Myelofibrosis diagnostic study of choice

Myelofibrosis Microchapters
Home
Patient Information
Overview
Historical Perspective
Classification
Pathophysiology
Causes
Differentiating Myelofibrosis from other Diseases
Epidemiology and Demographics
Risk Factors
Screening
Natural History, Complications and Prognosis
Diagnosis
Diagnostic Study of Choice
History and Symptoms
Physical Examination
Laboratory Findings
Electrocardiogram
X-ray
Echocardiography and Ultrasound
CT scan
MRI
Other Imaging Findings
Other Diagnostic Studies
Treatment
Medical Therapy
Surgery
Primary Prevention
Secondary Prevention
Cost-Effectiveness of Therapy
Future or Investigational Therapies
Case Studies
Case #1
Myelofibrosis diagnostic study of choice On the Web
Most recent articles
Most cited articles
Review articles
CME Programs
Powerpoint slides
Images
American Roentgen Ray Society Images of Myelofibrosis diagnostic study of choice All Images X-rays Echo and Ultrasound CT Images MRI
Ongoing Trials at Clinical Trials.gov
US National Guidelines Clearinghouse
NICE Guidance
FDA on Myelofibrosis diagnostic study of choice
CDC on Myelofibrosis diagnostic study of choice
Myelofibrosis diagnostic study of choice in the news
Blogs on Myelofibrosis diagnostic study of choice
Directions to Hospitals Treating Myelofibrosis
Risk calculators and risk factors for Myelofibrosis diagnostic study of choice

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]

Overview

Diagnosis of myelofibrosis may be made based upon a thorough clinical evaluation, detailed patient history, and specialized tests. The World Health Organization (WHO) has set the criteria for diagnosing primary myelofibrosis (PMF). It has determined set rules for distinguishing the prefibrotic/early (pre-primary myelofibrosis) phase and the overtly fibrotic (overt primary myelofibrosis) phase. The World Health Organization (WHO) has also introduced a proposed revised criteria for primary myelofibrosis (PMF).

Diagnostic Criteria

2001 World Health Organization (WHO) criteria for prefibrotic/early (pre-primary myelofibrosis) phase

Clinical findings	Morphological findings
Spleen and liver No or mild splenomegaly or hepatomegaly	Blood No or mild leukoerythroblastosis No or mild red blood cell poikilocytosis Few if any dacryocytes
Hematology (variable) Mild anemia Mild to moderate leukocytosis Mild to marked thrombocytosis	Bone marrow Hypercellularity Neutrophilic proliferation Megakaryocytic proliferation

2001 World Health Organization (WHO) criteria for overtly fibrotic (overt primary myelofibrosis) phase

Clinical findings	Morphological findings
Spleen and liver Moderate to marked splenomegaly or hepatomegaly	Blood Leukoerythroblastosis Prominent red blood cell poikilocytosis Prominent dacryocytosis
Hematology (variable) Leukoerythroblastosis White blood cells decreased to elevated Platelet count decreased to elevated	Bone marrow Reticulin and/or collagen fibrosis Decreased cellularity Dilated marrow sinuses Intraluminal hematopoiesis Neutrophilic proliferation Prominent megakaryocytic proliferation Megakaryocytic atypia* New bone formation (osteosclerosis) *Clustering of megakaryocytes, abnormally lobulated megakaryocytic nuclei, naked megakaryocytic nuclei

According to the World Health Organization (WHO) diagnostic criteria for primary myelofibrosis, polycythemia vera, and essential thrombocythemia, the diagnosis of primary myelofibrosis is made when all three of the following major diagnostic criteria and at least two minor criteria are met.

Major Criteria	Minor Criteria
I. On bone marrow biopsy, proliferation and atypia of megakaryocytes, with or without reticulin or collagen fibrosis and it must be accompanied by hypercellular bone marrow II. Finding of JAK2-V617F mutation or other clonal marker III. Not meeting the WHO criteria for chronic myeloid leukemia (CML), polycythemia vera (PV), myelodysplastic syndrome (MDS), or other myeloid neoplasm	I. Anemia II. Increased serum lactate dehydrogenase (LDH) III. Leukoerythroblastosis on blood smear IV. Palpable splenomegaly