Myelofibrosis

Primary myelofibrosis (PMF) is a Philadelphia chromosome negative myeloproliferative neoplasm (MPN) with adverse prognosis and is associated with bone marrow fibrosis and extramedullary hematopoiesis. Its diagnosis is challenging, and for effective management, it is important to differentiate it from other myeloproliferative disorders such as CML, PV, and ET. In this chapter, a clinical case scenario is presented followed by a step-by-step evidence-based approach to utilize specialized and advanced tests to unravel final diagnosis of primary MF. A comprehensive description of pathophysiology of disease, role of transforming growth factor beta (TGF-β), differentiation of PMF from other myeloproliferative neoplasms and correlation of signs and symptoms with results of investigations are discussed in question–answer format. Salient points are summarized for quick revision. To gain expertise on this topic and enhance your skills for effective planning of management, read this chapter on primary myelofibrosis.