Fibrosis Identified in the Bone Marrow Biopsies of Patients with Essential Thrombocythemia: its Incidence and Significance for the Differential Diagnostic Considerations
Authors:
J. Marcinek; L. Plank; P. Szépe; T. Balhárek
Authors‘ workplace:
Department of Pathology, Comenius University, Jessenius Medical Faculty and Martin’s Faculty Hospital, Martin, Slovakia
Published in:
Čes.-slov. Patol., 44, 2008, No. 3, p. 62-66
Category:
Original Article
Overview
Myelofibrosis (MF) may develop in all types of myeloproliferative disorders and its identification is of clinical relevance. Typical bone marrow (BM) morphology of patients with essential thrombocythemia (ET) shows either “normal“ amount or “a slight increase“ of reticulin fibers, but the published data differ in relation to the applied MF definition and ET diagnostic criterias. The aim of this study was to evaluate retrospectivelly MF in BM biopsies of 30 cases in which the diagnosis of ET was confirmed also clinically by local hematologists. In 7 of the patients not only primary but also sequential biopsy was available. The MF grade and extent were evaluated semiquantitativelly in archival slides stained by Gömöri silver impregnation. The analysis was based on the European clinico-pathological criteria 2004 (ECP) defining a) normal bone marrow fibrosis (MF0), b) slight reticulin fibrosis (MF1), c) advanced reticulin and initial collagen fibrosis (MF2) and d) advanced collagen fibrosis (MF3). Generally, in majority of the biopsies MF0 (n = 6) or MF1 (n = 25, 18× focal and 7× diffuse) was found. More advanced MF2 was much less common as it was present in 6 biopsies (5× focal and 1× diffuse). In relation to the actual time of BM biopsy during course of the disease, the introductory biopsies done at the time of diagnosis (n=18) showed 3× MF0, 14× MF1 and 1× MF2. The biopsies performed after a long time of patients observations (n = 12) showed 3× MF0, 7× MF1 and 2× MF2. In 5 of 7 sequential biopsies the progress of MF was evident, but 4 of these patients were treated by cytoreductive therapy. We conclude that the BM of patients with ET in initial phase shows either MF0 or focal slight increase of reticulin fibers (MF1). In addition, the long course of the disease and/or applied therapy may lead to more developed MF and more advanced MF stages (diffuse MF1 or MF2). Therefore their finding in the BM biopsies examined in the later phases of the disease should not exclude the diagnosis of ET.
Key words:
myelofibrosis - essential thrombocythemia - bone marrow - reticulin and collagen fibers
Sources
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Anatomical pathology Forensic medical examiner ToxicologyArticle was published in
Czecho-Slovak Pathology
2008 Issue 3
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