Myelofibrosis

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Myelofibrosis
Bone marrow: Myelofibrosis: High mag HandE 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	C 94.5, D 47.1
ICD-9	289.89
ICD-O:	9932, 9961
DiseasesDB	8616
MeSH	D009191


Myelofibrosis Microchapters
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Patient Information

Overview

Historical Perspective
Classification

Pathophysiology

Causes

Differentiating Myelofibrosis from other Diseases

Epidemiology and Demographics

Risk Factors

Natural History, Complications and Prognosis

Diagnosis

Staging

History and Symptoms

Physical Examination

Laboratory Findings

X Ray

CT

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 On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Myelofibrosis 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

CDC on Myelofibrosis

Myelofibrosis in the news

Blogs on Myelofibrosis</small>

Directions to Hospitals Treating Myelofibrosis

Risk calculators and risk factors for Myelofibrosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Synonyms and keywords: Assmann's Disease; Heuck-Assmann disease; agnogenic myeloid metaplasia; chronic idiopathic myelofibrosis; primary myelofibrosis

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Myelofibrosis from other Diseases

Epidemiology and Demographics

Risk Factors

Natural History, Complications and Prognosis

Diagnosis

Treatment

Case Studies

Case # 1

Related chapters

Myelophthisic anemia

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, Eosinopenia, Basopenia)

See also hematological malignancy and immune disorders

v • d • e

Myeloid Hematological malignancy/leukemia histology (ICD-O 9590-9989, C81-C96, 200-208)

CFU-GM/
and other granulocytes

CFU-GM

Myelocyte	AML: Acute myeloblastic leukemia (M0, M1, M2), APL/M3 MP (Chronic neutrophilic leukemia)
Monocyte	AML (AMoL/M5, Myeloid dendritic cell leukemia) CML (Philadelphia chromosome, Accelerated phase chronic myelogenous leukemia)
Myelomonocyte	AML (M4) MD-MP (Juvenile myelomonocytic leukemia, Chronic myelomonocytic leukemia)
Other	Histiocytosis

CFU-Baso

AML (Acute basophilic)

CFU-Eos

AML (Acute eosinophilic)
MP (Chronic eosinophilic leukemia/Hypereosinophilic syndrome)

MEP

CFU-Meg	AML (AMKL/M7) MP (Essential thrombocytosis)
CFU-E	AML (Erythroleukemia/M6) MP (Polycythemia vera) MD (Refractory anemia, Refractory anemia with excess of blasts, Chromosome 5q deletion syndrome, Sideroblastic anemia, Paroxysmal nocturnal hemoglobinuria, Refractory cytopenia with multilineage dysplasia)