Significance of cytogenetic-risk categories and refined international prognostic scoring system for overall survival in primary myelofibrosis: A single-center experience

  • Djordje Taušan Military Medical Academy, Pulmonology Clinic
  • Zoran Kostić Military Medical Academy, Clinic for General Surgery ; University of Defence, Faculty of Medicine of Military Medical Academy
  • Damjan Slavković Military Medical Academy, Clinic for General Surgery
  • Branimir Nešković Military Medical Academy, Clinic for General Surgery
  • Dubravko Bokonjić University of Defence, Faculty of Medicine of Military Medical Academy
  • Sandra Šipetić-Grujičić University of Belgrade, Faculty of Medicine, Institute of Epidemiology
  • Nenad Ratković University of Defence, Faculty of Medicine of Military Medical Academy; Military Medical Academy, Clinic for Emergency Internal Medicine
  • Vesna Šuljagić Military Medical Academy, Department of Nosocomial Infections Control, University of Defence, Faculty of Medicine of Military Medical Academy
Keywords: cross infection;, pneumonia;, digestive system surgical procedures;, risk factors;, incidence;, mortality

Abstract


Background/Aim. Primary myelofibrosis (PMF) is a chronic, malignant hematological disease characterized by a leucoerythroblastic blood picture, anisopoikilocytosis tear­drop-shaped erythrocytes, different degrees of bone marrow fibrosis and hepatosplenomegaly due to extramedullary he­matopoiesis. Among genetic specificities of the disease, those that stand out are chromosomal aberrations in pathological, myeloid blood cells. The aim of this study was to examine the prognostic significance of clinical, hema­tologic and cytogenetic parameters in PMF. Methods. A retrospective study included 144 patients with PMF. Karyotypes were analyzed using conventional cytogenetic methods. Results. The chromosome examinations were successful in 126 (88%) patients and failed in the remainder ones (12%). Karyotype was abnormal in 36/126 (29%) subjects at presentation. The most frequent changes in­cluded +9, 13q- and 20q- (28%). Other abnormalities were: aberrations of chromosome 18 and 16, deletions (9q-, 12p-, 7q-, 5q-, 6q-, 8q-), trisomies (+1q, +8, +10, +21), mono­somies (-7, -11), 3q inversion and loss of Y chromosome. We detected four novel balanced translocations in PMF: t(17;22)(q11;q13), t(15;17)(q22;q25), t(9;12)(q22;q24) and t(2;4)(q21;p16), one constitutional translocation-rob(13;14)(q10;q10) and some new karyotype anomalies ˗ deletion of both homologues, hyperdiploidy and the coex­istence of unrelated pathological clones. Conclusion. Chromosomal aberrations had a significant influence on overall survival of patients with PMF according to the re­fined cytogenetic-risk of the International Prognostic Scor­ing System (Refined CIPSS) (= 0.004). Our patients matched the pattern of chromosome aberrations usually ob­served in PMF but some newly registered, balanced translocations and other rare karyotype anomalies were recorded as well. 

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Published
2021/05/06
Section
Original Paper