What is pre-fibrotic myelofibrosis and how should it be managed in 2018? 2018

Natalia Curto-Garcia, and Jean-Christophe Ianotto, and Claire N Harrison
Department of Haematology, Guy's and St Thomas' NHS Foundation Trust, London, UK.

The 2016 World Health Organization (WHO) classification for myeloproliferative neoplasms (MPN) divided myelofibrosis (MF) into pre-fibrotic (pre-MF) and overt-MF categories. This new classification, particularly the entity pre-MF, has been a subject of discussion between experts. Important questions have been raised in recent years, such as the need for bone marrow trephine for diagnosis; how this is interpreted and the weighting given to it in assigning a diagnosis; determination of prognosis for pre-MF patients; including which scoring system to use and, ultimately, an evidence-based management plan for this group of patients. Many pre-MF patients present as young adults, with thrombocytosis, elevated lactate dehydrogenase levels and increased bone marrow fibrosis (i.e. ≥ grade 1). Current management strategies differ in view of age, comorbidities and bone marrow features and the opinion of the managing clinicians. Prognostic scoring systems have some limitations regarding this entity, and at the present time there is limited information about the overall survival and incidence of progression to overt-MF and acute leukaemia for pre-MF. In this clinically focussed article, we review the main characteristics of this new disease category in view of the current published literature and illustrate our discussion with some real patient cases. Lastly, we propose a management strategy for patients to whom this diagnostic label is applied.

UI MeSH Term Description Entries
D009196 Myeloproliferative Disorders Conditions which cause proliferation of hemopoietically active tissue or of tissue which has embryonic hemopoietic potential. They all involve dysregulation of multipotent MYELOID PROGENITOR CELLS, most often caused by a mutation in the JAK2 PROTEIN TYROSINE KINASE. Disorder, Myeloproliferative,Disorders, Myeloproliferative,Myeloproliferative Disorder
D011379 Prognosis A prediction of the probable outcome of a disease based on a individual's condition and the usual course of the disease as seen in similar situations. Prognostic Factor,Prognostic Factors,Factor, Prognostic,Factors, Prognostic,Prognoses
D005355 Fibrosis Any pathological condition where fibrous connective tissue invades any organ, usually as a consequence of inflammation or other injury. Cirrhosis,Fibroses
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D014944 World Health Organization A specialized agency of the United Nations designed as a coordinating authority on international health work; its aim is to promote the attainment of the highest possible level of health by all peoples. Organization, World Health,WHO
D055728 Primary Myelofibrosis A de novo myeloproliferation arising from an abnormal stem cell. It is characterized by the replacement of bone marrow by fibrous tissue, a process that is mediated by CYTOKINES arising from the abnormal clone. Agnogenic Myeloid Metaplasia,Bone Marrow Fibrosis,Chronic Idiopathic Myelofibrosis,Fibrosis, Bone Marrow,Idiopathic Myelofibrosis,Myelofibrosis,Myelofibrosis With Myeloid Metaplasia,Myeloid Metaplasia,Myelosclerosis,Myelosis, Nonleukemic,Agnogenic Myeloid Metaplasias,Bone Marrow Fibroses,Fibroses, Bone Marrow,Metaplasia, Agnogenic Myeloid,Metaplasia, Myeloid,Metaplasias, Agnogenic Myeloid,Metaplasias, Myeloid,Myelofibroses,Myelofibroses, Primary,Myelofibrosis, Primary,Myeloid Metaplasia, Agnogenic,Myeloid Metaplasias,Myeloid Metaplasias, Agnogenic,Myeloscleroses,Myeloses, Nonleukemic,Nonleukemic Myeloses,Nonleukemic Myelosis,Primary Myelofibroses
D019468 Disease Management A broad approach to appropriate coordination of the entire disease treatment process that often involves shifting away from more expensive inpatient and acute care to areas such as preventive medicine, patient counseling and education, and outpatient care. This concept includes implications of appropriate versus inappropriate therapy on the overall cost and clinical outcome of a particular disease. (From Hosp Pharm 1995 Jul;30(7):596) Disease Managements,Management, Disease,Managements, Disease

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