Guideline for the diagnosis and management of myelofibrosis

Abstract

SummaryThe guideline group regarding the diagnosis and management of myelofibrosis was selected to be representative of UK-based medical experts, together with a contribution from a single expert from the USA. MEDLINE and EMBASE were searched systematically for publications in English from 1966 until August 2011 using a variety of key words. The writing group produced the draft guideline, which was subsequently revised by consensus of the members of the General Haematology and Haemato-oncology Task Forces of the British Committee for Standards in Haematology (BCSH). The guideline was then reviewed by a sounding board of UK haematologists, the BCSH and the British Society for Haematology Committee and comments incorporated where appropriate. The criteria used to state levels and grades of evidence are as outlined in the Procedure for Guidelines commissioned by the BCSH; the 'GRADE' system was used to score strength and quality of evidence. The objective of this guideline is to provide healthcare professionals with clear guidance on the investigation and management of primary myelofibrosis, as well as post-polycythaemic myelofibrosis (post-PV MF) and post-thrombocythemic myelofibrosis (post-ET MF) in both adult and paediatric patients.

Treatment options for patients with primary myelofibrosis

“…(3) There are manifestations of constitutional symptoms such as low-grade temperature, night sweats and cachexia. (4) Hepatomegaly due to extramedullary haemopoiesis results in symptoms such as pain, abdominal distension, early satiety, diarrhoea, dyspnoea, portal hypertension and splenic infarction. (4,5) Janus-associated kinase 2 A mutation (Val617Phe) in JAK2 (a tyrosine kinase in the cytoplasm of the bone marrow and blood) is present in nearly every patient with PRV, and half the patients with ET and MF.…”

“…Therefore, radiotherapy is not an alternative for patients who are eligible for surgery. (4) Blood transfusions are recommended for the treatment of anaemia in PMF. This is the standard therapy; however, patients must be assessed individually.…”

“…(4) Fatigue, weakness, night sweats, bone pain, abdominal pain, weight loss, pruritus and cachexia represent the main constitutional symptoms of PMF. Increased pro-inflammatory cytokines and splenomegaly cause these incapacitating symptoms in advanced stages of the disease andaccording to Scherber et al, as cited by Reilly et al (4) -quality of life for PMF patients is similar to that of patients suffering from metastatic cancer. There is no evidence of benefit from conventional agents for the treatment of constitutional symptoms in PMF.…”

“…There is no evidence of benefit from conventional agents for the treatment of constitutional symptoms in PMF. (4) In order to control thrombocytosis; leukocytosis; and symptoms such as bone pains, night sweats, fatigue and weight loss which are associated with hypercatabolism and splenomegaly, myelosuppressive therapy is indicated. First-line medication for hyperproliferation includes hydroxycarbamide.…”

“…In early stages of the disease IFN-α should only be used in patients with proliferative features as it has been shown to slow the disease and even show regression of the fibrosis. (4,9) Allogeneic haemopoietic stem cell transplantation (allo-HSCT) has a potentially curative effect with regression in bone marrow fibrosis. However the reports have been non-comparative.…”