User menu

Accès à distance ? S'identifier sur le proxy UCLouvain

Myelodysplastic Syndromes With Bone-marrow Fibrosis - a Myelodysplastic Disorder With Proliferative Features

Primary tabs

Verhoef, GEG. Ferrant, Augustin [UCL] Dewolfpeeters, C. Deprez, S. Meeus, Peter [UCL] Boogaerts, MA.

We report on 22 patients with myelodysplastic syndrome (MDS), all of whom showed striking marrow fibrosis. Variable blood counts, often with teardrop poikilocytosis and a leukoerythroblastic picture, were present at diagnosis. Visceral enlargement was detected in 17 patients with a distinct splenomegaly in seven cases. All cases demonstrated dysplasia in at least two cell lineages. No specific cytogenetic abnormality seems to characterize this group of patients. Southern blot analysis showed no breakpoint cluster region rearrangement as observed in classical chronic myeloid leukemia. Ferrokinetic studies revealed quantitatively deficient erythropoiesis in all except two cases and an abnormally high fraction of ineffective erythropoiesis in all. Splenic erythropoiesis was present in eight patients. The median survival was 18 months. At the time of this report, 12 patients had died. The causes of death were disease progression (7 patients) and infection (5 patients). One might speculate that the present series of cases represents a transition between MDS and myeloproliferative disease, thereby displaying characteristics of both groups of diseases.

Document type Article de périodique (Journal article) – Article de recherche
Access type Accès restreint
Publication date 1991
Language Anglais
Journal information "Annals of Hematology" - Vol. 63, no. 5, p. 235-241 (1991)
Peer reviewed yes
Publisher Springer Verlag (New York)
issn 0939-5555
e-issn 1432-0584
Publication status Publié
Affiliations UCL - Cliniques universitaires Saint-Luc
UCL - MD/MINT - Département de médecine interne
Keywords MYELODYSPLASTIC SYNDROME ; MYELOFIBROSIS ; MYELOPROLIFERATIVE DISORDER
Links
  1. Assoian RK, Grotendorst GR, Miller DM, Sporn MB (1984) Cellular transformation by coordination of three peptide growth factors from human platelets. Nature 309: 806
  2. Bain BJ, Catovsky D, O'Brien M, Prentice HG, Lawlor E, Kumaran TO, McCann SR, Matutes E, Galton DAG (1981) Megakaryoblastic leukemia presenting as acute myelofibrosis. A study of four cases with the platelet-peroxidase reaction. Blood 58: 206–213
  3. Barosi G., Cazzola M., Frassoni F., Orlandi E., Stefanelli M., Erythropoiesis in Myelofibrosis with Myeloid Metaplasia: Recognition of Different Classes of Patients by Erythrokinetics, 10.1111/j.1365-2141.1981.tb08459.x
  4. Bearman RM, Pangalis G, Rappaport H (1979) Acute (“malignant”) myelosclerosis. Cancer 43: 279–293
  5. Bennett J. M., Catovsky D., Daniel M. T., Flandrin G., Galton D. A. G., Gralnick H. R., Sultan C., Proposals for the classification of the myelodysplastic syndromes, 10.1111/j.1365-2141.1982.tb08475.x
  6. Bentley SA, Murray KH, Lewis SM, Roberts PD (1977) Erythroid hypoplasia in myelofibrosis: a feature associated with blastic transformation. Br J Hematol 36: 41–47
  7. Castoldi G, Cuneo A, Tomasi P, Ferrari L (1987) Chromosome abnormalities in myelofibrosis. Acta Haematol 78: 104–108
  8. 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, New York pp 427–454
  9. Castro-Malaspina H, Rabellio EM, Yen A, Nachman RL, Moore MAS (1981) Human megakaryocyte stimulation of proliferation of bone marrow fibroblasts. Blood 57: 781–787
  10. Davis MP, Dewald GW, Pierre RV, Hoagland HC (1984) Hematological manifestations associated with deletions of the long arm of chromosome 20. Cancer Genet Cytogenet 12: 63–71
  11. Elias L, Van Epps DE, Smith KJ, Savage B (1987) A trial of recombinantα 2 interferon in the myelodysplastic syndrome. II. Characterization and response of granulocyte and platelet dysfunction. Leukemia 2: 111–115
  12. Feinberg A, Vogelstein B (1984) A technique for radiolabeling DNA restriction endonuclease fragments to high activity. Ann Biochem 137: 266–267
  13. Felman P, Bryon P, Gentilomme O, Ferench M, Charrin C, Espinouse D, Viala JJ (1988) The syndrome of abnormal chromatin clumping in leukocytes: a myelodysplastic disorder with proliferative features. Br J Haematol 70: 49–54
  14. Fenaux P, Jouet JP, Zandecki M, Lai JL, Simon M, Pollet JP, Bauters F (1987) Chronic and subacute myelomonocytic leukaemia in the adult: a report of 60 cases with special reference to prognostic factors. Br J Haematol 65: 101–106
  15. Ferrant A, Cauwe F, Cogneau M, Beckers C, Verwilghen RL, Sokal G (1980) Measurement of erythropoiesis using52Fe. Br J Haematol 45: 447–457
  16. Ferrant A, Rodhain J, Cauwe F, Cogneau M, Beckers C, Michaux JL, Verwilghen RL, Sokal G (1982) Assessment of bone marrow and splenic erythropoiesis in myelofibrosis. Scand J Haematol 29: 373–380
  17. Galton DAG (1986) The myelodysplastic syndromes. Scand J Haematol 36 [Suppl 45]: 11–20
  18. Ganser A, Carlo-Stella C, Greher J, Volkers B, Hoelzer D (1987) Effect of recombinant interferonsα and γ on human bone marrow-derived megakaryocytic progenitor cells. Blood 70: 1173–1179
  19. Geddes AD, Bowen DT, Jacobs A (1990) Clonal karyotype abnormalities and clinical progress in the myelodysplastic syndrome. Br J Haematol 76: 194–202
  20. GROOPMAN JEROME E., The Pathogenesis of Myelofibrosis in Myeloproliferative Disorders, 10.7326/0003-4819-92-6-857
  21. Gyger M, Infante-Rivard C, D'Angelo G, Forest L, Lussier P (1988) Prognostic value of clonal chromosomal abnormalities in patients with primary myelodysplastic syndromes. Am J Hematol 28: 13–20
  22. Heim S, Mitelman F (1987) Cancer cytogenesis. Alan R Liss, New York, pp 129–140
  23. International Committee for Standardization in Haematology (1971) Recommended methods for radioisotope red cell survival. Blood 38: 378–386
  24. Jacobs A (1985) Myelodysplastic syndromes: pathogenesis, functional abnormalities and clinical implications. J Clin Pathol 38: 1201–1217
  25. Jaén A, Irriguible D, Milla F, Vallespi T, Torrabadella M, Abella E, Lafuente R, Woessner S (1990) Abnormal chromatin clumping in leukocytes: a clue to a new subtype of myelodysplastic syndrome. Eur J Haematol 45: 209–214
  26. Kerkhofs H, Hermans I, Haak HL, Leeksma CHW (1987) Utility of the FAB classification for myelodysplastic syndromes: investigation of prognostic factors in 237 cases. Br J Haematol 61: 73–81
  27. Kimura A, Katoh O, Kuramoto A (1988) Effects of plateletderived growth factor, epidermal growth factor, and transforming growth factor-β on the growth of human marrow fibroblast. Br J Haematol 69: 9–12
  28. Kimura A, Katoh O, Kuramoto A (1988) Marrow fibroblasts from patients with myeloproliferative disorders show increased sensitivity to human serum mitogens. Br J Haematol 69: 153–156
  29. Lambertenghi-Deliliers G, Orazi A, Luksch R, Annaloro C, Soligo D (1991) Myelodysplastic syndrome with increased marrow fibrosis: a distinct clinico-pathological entity. Br J Haematol 78: 161–166
  30. McCarthy DM (1985) Fibrosis of the bone marrow: content and causes. Br J Haematol 59: 1–7
  31. Manoharan A, Horsley R, Pitney WR (1979) The reticulin content of bone marrow in acute leukaemia in adults. Br J Haematol 43: 185–190
  32. May SJ, Smith SA, Jacobs A, Williams A, Bailey-Wood R (1985) The myelodysplastic syndrome: analysis of laboratory characteristics in relation to the FAB classification. Br J Haematol 59: 311–319
  33. Miyazono K, Okabe T, Urabe A, Takaku F, Heldin CH (1987) Purification and properties of an endothelial cell growth factor from human platelets. J Biol Chem 262: 4098–5003
  34. Najean Y, Cacchione R, Castro-Malaspina H, Dresch C (1978) Erythrokinetic studies in myelofibrosis: their significance for prognosis. Br J Haematol 40: 205–217
  35. Ottolander GJ den, te Velde J, Brederoo P, Geraedts JPM, Slee PHT, Willemze R, Zwaan FE, Haak HL, Muller HP, Bieger R (1979) Megakaryoblastic leukaemia (acute myelofibrosis): a report of three cases. Br J Haematol 42: 9–20
  36. Pagliuca A, Layton DM, Manoharan A, Gordon S, Green PJ, Mufti GJ (1989) Myelofibrosis in primary myelodysplastic syndromes: a clinico-morphological study of 10 cases. Br J Haematol 71: 499–504
  37. Rege-Cambrin G, Mecucci C, Tricot G, Michaux JI, Louwagie A, van Hove W, Francart H, Van Den Berghe H (1987) A chromosomal profile of polycythemia vera. Cancer Genet Cytogenet 25: 233–245
  38. Ricketts C, Jacobs A, Cavill I (1975) Ferrokinetics and erythropoiesis in man: the measurement of effective erythropoiesis, ineffective erythropoiesis and red cell life span using59Fe. Br J Haematol 31: 65–75
  39. Sanz GF, Sanz MA, Vallespi T, Cañizo MC, Torrabadella M, Garcia S, Irriguible D, San Miguel JF (1989) Two progression models and a scoring system for predicting survival and planning treatment in myelodysplastic syndromes: a multivariate analysis of prognostic factors in 370 patients. Blood 74: 395–408
  40. Solal-Celigny P, Desaint B, Herrera A, Chastang G, Amar M, Vroclans M, Broussa N, Mancilla F, Renoux M, Bernard JF, Boivin P (1984) Chronic myelomonocytic leukaemia according to FAB classification. Analysis of 35 cases. Blood 63: 634–638
  41. Swolin B, Weinfeld A, Westin J (1988) A prospective long-term cytogenetic study in polycythemia vera in relation to treatment and clinical course. Blood 72: 386–395
  42. Tricot G, De Wolf-Peeters C, Hendrickx B, Verwilghen RL (1984) Bone marrow histology in myelodysplastic syndromes. I. Histological findings in myelodysplastic syndromes and comparison with bone marrow smears. Br J Haematol 57: 423–430
  43. Tricot G, Vlietinck R, Boogaerts MA, Hendrickx B, De WolfPeeters C, Van Den Berghe H, Verwilghen RL (1985) Prognostic factors in the myelodysplastic syndromes: importance of initial data on peripheral blood counts, bone marrow cytology, trephine biopsy and chromosomal analysis. Br J Haematol 60: 19–32
  44. Verhoef GEG, De Wolf-Peeters C, Ferrant A, Boogaerts MA (1990) A case of myelodysplastic syndrome and myelofibrosis. Br J Haematol 45: 373–375
  45. Verhoef G, De Wolf-Peeters C, Kerim S, Van De Broeck J, Mecucci C, Van Den Berghe H, Boogaerts M (1991) Update on the prognostic implication of morphology, histology and karyotype in primary myelodysplastic syndromes. Hematol Pathol 5 (4): 163–175
  46. Verhoef G, Meeus P, Stul M, Mecucci C, Cassiman JJ, Van Den Berghe H, Boogaerts M (1991) Cytogenetic and molecular studies of the Philadelphia translocation in the myelodysplastic syndromes: report of 2 cases and review of the literature. Cancer Genet Cytogenet (in press)
  47. Wang JC, Lang HD (1987) Recombinantα interferon inhibits the proliferation of bone marrow fibroblasts cultured from patients with myelofibrosis and myeloid metaplasia (MMM). Blood 70: 188 a
  48. 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
  49. White CW, Sondheimer HM, Crouch EC, Wilson H, Fan LL (1989) Treatment of pulmonary hemangiomatosis with recombinant interferon α-2a. N Engl J Med 320: 1197–1200
  50. Wolf BC, Neiman RS (1985) Myelofibrosis with myeloid metaplasia: Pathophysiologic implications of the correlation between bone marrow changes and progression of splenomegaly. Blood 65: 803–809
Bibliographic reference Verhoef, GEG. ; Ferrant, Augustin ; Dewolfpeeters, C. ; Deprez, S. ; Meeus, Peter ; et. al. Myelodysplastic Syndromes With Bone-marrow Fibrosis - a Myelodysplastic Disorder With Proliferative Features. In: Annals of Hematology, Vol. 63, no. 5, p. 235-241 (1991)
Permanent URL http://hdl.handle.net/2078.1/50642