DIAGNOSTIC APPROACH TO MYELOPROLIFERATIVE NEOPLASMS (MPNS)

Abstract

Anil Kumar

BACKGROUND MPNs are caused by clonal proliferation of a pluripotent haematopoietic progenitor. They neoplasms are characterised by an excessive production of mature blood cells of myeloid lineage, which are independent and/or hypersensitive to cytokines for cell survival, proliferation and differentiation. MATERIALS AND METHODS The 2008 WHO classification system for haematological malignancies comprehensive and includes histology and genetic information. According to WHO (2008), MPNs are classified as followsMyeloproliferative Neoplasms (MPN), chronic myelogenous leukaemia, BCR-ABL I -positive, polycythaemia vera, primary myelofibrosis, essential thrombocythaemia, chronic eosinophilic leukaemia, not otherwise specified, mastocytosis, myeloproliferative neoplasms, unclassifiable. RESULTS Diagnosis of samples indicate that CML is the commonest MPN and in case of BCR-ABL1 negative MPNs, there is an increasing gamut of mutations, which are being discovered, nowadays. Hence, the diagnostic approach has to be updated slightly to carry out correct diagnosis of such mutations. Details of the suggested approach appears later in the article. CONCLUSION CML is the commonest MPN and in case of BCR-ABL1 negative MPNs, there is an increasing gamut of mutations, which are being discovered, nowadays. CALR is the 2nd frequently mutated gene in BCR-ABL negative MPNs. Treatment and prognosis of MPNs are guided by their molecular characteristics. For e.g., imatinib is useful in case of BCRABL1 fusion gene defects and JAK2 inhibitors are effective in case of JAK2 mutated PV. CALR mutated MPNs have a better prognosis than other types of mutations. Thus, it is important to correctly diagnose the MPNs for better and accurate postdiagnosis treatment.

image