This site is intended for healthcare professionals

Go to /sign-in page

You can view 5 more pages before signing in

Go to /pro/cpd-dashboard page

This page is worth 0.05 CPD credits. CPD dashboard

Go to /account/subscription-details page

This page is worth 0.05 CPD credits. Upgrade to Pro

Myelofibrosis

Last reviewed dd mmm yyyy. Last edited dd mmm yyyy

Authoring team

Myelofibrosis (also known as primary myelofibrosis, agnogenic myeloid metaplasia, chronic idiopathic myelofibrosis, myelofibrosis with myeloid metaplasia, and idiopathic myelofibrosis) is a chronic myeloproliferative disorder characterised by (1,2):

  • varying degrees of fibrosis of the bone marrow
  • extramedullary haemopoiesis with splenomegaly
  • anaemia with leukoerythroblastosis with teardrop poikilocytosis in peripheral blood (3)

The term “myelofibrosis” is being used to describe any increase in bone marrow stromal fibres (without considering the type of fibre or the associated disease) (4)

Marrow fibrosis is thought to occur as a result of increased secretion of platelet derived growth factor.

In myelofibrosis patients the disease initiating mutation is unknown. In a majority, a somatic Janus kinase 2 (JAK2) mutations (JAK2V617F) and in a minority MPL, LNK mutation is present (5)

  • the discovery of mutations in JAK2, CALR, and MPL have uncovered activated JAK-STAT signaling as a primary driver of MF, supporting a rationale for JAK inhibition (6)

Loss of marrow capacity results in extramedullary haematopoiesis in the liver, spleen and lymph nodes.

Reference:


Create an account to add page annotations

Annotations allow you to add information to this page that would be handy to have on hand during a consultation. E.g. a website or number. This information will always show when you visit this page.

The content herein is provided for informational purposes and does not replace the need to apply professional clinical judgement when diagnosing or treating any medical condition. A licensed medical practitioner should be consulted for diagnosis and treatment of any and all medical conditions.

Connect

Copyright 2024 Oxbridge Solutions Limited, a subsidiary of OmniaMed Communications Limited. All rights reserved. Any distribution or duplication of the information contained herein is strictly prohibited. Oxbridge Solutions receives funding from advertising but maintains editorial independence.