Publication: Therapy-related changes of the bone marrow in chronic idiopathic myelofibrosis
Authors
Thiele, J. ; Kvasnicka, H.M. ; Schmitt-Gräff, A. ; Hülsemann, R. ; Diehl, V.
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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
(CMPDs) a conflict of opinion exists regarding therapyinduced
bone marrow (BM) changes and the evolution
of myelofibrosis during the lengthy course of the
disease. For a more elaborate study of these features
chronic idiopathic myelofibrosis (IMF) seems to be a
most suitable condition. Therefore this review is focused
on this CMPD and amongst other findings analyzes data
from a series of 340 patients with a long follow-up
including 893 biopsies (median interval of 32 months).
The ensuing results were compared with those
communicated in the relevant literature. In addition to a
control group of 153 patients with IMF who received
only symptomatic treatment, therapy groups included
busulfan, hydroxyurea, interferon and various
combinations. In all groups hypoplasia of a varying
degree was a frequent finding (6%) and often
accompanied by a patchy arrangement of hematopoiesis.
Most conspicuous was a gelatinous edema showing a
tendency to develop a discrete reticulin fibrosis
(scleredema). Aplasia developed in 7.7% of patients,
usually at terminal stages of the disease independently of
treatment. Minimal to moderate maturation defects of
hematopoiesis involved especially megakaryocytes and
erythroid precursors, but overt myelodysplastic features
were most prominent following hydroxyurea and busulfan therapy. Acceleration and blastic crisis were
characterized not only by increasing dysplastic changes,
but also by the appearence of blasts including CD34+
cells. Semiquantitative grading of the fiber content
revealed that 183 patients (54%) without or with
moderate fibrosis at the beginning showed a significant
progression and therefore contrasted with the 66 patients
with a stable state. Following this calculation no relevant
differences in the evolution of myelofibrosis were
evident in the various therapy groups especially not
following interferon treatment. In a few patients a
regression was found which was accompanied by a severe hypoplasia or aplasia compatible with a myeloablative
effect. In conclusion, peculiar BM changes, in
particular conspicuously expressed myelodysplastic
features are consistent with therapy-related lesions.
Development of myelofibrosis in IMF is obviously due
to disease progression unrelated to stage at diagnosis and
not significantly influenced by treatment modalities.
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