TORNA ALL’ELENCO

Myelodysplastic syndromes with hypocellular marrow: Clinical characteristics and evaluation of outcome in Blood

2018
ASL Asti
ASL Torino 5
AO Cuneo
AOU Città della Salute di Torino

Tipo pubblicazione

Conference Abstract

Autori/Collaboratori (27)Vedi tutti...

Santini V

Gioia DM

Masiera E


et alii...

Abstract

Background. MDS may be characterized by hypocellular marrow, irrespective of their WHO classification or molecular characteristics. Their prognostic weight must still be completely evaluated. MDS with hypocellular marrow tend to be considered an aplastic anemia “overlap syndrome” or a pre-aplastic anemia stage. There are no strong specific therapy recommendations, and large studies analyzing the outcome of hypocellular MDS are lacking. While selective sensitivity to immunosuppressive therapy is suggested, evidence in this sense is controversial. Aims. We wanted to evaluate the clinical characteristics, outcome and choice of therapy of patients with hypocellular MDS and compare them with normocellular MDS. Methods. We analyzed 2559 MDS cases with complete clinical annotations and with evaluable bone trephine biopsy, enrolled in our Italian National Registry FISMonlus. In this cohort of patients, 438 had a bone marrow cellularity <= 30% and 2121 cellularity above 30%. We proceeded by comparing these two groups in terms of age, gender, WHO classification, IPSS-R categories, overall survival and first line therapies. As a validation cohort, 874 unselected MDS cases enrolled in Rete Ematologica Lombarda (REL) registry were analyzed. In this population, 108 patients had cellularity <= 30%. Results. In FISM cohort median age was 72.5 yrs in the hypocellular group and 72,3 yrs in the normocellular group; M/F were 53.2%/46.8% for hypocellular MDS vs 62.6%/37.4% in normocellular MDS. IPSS-R risk categories were distributed as follows: Hypocellular MDS Very Low 15.5%, Low 35.1%, Intermediate 30.1%, High 11.3%, Very high 8%; Normocellular MDS Very Low 12.8%, Low 37.2%, intermediate 23.7%, High 15.5%, Very High 11.4%. Global median overall survival (OS) was 77 months for hypocellular MDS and 56 months for normocellular MDS. When OS was evaluated in the different IPSS-R risk groups, Lower risk MDS cases with hypocellular BM had a median OS of 125 mos while normocellula

DOI : 10.1182/blood-2018-99-118123

Keywords

validation process; risk assessment; practice guideline; overall survival; myelodysplastic syndrome; male; major clinical study; International Prognostic Scoring System; immunosuppressive treatment; human; high risk population; gender; female; drug therapy; controlled study; conference abstract; cohort analysis; clinical feature; cancer survival; cancer staging; cancer chemotherapy; bone trephine; biopsy; aged; aplastic anemia; immunosuppressive agent; central stimulant agent; azacitidine;