greenberg P, Cox C, LeBeau MM, Fenaux P, Morel P, Sanz G, Sanz M, Vallespi T, Hamblin T, Oscier D, Ohyashiki K, Toyama K, Aul C, Mufti G, Bennett J (1997)
International scoring system for evaluating prognosis in myelodysplastic syndromes.
Blood 89: 2079-88



3
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Greenberg P, Cox C, LeBeau MM, Fenaux P, Morel P, Sanz G, Sanz M, Vallespi T, Hamblin T, Oscier D, Ohyashiki K, Toyama K, Aul C, Mufti G, Bennett J (1997)
International scoring system for evaluating prognosis in myelodysplastic syndromes.
Blood 89: 2079-88



. However, values in excess of these thresholds are not exclusionary of a diagnosis of MDS if definitive morphologic and/or cytogenetic findings are consistent with a diagnosis, e.g. complex cytogenetic abnormalities. The thresholds for dysplasia are ≥10% in each of the affected cell lines. In assessing dysplasia it is recommended that 200 neutrophils and precursors and 200 erythroid precursors be evaluated in smear and/or trephine imprint preparations. The neutrophil dysplasia may be evaluated in PB or BM smears. At least 30 megakaryocytes should be evaluated for dysplasia in BM smears or sections. In some cases, dysplastic megakaryocytes may be more readily identified in sections than smears. In particular the presence of micro?megakaryocytes should be noted. Cases with multilineage dysplasia and 2−4% blasts in the PB, <5% in the BM, and no Auer rods should be classified as refractory anemia with excess blasts(RAEB)-1; cases with 1% blasts or fewer in the PB and <5% blasts in the BM, and Auer rods should be classified as RAEB-2; cases with 1% blasts in the PB and <5% in the BM and no Auer rods should be classified as myelodysplastic syndrome, unclassifiable (MDS-U). Some cases of RCMD have ≥15% ring sideroblasts 4
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Germing U, Strupp C, Kuendgen A, Isa S, Knipp S, Hildebrandt B, Giagounidis A, Aul C, Gattermann N, Haas R (2006)
Prospective validation of the WHO proposals for the classification of myelodysplastic syndromes.
Haematologica 91: 1596-604



5
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Malcovati L, Porta MG, Pascutto C, Invernizzi R, Boni M, Travaglino E, Passamonti F, Arcaini L, Maffioli M, Bernasconi P, Lazzarino M, Cazzola M (2005)
Prognostic factors and life expectancy in myelodysplastic syndromes classified according to WHO criteria: a basis for clinical decision making.
J Clin Oncol 23: 7594-603



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