Bone marrow biopsy in ET, PV and PDGM Dr. Michiels (tomorrow at 12:00 PM)

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Apr 1, 2021, 12:00:21 PM4/1/21
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http://www.mpdsupport.org From: "Dr. J. J. Michiels Goodheart Institute, Rotterdam"
Sent: Monday, November 05, 2007 1:55 AM Subject: BMB

Bone marrow biopsy in ET, PV and PDGM

Information for clinicians, hematologists

Myelofibrosis is not specific for a disease and seen in patient with hairy cell leukemia, Ph-positive chronic myeloid leukemia, myeloid metaplasia (MM), chronic (dys)megakaryocytic granulocytic myeloproliferation, polycythemia vera and many other conditions. The term CIMF is derived from reticulin staining of bone marrow biopsies which only shows you black fine or coarse fibers and is absolutely no prognostic factors significance. In 1990 Georgii concluded that CIMF is secondary and in fact is based on Chronic Imagination in the Mind of Famous Pathologist. The use of CIMF in textbooks and so many articles by leading MPD experts is misleading and scientifically not sound. We have now eliminated this term in our 2008 WHO-ECMP criteria for the MPDs of JAK2V617F positive essential thrombocythemia ET and polycythemia vera PV, JAK2-negative primary thrombocythemia (PT) and primary dysmagakaryocytic granulocytic myeloproliferation (PDGM). PDGM (Poster MPD Conference New York November
8-10 2007). PV= as a trlinear MPD of thrombocythemia leukocythemia and polycythemia vera followed by myeloid metaplasia with no or different grades of reticulin fibrosis.

Myelofibrosis (MF) itself is not a disease because reticulin and collagen fibrosis are produced by polyclonal fibroblasts in response to cytokines released from the clonal granulocytic and megakaryocytic proliferative cells in both PV and CMF (figure 5). The presence of reticuline finbrosis is well documented in ET, PV, PT, PDGM, CML and in many other conditions. Various degrees of reticulin fibrosis rather rare in true ET and does occur in about one third of PV and in the majority of patients with PDGM during long-term follow-up.

Information for pathologists

The grading of the Baumeister scoring system of RF was developed on aspirated bone marrow samples, is poorly defined and not reproducible for the proper grading of myelofibrosis in bone marrow biopsies by pathologist. The Manoharan system used silver stain according to Gordon and Sweet scored the degree of reticulin in bone marrow biopsy in a completely different way. A scoring system based on morphometric analysis (point intersection with an ocular grid) and quality of fibers (reticulin and collagen fibers) and the bone marrow fiber density (fine or course reticulin and some or course bundles of collagen) has been proposed by German institutes of pathology. All these different scoring systems for MF use different criteria for grading of reticulin and collagen, are subjective and not comparable by lack of strict criteria. The easiest way in grading of reticulin fibrosis using the reticulin siver stain as has been done by the polycythemia vera study group (PVSG) and in the recent study of Wilkins et al published in Blood on PubMed showing that RF itself has no prognotic values.

A panel

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