Chronic myeloproliferative disorders (CMD): clinicopathologic and molecular features

Written by Horváth Emőke, Vizi Emőke oh., Pávai Zoltán, Pap Zsuzsa, Demian Smaranda, Dorcioman Bogdana, Molnár Enikő

The Philadelphia chromosome-negative chronic myeloproliferative disorders (CMPD), polycythemia vera (PV), essential thrombocythemia (ET) and chronic idiopathic myelofibrosis (CIMF), have overlapping clinical features but exhibit different natural histories and different therapeutic requirements. The bone marrow criteria of the World Health Organization (WHO) are defined by pathologists to explicitly define the pathological criteria for the diagnostic differentiation of ET, PV, and prefibrotic and fibrotic CIMF. Material and method. Our study was composed from 17 bone marrow trephine biopsy diagnosticated with CMPD. We used special and immunohistochemical stainings for positive and differential diagnosis. In order to complement initial diagnosis we studied the tyrosin kinase mutation (JAK2 V617) with PCR method using periferial blood samples from patients diagnosticated with PV. Conclusions. The use of biological markers including JAK2 V617 PCR test and peripheral blood parameters combined with bone marrow histopathology has a high sensitivity and specificity (almost 100%) to diagnose the early and overt stages of ET, PV and CIMF in JAK2 V617F positive and negative MPDs.

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