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Abstract
Background: Myelodysplastic Syndromes (MDS) are typically characterized by peripheral blood cytopenias. However, some MDS is associated with thrombocytosis rather than thrombocytopenia. The prognostic significance of thrombocytosis is unknown. The purpose of this study is to determine if thrombocytosis predicts Overall Survival (OS). Methods: We reviewed a prospective cohort of patients with myeloid disorders from 1994 to July 2011. The median duration of observation was 6.3 years. The cohort included 5,886 bone marrow biopsies or aspirates from 2,042 patients. OS of patients with thrombocytosis was compared to WHO case matched patients with normal (100-450,000) and low platelet counts (<100,000). All-cause mortality was evaluated using Cox proportional hazard regression modeling and Kaplan Meier methods. Results: Thrombocytosis occurred in 5% (102/2,042) of patients throughout their disease. Platelet counts remained stable in 86% of patients (1,593/1,856) and thrombocytosis was constant in 93% of patients (86/92). Patients with thrombocytosis had significantly decreased OS compared to those with normal platelet counts in multivariate analysis adjusted for age and IPSS score (P< 0.035). Patients with normal platelet counts had the best prognosis and patients whose platelet counts changed during their disease course had the worst survival (p< 0.0009). Conclusions: MDS patients with thrombocytosis have significantly decreased OS compared to those with normal platelet counts. The IPSS score does not accurately predict their prognosis.
Highlights
Myelodysplastic Syndromes (MDS) are typically characterized by peripheral blood cytopenias
Multiple studies have demonstrated that thrombocytopenia at diagnosis is associated with worse Overall Survival (OS) [1,2,3,4,5]
MDS subtypes associated with thrombocytosis include: 1) Deletion 5q 2) Refractory anemia with ringed sideroblasts and thrombocytosis (RARS-T) and 3) 3q21.q26 syndrome that affects the thrombopoietin gene [6]
Summary
Myelodysplastic Syndromes (MDS) are typically characterized by peripheral blood cytopenias. The clinical significance of MDS that presents with thrombocytosis is unknown. Thrombocytosis is rare with an 8% incidence [7] in the largest previously reported case series that included 31 patients with MDS and platelets > 400,000. Thrombocytosis in this series was associated with decreased incidence of high grade MDS, equal Acute Myelogenous Leukemia (AML) transformation, with a trend toward longer Overall Survival (OS) compared to patients with normal platelets with similar IPSS and age. The World Health Organization (WHO) 2008 classification defined thrombocytosis in RARS-T as having a platelet count ≥ 450,000 in 2008, decreased from ≥ 600,000 in its 2003 classification. Since the International Prognostic Scoring System (IPSS) includes the number of cytopenias in its scoring system, it may be inaccurate in predicting OS in thrombocytosis
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