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Primary myelofibrosis: A detailed statistical analysis of the clinicopathological variables influencing survival

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Summary

In the present study we analyzed the prognostic significance of several clinical, hematological, and histological parameters recorded at diagnosis in a consecutive series of 72 patients with primary myelofibrosis (PMF). Univariate analysis showed that the most significant indicators of poor survival were the following: age greater than 60, splenomegaly, anemia (hemoglobin > 10 g/dl), leukopenia (WBC<4×109/l or leukocytosis >14x109/l), and any of these histological features: adipose tissue and megakaryocyte reduction, prominent osteoblastic rims along the trabecular bone, presence of peritrabecular megakaryocytes (Mk), absence of normal or giant Mk. The multivariate analysis showed that only the level of hemoglobin and the presence of both normal Mk and fever independently influenced the prognosis. These parameters were used to set up a prognostic scoring system, allowing a feasible prognosis to be made for each patient at the time of diagnosis and identifying those patients in urgent need of new therapeutic approaches.

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References

  1. Anger B, Seidler R, Haug U, Popp C, Heimpel H(1990) Idiopathic myelofibrosis: a retrospective study of 103 patients. Haematologica (Pavia) 74:228–234

    Google Scholar 

  2. Barosi G, Cazzola M, Frassoni F, Orlandi E, Stefanelli M (1981) Erythropoiesis in myelofibrosis with myeloid metaplasia: recognition of different classes of patients by erythrokinetics. Br J Haematol 48:263–272

    PubMed  Google Scholar 

  3. Barosi G, Berzuini C, Liberato LN, Costa A, Polino G, Ascari E (1988) A prognostic classification of myelofibrosis with myeloid metaplasia. Br J Haematol 70:397–401

    PubMed  Google Scholar 

  4. Barthl R, Frisch B, Burkhardt R (1985) Myeloproliferative disorders. In: Bone marrow biopsies revisited. A new dimension for haematologic malignancies, 2nd edn. Karger, Basel

    Google Scholar 

  5. Bouroncle BA, Doan CA (1962) Myelofibrosis — clinical, haematologic and pathologic study of 110 patients. Am J Med Sci 243:697–715

    PubMed  Google Scholar 

  6. Breton-Gorius J, Biet M, Reyes F, Dupuy E, Mear C, Vannier JP, Trons P (1982) Myelofibrosis and acute megakaryoblastic leukemia in a child: topographic relationship between fibroblasts and megakaryocytes with anα-granule defect. Leuk Res 6:97–110

    PubMed  Google Scholar 

  7. Buschle M, Janssen JWG, Drexler H, Lyons J, Anger B, Bartram CR (1988) Evidence for pluripotent stem cell origin of idiopathic myelofibrosis: clonal analysis of a case characterised by a N-ras gene mutation. Leukemia 2:658–660

    PubMed  Google Scholar 

  8. Burkhardt R, Bartl R, Jaegger K, Frisch B, Kettner G, Mahl G, Sund M(1984) Chronic myeloproliferative disorders (CMPD). Pathol Res Pract 179:131–186

    PubMed  Google Scholar 

  9. Burkhardt R, Barth R, Jager K et al. (1986) Working classification of chronic myeloproliferative disorders based on histological, haematological and clinical findings. J Clin Pathol 39:237–252

    PubMed  Google Scholar 

  10. Castro-Malaspina H, Rabellino EM, Yen A, Nachman RL, Moore MAS (1981) Human megakaryocyte stimulation of proliferation of bone marrow fibroblasts. Blood 57:781

    PubMed  Google Scholar 

  11. Castro-Malaspina H (1984) Pathogenesis of myelofibrosis: role of ineffective megakaryopoiesis and megakaryocyte components. In: Berk PD, Castro-Malaspina H, Wasserman LR (eds) Myelofibrosis and the biology of connective tissue. Alan R. Liss, INC, New York, pp 427–454

    Google Scholar 

  12. Chelloul N, Jacquillat C, Brière J, Laval-Jeantet M, Vorhauer-Atlan W (1975) Valeur pronostique des lesions osteomedullaires de la splenomegalie myeloide primitive. Nouv Presse Med 4:3060–3062

    PubMed  Google Scholar 

  13. Demory JL, Dupriez B, Fenaux P, Lai JI, Benscart R, Jouet JP, Deminatti M, Bautes F (1988) Cytogenetic studies and their prognostic significance in agnogenic myeloid metaplasia. Blood 72:855–859

    PubMed  Google Scholar 

  14. Gardner MJ, Altmann DG (1990) Gli intervalli di confidenza. Oltre la significatività statistica. Il Pensiero Scientifico Editore, Roma

    Google Scholar 

  15. Georgii A, Thiele J, Vykoupil KF (1980) Osteomyelofibrosis: a histological and cytogenetic study on core biopsies of the bone marrow. Virchows Arch [A] 389:269–286

    Google Scholar 

  16. Gustavsson P, Holm J, Wahlin A, Norberg B (1985) Staging of idiopathic myelofibrosis. Acta Med Scand 218:487–491

    PubMed  Google Scholar 

  17. Hasselbalch H, Jensen BA (1990) Prognostic factors in idiopathic myelofibrosis. A simple clinical scoring system with prognostic significance. Eur J Haematol 44:172–178

    PubMed  Google Scholar 

  18. Hintze JL (1990) Number cruncher statistical system (NCSS), version 5.5. J. L. Hintze, Kaysville, Utah USA

    Google Scholar 

  19. Jacobson RJ, Salo A, Fialkow PJ (1978) Agnogenic myeloid metaplasia: a clonal proliferation of haematopoietic stem cells with secondary myelofibrosis. Blood 51:189–194

    PubMed  Google Scholar 

  20. Kaplan EL, Meier P (1958) Nonparametric estimation from incomplete observations. J Am Stat Assoc 53:457

    Google Scholar 

  21. Kreipe H, Jaquet K, Feigner J, Radzun HJ, Parwaresch MR (1991) Clonal granulocytes and bone marrow cells in the cellular phase of agnogenic myeloid metaplasia. Blood 78:1814–1817

    PubMed  Google Scholar 

  22. Lawler SD, Swansbury GJ (1985) Cytogenetic studies in myelofibrosis and related conditions. In: Lewis SM (ed) Myelofibrosis: pathophysiologic and clinical management. Marcel-Dekker, New York, pp 167–194

    Google Scholar 

  23. Lewis SM(1985) Myelofibrosis: historical perspective. In: Lewis SM (ed) Myelofibrosis: pathophysiologic and clinical management. Marcel-Dekker, New York, pp 1–13

    Google Scholar 

  24. Manoharan A, Smart RC, Pitney WR (1982) Prognostic factors in myelofibrosis. Pathology 14:455–461

    PubMed  Google Scholar 

  25. Manoharan A(1988) Annotation. Myelofibrosis — prognostic factors and treatment. Br J Haematol 69:295–298

    PubMed  Google Scholar 

  26. Najean YU, Cacchione R, Castro-Malaspina H, Dresh C (1978) Erythrokinetic studies in myelofibrosis: their significance for prognosis. Br J Haematol 40:205–217

    PubMed  Google Scholar 

  27. Nakai GS, Craddock CG, Figueroa WG (1962) Agnogenic myeloid metaplasia: a survey of twenty-nine cases and a review of the literature. Ann Intern Med 57:419–440

    PubMed  Google Scholar 

  28. Njoku OS, Lewis SM, Catovsky D, Gordon-Smith EC (1983) Anaemia in myelofibrosis: its value in prognosis. Br J Haematol 54:79–89

    PubMed  Google Scholar 

  29. Pereira A, Cervantes F, Brugues R, Rozman C (1990) Bone marrow histopathology in primary myelofibrosis: clinical and haematologic correlations and prognostic evaluation. Eur J Haematol 44:94–98

    Google Scholar 

  30. Rosenthal DS, Moloney WC (1969) Myeloid metaplasia: a study of 98 cases. Postgrad Med 45:136–142

    Google Scholar 

  31. Schultz A, Knor H (1984) Comparative histological and clinical study on the staging of polycythemia vera and osteosclerosis. In: Lennert K, Hubner K(eds) Pathology of the bone marrow. Gustav Fisher, Stuttgart, pp 177–181

    Google Scholar 

  32. Silverstein MN (1975) Agnogenic myeloid metaplasia. Publishing Sciences Group, Boston

    Google Scholar 

  33. Silverstein MN (1983) Agnogenic myeloid metaplasia. In: Williams WJ, Bentler E, Erslev AJ, Lichtman MA (eds) Hematology. Mc Graw-Hill, New York, pp 214–223

    Google Scholar 

  34. Van Slyck EJ, Weiss L, Dully M (1970) Chromosomal evidence for the secondary role of bone marrow fibroblast proliferation in acute myelofibrosis. Blood 36:729–735

    PubMed  Google Scholar 

  35. Varki A, Lottenberg R, Griffith R, Reinhard E (1983) The syndrome of idiopathic myelofibrosis: a clinicopathologic review with emphasis on the prognostic variable predicting survival. Medicine 62:353–371

    PubMed  Google Scholar 

  36. Visani G, Finelli C, Castelli U, Petti C, Ricci P, Vianelli N, Gianni L, Zuffa E, Aloe Spiriti MA, Latagliata R, Pileri S, Magrini U, Gugliotta L, Morra E, Bernasconi C, Mandelli F, Tura S (1990) Myelofibrosis with myeloid metaplasia: clinical and haematological parameters predicting survival in a series of 133 patients. Br J Haematol 75:4–9

    PubMed  Google Scholar 

  37. Ward HP, Block MH (1971) The natural history of agnogenic myeloid metaplasia (AMM) and a critical evaluation of its relationship with the myeloproliferative syndrome. Medicine 50:357–420

    PubMed  Google Scholar 

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Rupoli, S., Da Lio, L., Sisti, S. et al. Primary myelofibrosis: A detailed statistical analysis of the clinicopathological variables influencing survival. Ann Hematol 68, 205–212 (1994). https://doi.org/10.1007/BF01834368

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  • DOI: https://doi.org/10.1007/BF01834368

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