title: Clinical impact of bone marrow morphology for the diagnosis of essential thrombocythemia: comparison between the BCSH and the WHO criteria.
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.
authors.affiliation: Division of Hematology and Hemostaseology, Department of Internal Medicine I, Medical University of Vienna, Vienna, Austria.
authors.forename: H
authors.initials: H
authors.lastname: Gisslinger
authors.affiliation: Division of Hematology and Hemostaseology, Department of Internal Medicine I, Medical University of Vienna, Vienna, Austria.
authors.forename: G
authors.initials: G
authors.lastname: Jeryczynski
authors.affiliation: Division of Hematology and Hemostaseology, Department of Internal Medicine I, Medical University of Vienna, Vienna, Austria.
authors.forename: B
authors.initials: B
authors.lastname: Gisslinger
authors.affiliation: Division of Hematology, Department of Internal Medicine, Medical University of Graz, Graz, Austria.
authors.forename: A
authors.initials: A
authors.lastname: Wölfler
authors.affiliation: Department of Internal Medicine IV, Klinikum Wels-Grieskirchen, Wels, Austria.
authors.forename: S
authors.initials: S
authors.lastname: Burgstaller
authors.affiliation: Department of Internal Medicine I, Elisabethinen Hospital Linz, Linz, Austria.
authors.forename: V
authors.initials: V
authors.lastname: Buxhofer-Ausch
authors.affiliation: Division of Hematology and Hemostaseology, Department of Internal Medicine I, Medical University of Vienna, Vienna, Austria.
authors.forename: M
authors.initials: M
authors.lastname: Schalling
authors.affiliation: Division of Hematology and Hemostaseology, Department of Internal Medicine I, Medical University of Vienna, Vienna, Austria.
authors.forename: M-T
authors.initials: MT
authors.lastname: Krauth
authors.affiliation: Clinical Institute of Pathology, Medical University of Vienna, Vienna, Austria.
authors.forename: A-I
authors.initials: AI
authors.lastname: Schiefer
authors.affiliation: Clinical Institute of Pathology, Medical University of Vienna, Vienna, Austria.
authors.forename: C
authors.initials: C
authors.lastname: Kornauth
authors.affiliation: Clinical Institute of Pathology, Medical University of Vienna, Vienna, Austria.
authors.forename: I
authors.initials: I
authors.lastname: Simonitsch-Klupp
authors.affiliation: Institute of Pathology, Medical University of Graz, Graz, Austria.
authors.forename: C
authors.initials: C
authors.lastname: Beham-Schmid
authors.affiliation: Clinical Institute of Pathology, Medical University of Vienna, Vienna, Austria.
authors.forename: L
authors.initials: L
authors.lastname: Müllauer
authors.affiliation: Institute of Pathology, University of Cologne, Cologne, Germany.
authors.forename: J
authors.initials: J
authors.lastname: Thiele
mesh_terms: D000047:Academies and Institutes; D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001706:Biopsy; D001853:Bone Marrow / Q000473:pathology*; D001856:Bone Marrow Examination; D006801:Humans; D007770:L-Lactate Dehydrogenase / Q000097:blood; D008875:Middle Aged; D017410:Practice Guidelines as Topic / Q000592:standards*; D011379:Prognosis; D013163:Splenomegaly; D013920:Thrombocythemia, Essential / Q000175:diagnosis*; D014944:World Health Organization; D055815:Young Adult
chemical_list: D007770:L-Lactate Dehydrogenase