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
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.