Myelofibrosis Disease Summary

Overview

Myelofibrosis (MF) is a clonal myeloproliferative stem cell disorder, and its characteristics are described in the Introduction section.

Myelofibrosis occurs mainly among middle-aged and older adults with a median age of onset at 67 years old. Incidence and prevalence of this cancer worldwide and regionally can be found in the Epidemiology section.

The pathogenesis of myelofibrosis is discussed in the Pathophysiology section.

The Classification section states that myelofibrosis presents either as a de novo disorder (primary myelofibrosis) or can develop from the transformation of polycythemia vera and essential thrombocythemia. Primary myelofibrosis may be sub-classified into prefibrotic or early-stage primary myelofibrosis and overt fibrotic-stage primary myelofibrosis.

Myelofibrosis_Disease Summary

History and Physical Examination

The Clinical Presentation section enumerates the early manifestations of myelofibrosis, among which are fatigue, enlarged spleen, weight loss, low-grade fever, night sweats, and bone pain.

During history-taking, it is important to assess cardiovascular risk factors and medication or transfusion history as stated in the History section.

In the Physical Examination section, it is stated that it is essential to look for signs of spleen enlargement since it is the hallmark of primary myelofibrosis.

Diagnosis

The Laboratory Tests and Ancillaries section includes discussion on the essential tests (eg complete blood count, metabolic panel), biopsy and molecular and genetic analysis done in patients with myelofibrosis.

The Diagnosis or Diagnostic Criteria section enumerates the International Consensus Classification (ICC)and 2022 World Health Organization (WHO)diagnostic criteria of primary myelofibrosis and International Working Group for Myelofibrosis Research and Treatment (IWG-MRT) diagnostic criteria post-polycythemia vera myelofibrosis. The myelofibrosis grading is also in this section.

The Differential Diagnosis section enumerates the diseases that should be ruled out before diagnosing myelofibrosis. 

Management

The Evaluation section enumerates the different risk stratification systems that can be used in categorizing patients according to risks. Based on the results of prognostic scoring systems, the section discusses the classification of patients with myelofibrosis, whether patient is at low, intermediate or higher risk.

Goals of treatment, therapeutic recommendations and goals of clinical trials are discussed in the Principles of Therapy section.

The Pharmacological Therapy section includes the discussion of drug options used in the treatment of patients with myelofibrosis. This includes interferons and Janus Kinase 2 (JAK2) inhibitors. In this section also discussed the treatment recommendations based on risk stratification and symptom burden assessment and management of myelofibrosis-associated anemia.

It is enumerated in the Nonpharmacological section the different strategies for symptom management as well as discussions on supportive therapy, transfusion, antifibrolytic agents, iron chelation, cytoreductive therapy, monitoring and treatment of infections, prophylaxis for tumor lysis syndrome and transjugular intrahepatic portosystemic shunts.  

Splenectomy is an option for patients with symptomatic splenomegaly refractory to pharmacological therapy.  While allogeneic hematopoietic stem cell transplantation is the only potentially curative treatment option and modality capable of prolonging the survival of patients with myelofibrosis. Discussions of these 2 surgical procedures are in the Surgery section.

Radiotherapy is an alternative to splenectomy in patients with symptomatic splenomegaly but not eligible for surgery and it is discussed in the Radiation Therapy section.

Treatment response monitoring and management of accelerated/blast phase myeloproliferative discussions are in the Monitoring section. While the Prognosis section enumerates the prognostic markers.