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Evolution of fibro-osteosclerotic bone marrow lesions in primary (idiopathic) osteomyelofibrosis--a histomorphometric
J Thiele1, Y S Chen, H M Kvasnicka
1Institute of Pathology, University of Cologne, Germany.
Abstract:
Evolution of fibro-osteosclerotic bone marrow lesions in the course of primary (idiopathic) osteomyelofibrosis (OMF) was studied in 36 patients (17 males, 19 females; median age 57 years) by morphometric evaluation of sequential trephine biopsies. The mean interval between first and terminal examination was 33 months (range 6 to 121 months). Two biopsies were performed in 31 and three and more in five patients. Morphometry consisted of a determination of argyrophilic (reticulin and collagen) fiber density, measured per area of hematopoiesis or marrow cellularity, and the calculation of the extent of trabecular bone tissue. In 13 of our 36 patients increase in reticulin and collagen deposits was only borderline to minimal during the observation period. On the other hand, in 23 of the 36 patients a slight to gross accumulation of reticulin and collagen fibers, partially associated with osteosclerotic changes was recognizable. No regression of fibrosis was encountered in our cohort of patients which included three cases with preceding low-dose busulfan therapy. Analysis of the different lengths of intervals between the first and the last biopsy and degree of fibrosis as well as osteosclerosis, suggested that alterations developed progressively, however, at an unpredictable and considerably varying rate. Thus our findings were not in keeping with several studies on smaller series of patients, which generally contested a progression of fibro-osteosclerotic lesions in OMF and additionally reported reversal of the pathology following chemotherapy.(ABSTRACT TRUNCATED AT 250 WORDS)
Insights
Fibro-osteosclerotic bone lesions in primary osteomyelofibrosis (OMF) generally progress over time, showing increasing fibrosis and osteosclerosis. No regression of these lesions was observed in this study.
Area of Science:
- Hematology
- Oncology
- Pathology
Background:
- Primary osteomyelofibrosis (OMF) is characterized by bone marrow fibrosis and osteosclerosis.
- The natural progression of fibro-osteosclerotic lesions in OMF requires further elucidation.
Purpose of the Study:
- To investigate the longitudinal evolution of fibro-osteosclerotic bone marrow lesions in patients with primary osteomyelofibrosis (OMF).
Main Methods:
- Morphometric evaluation of sequential trephine biopsies from 36 OMF patients over a median of 33 months.
- Quantification of argyrophilic fiber density and trabecular bone extent.
Main Results:
- 23 out of 36 patients showed progressive accumulation of reticulin and collagen fibers, often with osteosclerosis.
- No regression of fibrosis was observed; lesions developed progressively at a variable rate.
- Findings contrast with studies reporting lesion reversal after chemotherapy.
Conclusions:
- Fibro-osteosclerotic lesions in OMF typically demonstrate progressive changes.
- The rate of progression is variable and unpredictable.
- Observed progression contradicts some previous reports suggesting lesion reversal.