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Comparative Study
. 2016 May;30(5):1126-32.
doi: 10.1038/leu.2015.360. Epub 2015 Dec 29.

Clinical impact of bone marrow morphology for the diagnosis of essential thrombocythemia: comparison between the BCSH and the WHO criteria

H Gisslinger  1 , G Jeryczynski  1 , B Gisslinger  1 , A Wölfler  2 , S Burgstaller  3 , V Buxhofer-Ausch  4 , M Schalling  1 , M-T Krauth  1 , A-I Schiefer  5 , C Kornauth  5 , I Simonitsch-Klupp  5 , C Beham-Schmid  6 , L Müllauer  5 , J Thiele  7
Affiliations
Comparative Study

Clinical impact of bone marrow morphology for the diagnosis of essential thrombocythemia: comparison between the BCSH and the WHO criteria

H Gisslinger et al. Leukemia. 2016 May.

Erratum in

Abstract

Essential thrombocythemia (ET) is currently diagnosed either by the British Committee of Standards in Haematology (BCSH) criteria that are predominantly based on exclusion and not necessarily on bone marrow (BM) morphology, or the World Health Organization (WHO) criteria that require BM examination as essential criterion. We studied the morphological and clinical features in patients diagnosed according either to the BCSH (n=238) or the WHO guidelines (n=232). The BCSH-defined ET cohort was re-evaluated by applying the WHO classification. At presentation, patients of the BCSH group showed significantly higher values of serum lactate dehydrogenase and had palpable splenomegaly more frequently. Following the WHO criteria, the re-evaluation of the BCSH-diagnosed ET cohort displayed a heterogeneous population with 141 (59.2%) ET, 77 (32.4%) prefibrotic primary myelofibrosis (prePMF), 16 (6.7%) polycythemia vera and 4 (1.7%) primary myelofibrosis. Contrasting WHO-confirmed ET, the BCSH cohort revealed a significant worsening of fibrosis-free survival and prognosis. As demonstrated by the clinical data and different outcomes between WHO-diagnosed ET and prePMF, these adverse features were generated by the inadvertent inclusion of prePMF to the BCSH group. Taken together, the diagnosis of ET without a scrutinized examination of BM biopsy specimens will generate a heterogeneous cohort of patients impairing an appropriate clinical management.

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Figures

Figure 1
Figure 1
(a–d) Kaplan–Meier estimates of fibrosis-free survival and overall survival with significant differences in 232 WHO-confirmed versus 238 BCSH-defined ET patients (Figures a and b). Separate analysis of the WHO-diagnosed prePMF fraction of 77 patients included in the BCSH-classified ET cohort of 238 patients reveals a significant worsening of fibrosis-free and overall survival (Figures c and d). Abbreviation: CI, confidence interval.
See this image and copyright information in PMC

Comment in

References

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