Life expectancy in primary myelodysplastic syndromes: A prognostic score based upon histopathology from bone marrow biopsies of 569 patients

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Abstract
Abstract:  The retrospective evaluation of bone marrow biopsies of 569 patients with 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 chronic myelo‐monocytic leukemias ‐ CMMOL ‐ according to FAB‐criteria, 23 patients were not otherwise specified (myelodysplastic syndrome: not otherwise specified ‐ MDS.NOS ‐). RARS‐patients had the best prognosis (median survival 41.9 months, incidence of leukemia 3.8%), followed by 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, incidence 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 parameters, among which the three most important ones were determined: quantity of myeloblasts, myelofibrosis and ALIP's. The scoring system allows a determination of three risk groups with significantly different survival times. It is valid also for patients without increase of myeloblasts (<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 without regarding further methods herewith presenting a system which could be considered independently from hematologic, cytological or laboratory data.