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Refractory anaemia according to the FAB classification: a report on 69 cases

Authors :
Jean-Pierre Jouet
Pascale Lepelley
Marc Zandecki
Pierre Fenaux
Jean-Luc Laï
M.H. Estienne
R. Beuscart
A. Cosson
Francis Bauters
Source :
European journal of haematology. 40(4)
Publication Year :
1988

Abstract

Between 1980 and 1986, we diagnosed refractory anaemia (RA), according to the FAB classification, in 69 patients, who constituted 22% of the 312 cases of myelodysplastic syndromes (MDS) seen over that period. The haematological features were variable, with pancytopenia in 14 cases (20%), bicytopenia in 24 (36%) and mono-cytopenia in the remaining patients, including 21 (30%) cases of anemia alone, 8 (12%) cases of refractory neutropenia and 2 (3%) cases of refractory thrombocytopenia. Myelodysplastic features were also quite variable, involving one, two or all three lineages. In patients with a single cytopenia or only one dysplastic lineage, FAB criteria appeared insufficient for adequate inclusion among RA and we suggest more precise diagnostic criteria, resulting from the utilization of cytogenetics, ferrokinetics, progenitor cultures and perhaps molecular biology, in such cases. Median survival was 42 months. 12 patients (17%) progressed to RAEB (of whom 7 finally developed ANLL) and 4 patients (6%) to CMML. In spite of the heterogeneity of haematological features, only two factors were associated with poor prognosis, namely age greater than 70 yr at diagnosis and haemoglobin less than 10 g/dl, whereas, to a lesser extent, neutropenia was associated with progression to RAEB.

Details

ISSN :
09024441
Volume :
40
Issue :
4
Database :
OpenAIRE
Journal :
European journal of haematology
Accession number :
edsair.doi.dedup.....07299cd6528a4d241aca14f8c331588c