Publication: Diagnostic differentiation of essential thrombocythaemia from thrombocythaemias
associated with chronic idiopathic myelofibrosis
by discriminate analysis of bone marrow features - a
clinicopathological study on 272 patients
Authors
Thiele, J. ; Kvasnicka, H.M.
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Publisher
Murcia : F. Hernández
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DOI
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info:eu-repo/semantics/article
Description
Abstract
Until now diagnosis of essential
thrombocythaemia (ET) is generally performed by
following the criteria of the Polycythaemia Vera Study
Group (PVSG) that only marginally regards
morphological features. Bone marrow biopsies were
studied from 272 patients with ET in strict accordance
with the PVSG guidelines and also from 35 control
patients with reactive thrombocytosis. To define
morphological features of distinctive impact more
accurately, we performed a stepwise discriminant
analysis of 16 morphological parameters based on
histochemical staining reactions and semiquantitative
grading of standardized features. A clear-cut separation
into three distinctive histological patterns was
accomplished that showed in more than 96% a correct
predicted classification. Variables of significant impact
included fibre content, quantity and cytological
abnormalities of megakaryopoiesis like bulbous (cloudlike)
nuclei, degree of nuclear lobulation and presence of
giant forms. These changes were not detectable in the
control group. The different constellations of
histopathological features could be assigned to true ET
(98 patients) and false ET, i.e. 136 patients with
prefibrotic and 38 patients with early fibrotic chronic
idiopathic myelofibrosis (IMF) accompanied by
thrombocythaemia. A re-evaluation of clinical findings
was in keeping with this classification into three
categories that exerted significant differences to develop myelofibrosis during observation time and also different
survival patterns. Contrasting IMF true ET is
characterized by a pronounced proliferation of the
megakaryocyte lineage showing large to giant cells
without maturation defects and no relevant increase in
reticulin fibres. Discrimination between these entities is
warranted, because of a significant difference in presenting haematological data, follow-up and life
expectancy.
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