Two regression models and a scoring system for predicting survival and planning treatment in myelodysplastic syndromes: a multivariate analysis of prognostic factors in 370 patients

Abstract

Therapy planning in patients with myelodysplastic syndromes (MDSs) is complicated by its high prognostic heterogeneity. Forty-one patient and disease characteristics at onset of 370 patients with MDS were analyzed to identify significant prognostic factors for survival and transformation to acute myeloblastic leukemia (AML), and to develop and validate a regression model for predicting survival. Multivariate regression analysis showed that the total bone marrow percentage of blast cells, age, platelet count, WBC count, and hemoglobin level were the characteristics more significantly associated with survival in the overall series. The bone marrow percentage of type I blast cells was the most important factor predicting transformation into AML. Proportional hazards regression analysis in a randomly selected training sample of 240 patients demonstrated that the combination of total bone marrow percentage of blast cells, platelet count, and age had the strongest predictive relation to survival length. The resulting regression models, continuous and categorized, were validated in the remaining test sample of 130 patients by demonstrating its capability of segregating patients into low-, intermediate-, and high-risk groups, with distinctively different survival curves (P less than .0001). A scoring system derived from the categorized model also had a great prognostic value (P less than .0001). These regression models and the simpler scoring system may be accurately used for decision-making regarding therapy in MDS patients.

Refractory anaemia with excess of blasts (RAEB): analysis of reclassification according to the WHO proposals

“…According to the Kaplan-Meier estimates, the median survival of patients with RAEB I was 16 months, as compared with 9 months in patients with RAEB II (P ¼ 0AE0031). This is in line with other smaller series (Sanz et al, 1989;Germing et al, 2000;Nosslinger et al, 2001;Lee et al, 2003;Lorand-Metze et al, 2004). Similarly, the risk of developing overt AML was significantly greater for patients with RAEB II versus RAEB I. Irrespective of the RAEB subgroup, there were four independent prognostic parameters for survival, namely age >70 years, haemoglobin concentration <10 g/dl, platelet count <100 · 10 9 /l and a proportion of more than 10% blasts in the bone marrow.…”

“…However, their prognosis is very heterogeneous with respect to survival and risk of leukaemic transformation, probably as a result of the wide range of medullary blast counts. After Spanish investigators had identified this as a shortcoming of the FAB classification (Sanz et al, 1989), several studies underscored the prognostic importance of blast counts (De Rosa et al, 1987;Michels et al, 1989;Chevret et al, 1994;Guerci et al, 1995;Ohyashiki et al, 2001;Strupp et al, 2003). These findings were taken into consideration when a working party of the World Health Organization (WHO) proposed a revised classification of neoplastic diseases of the haematopoietic and lymphoid tissues (Harris et al, 1999).…”

Platelet mass has prognostic value in patients with myelodysplastic syndromes

“…The IPSS (Greenberg et al, 1997) is widely used and incorporates bone marrow blast percentage, bone marrow cytogenetics and number of lineages with cytopenia to provide prognostic information. The IPSS follows on from other prognostic scoring systems that are similar, in so much as they all require bone marrow aspirate ± cytogenetic analysis (Mufti et al, 1985;Sanz et al, 1989;Aul et al, 1992;Morel et al, 1993). Bone marrow aspirate and cytogenetics are commonly, but not always, used in the diagnosis of MDS and may not be required when a definitive diagnosis of MDS would not alter management, for example, in the elderly (Bowen et al, 2003) -the age group with the highest incidence of the disease.…”

A case of CD56+ cutaneous aleukaemic granulocytic sarcoma with myelodysplastic syndrome

“…8 With regard to MDS, transformation into AML occurs in 6±37% of patients. 13 Byrd et al 4 reported that leukaemia cutis and granulocytic sarcoma have rarely been seen in patients with MDS, with only 46 patients previously described, and that 32 cases occurred in the absence of AML. Among these 32 cases, AML developed in 47% at a mean of 38 weeks after the initial diagnosis of granulocytic sarcoma.…”