Thrombosis in essential thrombocytemia and early/prefibrotic primary myelofibrosis: the role of the WHO histological diagnosis

Diagnostic Pathology

Pietro Leoni²  Elisa Honorati²  Antonio Zizzi¹  Lidia Da Lio³  Federica Giantomassi¹  Irene Federici²  Anna Rita Scortechini²  Lucia Canafoglia²  Giorgia Micucci²  Paola Picardi²  Gaia Goteri¹  Serena Rupoli² 

[1] Department of Biomedical Sciences and Public Health, Section of Pathologic Anatomy and Histopathology, Polytechnic University of Marche Region, Torrette, Ancona, Italy;Department of Clinical and Molecular Sciences, Clinic of Hematology, Polytechnic University of Marche Region, Ancona, Italy;Clinical Pathology, United Ancona Hospital, Ancona, Italy

关键词: Histopathology interpretation;    Vascular events;    Prefibrotic/early primary myelofibrosis;    Essential thrombocytemia;   

Others  :  1217780
DOI  :  10.1186/s13000-015-0269-1

 received in 2014-09-30, accepted in 2015-04-07,  发布年份 2015

【 摘 要 】

Background

Vascular events represent the most frequent complications of thrombocytemias. We aimed to evaluate their risk in the WHO histologic categories of Essential Thrombocytemia (ET) and early Primary Myelofibrosis (PMF).

Methods

From our clinical database of 283 thrombocytemic patients, we selected those with available bone marrow histology performed before any treatment, at or within 1 year from diagnosis, and reclassified the 131 cases as true ET or early PMF, with or without fibrosis, according to the WHO histological criteria. Vaso-occlusive events at diagnosis and in the follow-up were compared in the WHO-groups.

Results

Histologic review reclassified 61 cases as ET and 72 cases as early PMF (26 prefibrotic and 42 with grade 1 or 2 fibrosis). Compared to ET, early PMF showed a significant higher rate of thrombosis both in the past history (22% vs 8%) and at diagnosis (15.2% vs 1.6%), and an increased leukocyte count (8389 vs 7500/mmc). Venous thromboses (mainly atypical) were relatively more common in PMF than in ET. Patients with prefibrotic PMF, although younger, showed a significant higher 15-year risk of developing thrombosis (48% vs 16% in fibrotic PMF and 17% in ET). At multivariate analysis, age and WHO histology were both independent risk-factors for thrombosis during follow-up; patients >60 yr-old or with prefibrotic PMF showed a significantly higher risk at 20 years than patients <60 yr-old with ET or fibrotic PMF (47% vs 4%, p = 0.005).

Conclusions

Our study support the importance of WHO histologic categories in the thrombotic risk stratification of patients with thrombocytemias.

Virtual slides

The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/2020211863144412 webcite.

【 授权许可】

   
2015 Rupoli et al.; licensee BioMed Central.

【 图 表 】

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