Myelofibrosis

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Myelofibrosis Classification and external resources
Bone marrow: Myelofibrosis: High mag H&E excellent example of myelofibrosis. Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology
ICD-10	C94.5, D47.1
ICD-9	289.89
ICD-O:	9932, 9961
DiseasesDB	8616
MeSH	D009191

WikiDoc Resources for Myelofibrosis
Articles
Most recent articles on Myelofibrosis Most cited articles on Myelofibrosis Review articles on Myelofibrosis Articles on Myelofibrosis in N Eng J Med, Lancet, BMJ
Media
Powerpoint slides on Myelofibrosis Images of Myelofibrosis Photos of Myelofibrosis Podcasts & MP3s on Myelofibrosis Videos on Myelofibrosis
Evidence Based Medicine
Cochrane Collaboration on Myelofibrosis Bandolier on Myelofibrosis TRIP on Myelofibrosis
Clinical Trials
Ongoing Trials on Myelofibrosis at Clinical Trials.gov Trial results on Myelofibrosis Clinical Trials on Myelofibrosis at Google
Guidelines / Policies / Govt
US National Guidelines Clearinghouse on Myelofibrosis NICE Guidance on Myelofibrosis NHS PRODIGY Guidance FDA on Myelofibrosis CDC on Myelofibrosis
Books
Books on Myelofibrosis
News
Myelofibrosis in the news Be alerted to news on Myelofibrosis News trends on Myelofibrosis
Commentary
Blogs on Myelofibrosis
Definitions
Definitions of Myelofibrosis
Patient Resources / Community
Patient resources on Myelofibrosis Discussion groups on Myelofibrosis Patient Handouts on Myelofibrosis Directions to Hospitals Treating Myelofibrosis Risk calculators and risk factors for Myelofibrosis
Healthcare Provider Resources
Symptoms of Myelofibrosis Causes & Risk Factors for Myelofibrosis Diagnostic studies for Myelofibrosis Treatment of Myelofibrosis
Continuing Medical Education (CME)
CME Programs on Myelofibrosis
International
Myelofibrosis en Espanol Myelofibrosis en Francais
Businness
Myelofibrosis in the Marketplace Patents on Myelofibrosis
Experimental / Informatics
List of terms related to Myelofibrosis

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Overview

Myelofibrosis with myeloid metaplasia, also known as agnogenic myeloid metaplasia, chronic idiopathic myelofibrosis, and primary myelofibrosis,^[1] was first described in 1879 and is currently classified as a myeloproliferative disease caused by the growth and proliferation of an abnormal bone marrow stem cell, resulting in the replacement of the bone marrow with fibrous connective tissue. An eponym for the disease is Heuck-Assmann disease, or Assmann's Disease.

Presentation

The bone marrow is replaced by collagen fibrosis, impairing the patient's ability to generate new blood cells resulting in a progressive anemia. It is usually reactive following other myeloproliferative disorders, such as polycythemia rubra vera or essential thrombocytosis. Extramedullary hematopoeisis occurs as the haemopoetic cells migrate away from the bone marrow, to the liver and spleen. Patients often have hepatosplenomegaly and poikilocytosis. The mean survival is 5 years and causes of death include infection, bleeding, organ failure, portal hypertension, and leukemic transformation.

Diagnosis

Diagnosis is based upon:

1. Normochromic normocytic anaemia
2. Red cell poikilocytosis on blood film (tear drop RBCs)
3. JAK 2 mutation on Val 617 Phe locus in 50%
4. Raised lactate dehydrogenase
5. Raised neutrophil alkaline phosphatase score
6. Bone marrow biopsy may show increased cellularity and fibrosis

References

v • d • e Pathology: hematology (primarily D50-D77, 280-289)
RBCs/anemia/ hemoglobinopathy (Myeloid)	nutritional anemia: Iron deficiency anemia, Plummer-Vinson syndrome, Megaloblastic anemia (Pernicious anemia) hereditary hemolytic anemia: enzyme disorders (G6PD Deficiency, Pyruvate kinase deficiency,Triosephosphate isomerase deficiency), Thalassemia, Sickle-cell disease/trait, Hereditary spherocytosis, Hereditary elliptocytosis, Hereditary stomatocytosis acquired hemolytic anemia: Autoimmune (Warm), HUS, MAHA, PNH, PCH aplastic anemia: Acquired PRCA, Diamond-Blackfan anemia, Fanconi anemia • Sideroblastic anemia
Coagulation/platelets (Myeloid)	coagulopathy: DIC (Congenital afibrinogenemia, Purpura fulminans) • Hemophilia (A/VII, B/IX, C/XI, XIII) • Von Willebrand disease Purpura: Henoch-Schönlein, ITP (Evans syndrome), TTP primary hypercoagulable state: Protein C deficiency - Protein S deficiency - Antithrombin III deficiency - Factor V Leiden - Activated protein C resistance - Antiphospholipid syndrome other hemorrhagic conditions: Bernard-Soulier syndrome - Glanzmann's thrombasthenia - Grey platelet syndrome - May Hegglin anomaly Essential thrombocytosis - Thrombocytopenia
Monocytes/Macrophages (Myeloid)	WHO-I histiocytosis (Langerhans cell histiocytosis) WHO-II/non-Langerhans-cell histiocytosis (Juvenile xanthogranuloma, Hemophagocytic lymphohistiocytosis) WHO-III/malignant histiocytic disorders (Acute monocytic leukemia, Malignant histiocytosis, Erdheim-Chester disease) -cytosis: Monocytosis -penia: Monocytopenia
Granulocytes (Myeloid)	-cytosis: granulocytosis (Neutrophilia, Eosinophilia, Basophilia) -penia: Granulocytopenia/agranulocytosis (Neutropenia, Kostmann syndrome)
Other/general myeloid	Chronic granulomatous disease
Lymphoid	-cytosis: Lymphocytosis -penia: Lymphopenia
Other	Asplenia/hyposplenism - Methemoglobinemia - Pancytopenia
See also hematological malignancy and immune disorders

v • d • e Pathology: hematology, myeloid hematologic disease (primarily D50-D77, 280-289)
RBCs/ hemoglobinopathy	+ Polycythemia - Macrocytosis - Anemia Nutritional Iron deficiency anemia (Plummer-Vinson syndrome), Megaloblastic anemia (Pernicious anemia) Hemolytic Hereditary enzyme: G6PD Deficiency - Pyruvate kinase deficiency - Triosephosphate isomerase deficiency hemoglobin: Thalassemia - Sickle-cell disease/trait membrane: Hereditary spherocytosis - Hereditary elliptocytosis - Hereditary stomatocytosis Acquired Autoimmune (Warm, Cold), HUS, MAHA, PNH, PCH, Myelophthisic Aplastic Acquired PRCA, Diamond-Blackfan anemia, Fanconi anemia, Sideroblastic anemia Blood tests Normocytic - Microcytic - Macrocytic - Normochromic - Hypochromic Other Methemoglobinemia
Coagulation/platelets/ coagulopathy/ bleeding diathesis	+ Hypercoagulability primary: Antithrombin III deficiency - Protein C deficiency/Activated protein C resistance/Protein S deficiency/Factor V Leiden - Hyperprothrombinemia acquired: DIC (Congenital afibrinogenemia, Purpura fulminans) - autoimmune (Antiphospholipid) Other Essential thrombocytosis - clotting factor: Hemophilia (A/VIII, B/IX, C/XI) • Von Willebrand disease • Hypoprothrombinemia/II - XIII platelet function: Bernard-Soulier syndrome - Glanzmann's thrombasthenia - Gray platelet syndrome - May Hegglin anomaly - Pelger-Huet anomaly Purpura: Henoch-Schönlein, ITP (Evans syndrome), TTP Thrombocytopenia (Heparin-induced thrombocytopenia)
Monocytes/ macrophages	+ Histiocytosis WHO-I (Langerhans cell histiocytosis) WHO-II/non-Langerhans-cell (Juvenile xanthogranuloma, Hemophagocytic lymphohistiocytosis) WHO-III/malignant (Acute monocytic leukemia, Malignant histiocytosis, Erdheim-Chester disease) Other Chronic granulomatous disease -cytosis: Monocytosis - -penia: Monocytopenia
Granulocytes	+ -cytosis: granulocytosis (Neutrophilia, Eosinophilia, Basophilia) - -penia: Granulocytopenia/agranulocytosis (Neutropenia, Kostmann syndrome)
See also hematological malignancy and immune disorders

v • d • e WikiDoc Research Resources for Myelofibrosis (Click show to right to view)
Articles on Myelofibrosis	Most recent articles on Myelofibrosis • Most cited articles on Myelofibrosis • Review articles on Myelofibrosis • Articles on Myelofibrosis in N Eng J Med, Lancet, BMJ
Media (Slides, Video, Images, MP3) on Myelofibrosis	Powerpoint slides on Myelofibrosis • Images of Myelofibrosis • Photos of Myelofibrosis • Podcasts & MP3s on Myelofibrosis • Videos on Myelofibrosis
Evidence Based Medicine Regarding Myelofibrosis	Cochrane Collaboration on Myelofibrosis • Bandolier on Myelofibrosis • TRIP on Myelofibrosis
Cost Effectiveness of Myelofibrosis	Cost Effectiveness of Myelofibrosis
Clinical Trials Involving Myelofibrosis	Ongoing Trials on Myelofibrosis at Clinical Trials.gov • Trial results on Myelofibrosis • Clinical Trials on Myelofibrosis at Google
Guidelines / Policies / Government Resources (FDA/CDC) Regarding Myelofibrosis	US National Guidelines Clearinghouse on Myelofibrosis • NICE Guidance on Myelofibrosis • NHS PRODIGY Guidance • FDA on Myelofibrosis • CDC on Myelofibrosis
Textbook Information on Myelofibrosis	Books and Textbook Information on Myelofibrosis
Pharmacology Resources on Myelofibrosis	Dosing of Myelofibrosis • Drug interactions with Myelofibrosis • Side effects of Myelofibrosis • Allergic reactions to Myelofibrosis • Overdose information on Myelofibrosis • Carcinogenicity information on Myelofibrosis • Myelofibrosis in pregnancy • Pharmacokinetics of Myelofibrosis •
Genetics, Pharmacogenomics, and Proteinomics of Myelofibrosis	Genetics of Myelofibrosis • Pharmacogenomics of Myelofibrosis • Proteomics of Myelofibrosis
Newstories on Myelofibrosis	Myelofibrosis in the news • Be alerted to news on Myelofibrosis • News trends on Myelofibrosis
Commentary on Myelofibrosis	Blogs on Myelofibrosis
Patient Resources on Myelofibrosis	Patient resources on Myelofibrosis • Discussion groups on Myelofibrosis • Patient Handouts on Myelofibrosis • Directions to Hospitals Treating Myelofibrosis • Risk calculators and risk factors for Myelofibrosis
Healthcare Provider Resources on Myelofibrosis	Symptoms of Myelofibrosis • Causes & Risk Factors for Myelofibrosis • Diagnostic studies for Myelofibrosis • Treatment of Myelofibrosis
Continuing Medical Education (CME) Programs on Myelofibrosis	CME Programs on Myelofibrosis
International Resources on Myelofibrosis	Myelofibrosis en Espanol • Myelofibrosis en Francais
Business Resources on Myelofibrosis	Myelofibrosis in the Marketplace • Patents on Myelofibrosis
Informatics Resources on Myelofibrosis	List of terms related to Myelofibrosis

bn:মজ্জাকাঠিন্য

de:Osteomyelofibrose fr:Splénomégalie myéloïde it:Mielofibrosi

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Acknowledgement and Attribution Regarding Sources of Content

Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .