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
1. Adamkov M., Plank L., Szépe P.: Incidence of myelofibrosis among individual types of chronic myeloproliferative disorders. Brat. Lek. Listy, 99, 1998, s. 240-244.
2. Bauermeister D.E.: Quantitation of bone marrow reticulin – A Normal Range. Am. Jour. Clin. Pat., 56, 1971, s. 24-31.
3. Brunning R.D., McKenna R.W.: Chronic myeloproliferative diseases. In: Brunnig R.D., Mc Kenna R.W., ed. Atlas of Tumor Pathology – Atlas of the Bone Marrow, Washington: AFIP, 1994, s. 195-254.
4. Buesche G., Georgii A., Kreipe H.H.: Diagnosis and quantification of bone marrow fibrosis are significantly biased by the pre-staining processing of bone marrow biopsies. Histopathology, 48, 2006, s. 133-148.
5. Buhr T., Büsche G., Choritz H., Länger F., Kreipe H : Evolution of myelofibrosis in chronic idiopathic myelofibrosis as evidenced in sequential bone marrow biopsy specimens. Am. J. Clin. Pathol., 119, 2003, s. 152-158.
6. Buhr T., Georgii A., Choritz H.: Myelofibrosis in chronic myeloproliferative disorders. Incidence among subtypes according to the Hannover classification. Path. Res. Pract., 189, 1993, s.121-132.
7. Cervantes F., Alvarez-Larran A., Talarn C., Gomez M., Montserrat E.: Myelofibrosis with myeloid metaplasia following essential thrombocythaemia: actuarial probability, presenting characteristics and evolution in a series of 195 patients. Br. J. Haematol., 118, 2002, s. 786-790.
8. Duhamel G., Stachowiak J.: Bone marrow fibrosis in malignant hemopathies and cancers. Histological study of 2786 biopsies. Semm. Hop., 57, 1981, s. 111-116.
9. Frisch B., Bartl R.: Myelofibrosis and osteosclerosis and other fibroses in the bone marrow. In: Frisch B., Bartl R. ed. Biopsy Interpretation of Bone and Bone Marrow. Edward Arnold, 1999, s. 244-256.
10. Georgii A., Buhr T., Büsche G., Kreft A., Choritz H.: Classification and staging of Ph-negative myeloproliferative disorders by histopathology from bone marrow biopsies. Leukemia and Lymphoma, 22, 1996, s. 15-29.
11. Imbert M., Vardiman J.W., Pierre R., Brunning R.D., Thiele J., Flandrin G.: Essential thrombocytaemia. In: Jaffe E.S., Harris N.L., Stein H., Vardiman J.W., ed. World Health Organization Classification of Tumors, Pathology & Genetics, Tumours of Haemopoietic and Lymphoid Tissues, Lyon: IARC Press, 2001, s. 39-41.
12. Kreft A., Büsche G., Ghalibafian M., Buhr T., Fischer T., Kirkpatrick C.J : The incidence of myelofibrosis in Essential thrombocythemia, Polycythemia vera and Chronic idiopathic myelofibrosis. A retrospective evaluation of sequential bone marrow biopsies. Acta Haematol., 113, 2005, s. 137-143.
13. Lev P.R., Marta R.F., Vassallu P., Molinas F.C.: Variation of PDGF, TGFbeta, and bFGF levels in essential thrombocythemia patients treated with anagrelide. Am J Hematol., 70, 2002, s. 85-91.
14. Marcinek J., Plank L.: Chronická idiopatická myelofibróza: biologická charakteristika a diagnosticky a prognosticky relevantný histopatologický „grading“ fibrózy. Trans. a hemat. dnes, 12, 2006, s. 62-69.
15. Michiels J.J.: Diagnostic criteria of the myeloproliferative disorders (MPD): Essential thrombocythaemia, Polycythemia vera and Chronic megakaryocytic granulocytic metaplasia. Net. J. Med., 51, 1997, s. 57-64.
16. Michiels J.J., Juvonen E.: Proposal for revised diagnostic criteria of Essential thrombocythemia, and Polycythemia vera by the Thrombocythemia Vera Study Group. Sem. In Throm. and Hemost., 23, 1997, s. 339-346.
17. Michiels J.J., Kvasnicka H.M., Thiele J.: Chronic Ph1-negative myeloproliferative disorders (MPDs). In European MPD Workshop, Rotterdam, 2004, s. 2-40.
18. Van de Pette J.E., Prochazka A.V., Pearson T.C., ., ., .: Primary thrombocythemia treated with busulphan. Br. J. Haematol., 62, 1986, s. 229-237.
19. Thiele J., Hoeppner B., Zankovich R., Fischer R.: Histomorphometry of bone marrow biopsies in Primary osteomyelofibrosis/sclerosis (Agnogenic myeloid metaplasia) - correlations between clinical and morphological features. Wirchows Archiv A Pathol. Anat., 415, 1989, s. 191-202.
20. Thiele J., Kvasnicka H.M.: Chronic myeloproliferative disorders with thrombocythemia: a comparative study of two classification systems (PVSG, WHO) on 839 patients. Ann. Hematol., 82, 2003, s. 148-152.
21. Thiele J., Kvasnicka H.M., Facchetti F., Franco V., Van der Walt J., Orazi A.: European consensus on grading bone marrow fibrosis and assessment of cellularity. Haematologica, 90, 2005, s. 1128-1132.
22. Thiele J., Kvasnicka H.M., Fischer R.: Histochemistry and morphometry on bone marrow biopsies in Chronic myeloproliferative disorders - aids to diagnosis and classification. Ann. Hematol., 78, 1999, s. 495-506.
23. Thiele J., Kvasnicka H.M., Niederle N., et al.: The impact of Interferon versus Busulphan therapy on the reticulin stain-measured fibrosis in CML-a comparative morphometric study on sequential trephine biopsies. Ann. Hematol., 70, 1995, s. 121-128.
24. Thiele J., Kvasnicka H.M., Zankovich R., Diehl V.: Relevance of bone marrow features in the differential diagnosis between Essential thrombocythemia and early stage of Idiopathic myelofibrosis. Haematologica, 85, 2000, s. 1126-1134.
25. Vardiman J.W.: Chronic myelogenous leukemia and the Myeloproliferative disorders. In: Knowles D.M., ed. Neoplastic hematology, Baltimore: Williams Wilkins, 1992, s. 1405-1438.
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Czecho-Slovak Pathology
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