Skip to main content
Log in

Usefulness of spleen volume measured by computed tomography for predicting clinical outcome in primary myelofibrosis

  • Original Article
  • Published:
International Journal of Hematology Aims and scope Submit manuscript

Abstract

Although splenomegaly is major characteristic of primary myelofibrosis (PMF), splenomegaly has been devalued due to a less reliable method based on physical examination (PEx). We evaluated whether spleen volume (SV) on CT would accurately predict clinical outcomes in PMF. A total of 188 patients were enrolled. SV was quantitated by the automatic volume software. In ROC curve, the SV predicted prognosis more accurately than spleen length by PEx (p < 0.001). The ideal cut-off value was 378.1 cm3 for SV, which was divided into high- and low-volume status. Patients with low SV status had superior leukemia-free survival and overall survival compared to high SV status (p < 0.001, p < 0.001) In the Cox analysis, old age ≥65 years (p = 0.004, p = 0.001), low Hemoglobin <10.0 g/dL (p = 0.023, p = 0.021), high WBC counts ≥25 × 109/L (p = 0.003, p = 0.006), peripheral blasts ≥1 % (p = 0.029, p = 0.020), unfavorable cytogenetic abnormalities (p = 0.025, p = 0.028), and high SV status (p = 0.004, p = 0.003) were independently associated with survivals. SV measured by CT was important for predicting survival in patients with PMF.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2
Fig. 3

Similar content being viewed by others

References

  1. Vardiman JW, Thiele J, Arber DA, Brunning RD, Borowitz MJ, Porwit A, et al. The 2008 revision of the World Health Organization (WHO) classification of myeloid neoplasms and acute leukemia: rationale and important changes. Blood. 2009;114:937–51.

    Article  CAS  PubMed  Google Scholar 

  2. Cervantes F, Barosi G, Demory JL, Reilly J, Guarnone R, Dupriez B, Pereira A, et al. Myelofibrosis with myeloid metaplasia in young individuals: disease characteristics, prognostic factors and identification of risk groups. Br J Haematol. 1998;102:684–90.

    Article  CAS  PubMed  Google Scholar 

  3. Cervantes F, Pereira A, Esteve J, Rafel M, Cobo F, Rozman C, et al. Identification of ‘short-lived’ and ‘long-lived’ patients at presentation of idiopathic myelofibrosis. Br J Haematol. 1997;97:635–40.

    Article  CAS  PubMed  Google Scholar 

  4. Reilly JT, Snowden JA, Spearing RL, Fitzgerald PM, Jones N, Watmore A, et al. Cytogenetic abnormalities and their prognostic significance in idiopathic myelofibrosis: a study of 106 cases. Br J Haematol. 1997;98:96–102.

    Article  CAS  PubMed  Google Scholar 

  5. Kvasnicka HM, Thiele J, Werden C, Zankovich R, Diehl V, Fischer R. Prognostic factors in idiopathic (primary) osteomyelofibrosis. Cancer. 1997;80:708–19.

    Article  CAS  PubMed  Google Scholar 

  6. Dupriez B, Morel P, Demory JL, Lai JL, Simon M, Plantier I, et al. Prognostic factors in agnogenic myeloid metaplasia: a report on 195 cases with a new scoring system. Blood. 1996;88:1013–8.

    CAS  PubMed  Google Scholar 

  7. Cervantes F, Dupriez B, Pereira A, Passamonti F, Reilly JT, Morra E, et al. New prognostic scoring system for primary myelofibrosis based on a study of the international working group for myelofibrosis research and treatment. Blood. 2009;113:2895–901.

    Article  CAS  PubMed  Google Scholar 

  8. Passamonti F, Cervantes F, Vannucchi AM, Morra E, Rumi E, Pereira A, et al. A dynamic prognostic model to predict survival in primary myelofibrosis: a study by the IWG-MRT (international working group for myeloproliferative neoplasms research and treatment). Blood. 2010;115:1703–8.

    Article  CAS  PubMed  Google Scholar 

  9. Gangat N, Caramazza D, Vaidya R, George G, Begna K, Schwager S, et al. DIPSS plus: a refined dynamic International Prognostic Scoring System for primary myelofibrosis that incorporates prognostic information from karyotype, platelet count, and transfusion status. J Clin Oncol. 2011;29:392–7.

    Article  PubMed  Google Scholar 

  10. Reilly JT. Idiopathic myelofibrosis: pathogenesis, natural history and management. Blood Rev. 1997;11:233–42.

    Article  CAS  PubMed  Google Scholar 

  11. Koch CA, Li CY, Mesa RA, Tefferi A. Nonhepatosplenic extramedullary hematopoiesis: associated diseases, pathology, clinical course, and treatment. Mayo Clin Proc. 2003;78:1223–33.

    Article  PubMed  Google Scholar 

  12. Verstovsek S, Kantarjian H, Mesa RA, Pardanani AD, Cortes-Franco J, Thomas DA, et al. Safety and efficacy of INCB018424, a JAK1 and JAK2 inhibitor, in myelofibrosis. N Engl J Med. 2010;363:1117–27.

    Article  CAS  PubMed  Google Scholar 

  13. Tefferi A, Thiele J, Orazi A, Kvasnicka HM, Barbui T, Hanson CA, et al. Proposals and rationale for revision of the World Health Organization diagnostic criteria for polycythemia vera, essential thrombocythemia, and primary myelofibrosis: recommendations from an ad hoc international expert panel. Blood. 2007;110:1092–7.

    Article  CAS  PubMed  Google Scholar 

  14. Thiele J, Kvasnicka HM, Facchetti F, Franco V, van der Walt J, Orazi A. European consensus on grading bone marrow fibrosis and assessment of cellularity. Haematologica. 2005;90:1128–32.

    PubMed  Google Scholar 

  15. Hussein K, Pardanani AD, Van Dyke DL, Hanson CA, Tefferi A. International prognostic scoring system-independent cytogenetic risk categorization in primary myelofibrosis. Blood. 2010;115:496–9.

    Article  CAS  PubMed  Google Scholar 

  16. Caramazza D, Begna KH, Gangat N, Vaidya R, Siragusa S, Van Dyke DL, et al. Refined cytogenetic-risk categorization for overall and’leukemia-free survival in primary myelofibrosis: a single center study of 433 patients. Leukemia. 2011;25:82–8.

    Article  CAS  PubMed  Google Scholar 

  17. Mesa RA, Nagorney DS, Schwager S, Allred J, Tefferi A. Palliative goals, patient selection, and perioperative platelet management: outcomes and lessons from 3 decades of splenectomy for myelofibrosis with myeloid metaplasia at the Mayo Clinic. Cancer. 2006;107:361–70.

    Article  PubMed  Google Scholar 

  18. Mesa RA, Silverstein MN, Jacobsen SJ, Wollan PC, Tefferi A. Population-based incidence and survival figures in essential thrombocythemia and agnogenic myeloid metaplasia: an Olmsted County Study, 1976–1995. Am J Hematol. 1999;61:10–5.

    Article  CAS  PubMed  Google Scholar 

  19. Tefferi A, Huang J, Schwager S, Li CY, Wu W, Pardanani A, et al. Validation and comparison of contemporary prognostic models in primary myelofibrosis: analysis based on 334 patients from a single institution. Cancer. 2007;109:2083–8.

    Article  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  21. Visani G, Finelli C, Castelli U, Petti MC, Ricci P, Vianelli N, et al. Myelofibrosis with myeloid metaplasia: clinical and haematological parameters predicting survival in a series of 133 patients. Br J Haematol. 1990;75:4–9.

    Article  CAS  PubMed  Google Scholar 

  22. Rupoli S, Da Lio L, Sisti S, Campanati G, Salvi A, Brianzoni MF, et al. Primary myelofibrosis: a detailed statistical analysis of the clinicopathological variables influencing survival. Ann Hematol. 1994;68:205–12.

    Article  CAS  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  24. Miller JB, Testa JR, Lindgren V, Rowley JD. The pattern and clinical significance of karyotypic abnormalities in patients with idiopathic and postpolycythemic myelofibrosis. Cancer. 1985;55:582–91.

    Article  CAS  PubMed  Google Scholar 

  25. Demory JL, Dupriez B, Fenaux P, Laï JL, Beuscart R, Jouet JP, et al. Cytogenetic studies and their prognostic significance in agnogenic myeloid metaplasia: a report on 47 cases. Blood. 1988;72:855–9.

    CAS  PubMed  Google Scholar 

  26. Tefferi A, Mesa RA, Nagorney DM, Schroeder G, Silverstein MN. Splenectomy in myelofibrosis with myeloid metaplasia: a single-institution experience with 223 patients. Blood. 2000;95:2226–33.

    CAS  PubMed  Google Scholar 

  27. Tefferi A. Myelofibrosis with myeloid metaplasia. N Engl J Med. 2000;342:1255–65.

    Article  CAS  PubMed  Google Scholar 

  28. Panteli KE, Hatzimichael EC, Bouranta PK, Katsaraki A, Seferiadis K, Stebbing J, et al. Serum interleukin (IL)-1, IL-2, sIL-2Ra, IL-6 and thrombopoietin levels in patients with chronic myeloproliferative diseases. Br J Haematol. 2005;130:709–15.

    Article  CAS  PubMed  Google Scholar 

  29. Verstovsek S. Therapeutic potential of Janus-activated kinase-2 inhibitors for the management of myelofibrosis. Clin Cancer Res. 2010;16:1988–96.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Moo-Kon Song.

Ethics declarations

Conflict of interest

The authors have no conflict of interest.

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Song, MK., Chung, JS., Lim, SN. et al. Usefulness of spleen volume measured by computed tomography for predicting clinical outcome in primary myelofibrosis. Int J Hematol 104, 476–484 (2016). https://doi.org/10.1007/s12185-016-2050-y

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12185-016-2050-y

Keywords

Navigation