Myelodysplastický syndrom od FAB klasifikacek dnešku. Nové podjednotky či samostatné skupiny?

Myelodysplastic Syndrome from FAB Classification to thePresent Time. New Sub-units or Independent Groups?

The authors present a review of some sub-units of MDS with a specific clinical and laboratory characteristic.The myelodysplastic syndrome (MDS) must be divided into sub-types either according to FABor WHO classification. When establishing the diagnosis it is important to state whether primary orsecondary MDS is involved. Nowadays we can however define the diagnosis of MDS more accuratelyby assessing further sub-units which have their clinicalpicture,prognosisandlaboratory finding.Thesesub-units are divided into three groups: 1. mixed syndromes which comprise hypoplastic MDS andmixed MDS-MPS, 2. cases with a specific histological or morphological picture, i.e. MDS with myelofibrosis,MDSwith eosinophilia,MDS with aggregation of granulocyte chromatin, two forms of sideroblasticanaemia where we can add also idiopathic macrocytosis and finally 3. cases with specificcytogenetic findings such as the 5q minus, syndrome monosomy 7 in children, pathology of chromosome17 (17p-, isol17q) and some less frequent chromosomal aberrations. Knowledge of these sub-units willhelp us to establish the prognosis of the disease and thus also therapy decision.

Key words:
MDS diferentiation, overlap syndromes, morphological aberrations, fibrousis, cytogeneticsub-units