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Annals of Hematology

, Volume 93, Issue 1, pp 57–64 | Cite as

Prognostic impact of blast cell counts in dysplastic bone marrow disorders (MDS and CMML I) with concomitant fibrosis

  • Sigrid Machherndl-Spandl
  • W. Sega
  • H. Bösmüller
  • U. Germing
  • Ch. Gruber
  • K. Nachtkamp
  • P. Reinecke
  • W. R. Sperr
  • F. Wimazal
  • L. Müllauer
  • K. Sotlar
  • H. P. Horny
  • H. Tüchler
  • P. Valent
  • O. KriegerEmail author
Original Article

Abstract

In a retrospective study, 43 patients with dysplastic neoplasms of the bone marrow (myelodysplastic syndromes and myelodysplastic/myeloproliferative-overlap neoplasms) associated with marked (grades 2–3) fibrosis were examined. Histopathologic and morphologic findings as well as cytogenetic and molecular results were correlated with clinical endpoints. Multilineage dysplasia (34 of 43 patients, 79 %) and hypercellular bone marrow (64 %) were found in most patients. In ten of 35 patients, poor risk karyotypes according to the International Prognostic Scoring System (IPSS) were recorded. The JAK2 V617F mutation was detected in four of 30 patients (13 %), and the KIT D816V mutation was found in two of 30 patients (6 %). Patients were mainly treated with palliative drugs and best supportive care. After an observation time of 1–41 (median 21) months, ten of 43 patients (23 %) had developed a secondary acute leukemia. The median survival of all 43 patients was 21.4 months (range 1.8–88.2 months). Of all prognostic parameters examined, the blast cell count at diagnosis was found to be a most reliable and most predictive marker concerning survival and leukemia progression. This confirms previous studies in dysplastic bone marrow neoplasms without fibrosis.

Keywords

Myelodysplastic syndromes Chronic myelomonocytic leukemia Bone marrow fibrosis 

Notes

Conflict of interest

The authors declare that they have no conflict of interest.

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Copyright information

© Springer-Verlag Berlin Heidelberg 2013

Authors and Affiliations

  • Sigrid Machherndl-Spandl
    • 1
  • W. Sega
    • 2
  • H. Bösmüller
    • 2
  • U. Germing
    • 3
  • Ch. Gruber
    • 2
  • K. Nachtkamp
    • 3
  • P. Reinecke
    • 4
  • W. R. Sperr
    • 5
  • F. Wimazal
    • 5
  • L. Müllauer
    • 6
  • K. Sotlar
    • 7
  • H. P. Horny
    • 7
  • H. Tüchler
    • 8
  • P. Valent
    • 5
    • 9
  • O. Krieger
    • 1
    • 10
    Email author
  1. 1.Department of Hematology, Stem Cell Transplantation, Haemostaseology and Internal OncologyKH ElisabethinenLinzAustria
  2. 2.Pathologic DepartmentKH der Barmherzigen SchwesternLinzAustria
  3. 3.Hematologic DepartmentUniversity of DüsseldorfDüsseldorfGermany
  4. 4.Institute for PathologyUniversity of DüsseldorfDüsseldorfGermany
  5. 5.Division of Hematology and Hemostaseology, Department of Internal Medicine 1Medical University of ViennaViennaAustria
  6. 6.Institute of PathologyMedical University of ViennaViennaAustria
  7. 7.Institute of PathologyUniversity of MunichMunichGermany
  8. 8.Ludwig Boltzmann Institute for Leukemia Research and HaematologyHanusch HospitalViennaAustria
  9. 9.Ludwig Boltzmann Cluster OncologyMedical University of ViennaViennaAustria
  10. 10.Department of Internal Medicine ILinzAustria

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