LIFE EXPECTANCY IN PRIMARY MYELODYSPLASTIC-SYNDROMES - A PROGNOSTIC SCORE BASED UPON HISTOPATHOLOGY FROM BONE-MARROW BIOPSIES OF 569 PATIENTS

Citation
H. Maschek et al., LIFE EXPECTANCY IN PRIMARY MYELODYSPLASTIC-SYNDROMES - A PROGNOSTIC SCORE BASED UPON HISTOPATHOLOGY FROM BONE-MARROW BIOPSIES OF 569 PATIENTS, European journal of haematology, 53(5), 1994, pp. 280-287
Citations number
24
Categorie Soggetti
Hematology
ISSN journal
09024441
Volume
53
Issue
5
Year of publication
1994
Pages
280 - 287
Database
ISI
SICI code
0902-4441(1994)53:5<280:LEIPM->2.0.ZU;2-Q
Abstract
The retrospective evaluation of bone marrow biopsies of 569 patients w ith primary myelodysplastic syndrome - pMDS - revealed 256 refractory anemias - RA -, 52 refractory anemias with ringed sideroblasts RARS -, 133 refractory anemias with excess of blasts - RAEB -, 52 refractory anemias with excess of blasts in transformation - RAEB-t -, and 53 chr onic myelo-monocytic leukemias - CMMOL - according to FAB-criteria, 23 patients were not otherwise specified (myelodysplastic syndrome: not otherwise specified - MDS.NOS -). BARS-patients had the best prognosis (median survival 41.9 months, incidence of leukemia 3.8%), followed b y RA-patients (26.5 months, 16.4%), MDS.NOS-patients (22.4 months, 21. 7%), CMMOL-patients (12.5 months, 49.1%). RAEB-and RAEB-t-patients had the worst prognosis (median survival time 8.5 and 4.6 months, inciden ce of leukemia 42.1% and 57.7%, respectively). But the survival times showed a considerable range in each FAB-subgroup with 0-154 months in RA or 0-52 months in CMMOL. To forecast life expectancy more precisely , a scoring system was developed using nine histopathological paramete rs, among which the three most important ones were determined: quantit y of myeloblasts, myelofibrosis and ALIP's. The scoring system allows a determination of three risk groups with significantly different surv ival times. It is valid also for patients without increase of myelobla sts (<5% myeloblasts in the bone marrow) and identifies high-risk MDS patients in this group. By this proposed scoring system, a prognostic approval in primary MDS can be achieved applying histopathology withou t regarding further methods herewith presenting a system which could b e considered independently from hematologic, cytological or laboratory data.