Publication: Diagnostic impact of bone marrow
histopathology in polycythemia vera (PV)
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
The criteria of the Polycythemia Vera Study
Group (PVSG), although acknowledged as the gold
standard to establish the diagnosis of polycythemia vera
(PV), do not regard bone marrow (BM) histopathology.
Arguments include the existence of sufficient objective
markers of disease and the lack of independently
performed morphological studies or standardized
criteria. The aim of this review is to evaluate
morphological characteristics of erythrocytosis and to
determine whether distinctive patterns of histopathology
exist. A review of the pertinent literature and evaluation
of 334 patients from our files with a borderline to
marked increase in hemoglobin was performed. In
extension to former descriptions of BM features by the
PVSG, a tri-lineage myeloproliferation (panmyelosis)
with a pleomorphous appearance of megakaryopoiesis
revealed that, besides increase in size, there was a lack
of gross cytological anomalies. Differentiation from
secondary polycythemia (SP) was accomplished by
regarding these features and the conspicuously expressed
stromal changes (plasmacytosis, eosinophils, cell debris
and iron deposits). In about 96% of this cohort a clearcut
separation from SP was achieved, even in the initial
(latent) stages. When accompanied by an elevated
platelet count, these precursor stages may clinically
mimick essential thrombocythemia because they are not
recognized by the conventional criteria. Advanced stages
(spent phases) of PV were consistent with an increased
left-shifted granulocytic proliferation, accompanied by
reduction of erythroid precursors and progressive
myelofibrosis (post-polycythemic myeloid metaplasia).
Finally, an increase in dysplastic changes and immaturity
signalled a transition into blastic crisis.
In conclusion, PV is characterized by a distinctive
pattern of histopathology that has been gained in an
independent and blind fashion and therefore, dissolves
arguments about failing specificity.
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