Publication: Myelofibrosis in chronic myeloproliferative disorders - dynamics and clinical impact
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Date
2006
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
In chronic myeloproliferative disorders,
presenting or evolving myelofibrosis (MF) is associated
with significant morbidity and mortality. A
systematically conducted evaluation of previous studies
and data from our own material reveals a strikingly
expressed heterogeneity of findings. Assessment of MF
should be performed by a recently established
semiquantitative scoring system regarding quantity and
quality (reticulin versus collagen). It is important to
differentiate between a fiber increase in bone marrow
specimens and the clinical diagnosis that is explicitly
based on extramedullary hematopoiesis (myeloid
metaplasia). For this reason, prodromal stages of
(reticulin) fibrosis are overlooked by the clinicians. Up
to 30% of patients with chronic myelogenous leukemia
show a minimal to advanced MF that is significantly
associated not only with corresponding clinical
parameters but more importantly with prognosis. In
polycythemia vera about 20% of patients may display
some degree of reticulin fibrosis at diagnosis, depending
on stage of the disease. Transformation into (collagen)
MF after more than 10 years is accompanied by clinical
signs of myeloid metaplasia (spent phase). Essential
thrombocythemia (ET) is characterised by the absence of
increased reticulin at onset and an insignificant
progression into MF, provided diagnosis is performed
by the WHO criteria. Discrimination of prefibrotic and
early stages of chronic idiopathic myelofibrosis (CIMF)
from ET is relevant, especially concerning the rate and
time usually required for the development of MF with
myeloid metaplasia (full-blown CIMF).
In conclusion, more elaborate evaluations including
standardized grading of MF is warranted by regarding
bone marrow biopsy specimens in association with
clinical parameters including follow-up examinations.
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