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Encyclopedia > Myelofibrosis
Myelofibrosis
Classification & external resources
ICD-10 C94.5, D47.1
ICD-9 289.89
ICD-O: 9932, 9961
DiseasesDB 8616
MeSH D009191

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. The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or disease. ... The International Statistical Classification of Diseases and Related Health Problems 10th Revision (ICD-10) is a coding of diseases and signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or diseases, as classified by the World Health Organization (WHO). ... // C00-D48 - Neoplasms (C00-C14) Malignant neoplasms, lip, oral cavity and pharynx (C00) Malignant neoplasm of lip (C01) Malignant neoplasm of base of tongue (C02) Malignant neoplasm of other and unspecified parts of tongue (C03) Malignant neoplasm of gum (C04) Malignant neoplasm of floor of mouth (C05) Malignant neoplasm of... // C00-D48 - Neoplasms (C00-C14) Malignant neoplasms, lip, oral cavity and pharynx (C00) Malignant neoplasm of lip (C01) Malignant neoplasm of base of tongue (C02) Malignant neoplasm of other and unspecified parts of tongue (C03) Malignant neoplasm of gum (C04) Malignant neoplasm of floor of mouth (C05) Malignant neoplasm of... The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or disease. ... The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. ... The International Classification of Diseases for Oncology (ICD-O) is a domain specific extension of the International Statistical Classification of Diseases and Related Health Problems for tumor diseases. ... The Disease Bold textDatabase is a free website that provides information about the relationships between medical conditions, symptoms, and medications. ... Medical Subject Headings (MeSH) is a huge controlled vocabulary (or metadata system) for the purpose of indexing journal articles and books in the life sciences. ... The myeloproliferative diseases are a group of diseases of the bone marrow where excess cells are produced. ... Grays Anatomy illustration of cells in bone marrow. ... Fibrosis is the formation or development of excess fibrous connective tissue in an organ or tissue as a reparative or reactive process, as opposed to formation of fibrous tissue as a normal constituent of an organ or tissue. ...

Contents

Presentation

The replacement of the bone marrow tissue reduces the patient's ability to generate new blood cells resulting in progressive anemia. A prime feature is "extramedullary hematopoeisis", i.e. the remaining blood-forming cells migrate to other sites in the body, e.g. the liver or spleen. Patients will typically have an enlarged spleen and liver, (hepatosplenomegaly), and examination of the blood cells will show "teardrop cells". Causes of death include infection, bleeding, organ failure, portal hypertension, and leukemic transformation. The liver is an organ present in vertebrates and some other animals. ... The spleen is an organ located the abdomen, where it functions in the destruction of old white blood cells and holding a reservoir of blood. ... Hepatosplenomegaly is the simultaneous enlargement of both the liver (hepatomegaly) and the spleen (splenomegaly). ... Poikilocytosis is an increase in the number of abnormally shaped red blood cells as seen on a blood film. ...


Diagnosis

In order to diagnose myelofibrosis, the following criteria must be fulfilled:

The myeloproliferative disorders are relatively rare hematologic malignancies which include: Polycythemia vera (PV), Essential thrombocythemia (ET), Agnogenic myeloid metaplasia (AMM), Idiopathic myelofibrosis (IMF) Chronic myelogenous leukemia (CML). ... A leucoerythroblastic picture on blood film can be the bone marrow response to any irritation including marrow infiltration [causing immature red cells]. Marrow infilitrative disorders include myelomas, malignancy, myelofibrosis etc. ... Blood films, Giemsa stained A blood film or peripheral blood smear is a slide made from a drop of blood, that allows the cells to be examined. ... Splenomegaly is an enlargement of the spleen, which usually lies in the left upper quadrant (LUQ) of the human abdomen. ... Grays Anatomy illustration of cells in bone marrow. ...

Treatment

Typically affecting patients older than 60 years old, it is a chronic and debilitating condition. The median survival for those patients diagnosed after age 60 ranges from 3.5 to 10 years. Currently the only known cure is a bone marrow transplant. The disease progresses very slowly, however, and treated patients can live for five, ten, and even eighteen years after diagnosis. Since the typical patient is older, and the bone marrow replacement treatment is very rigorous, painful and high risk--involving the use of chemotherapy to destroy the patient's existing marrow tissue--the procedure is often not used.


There are other treatments available to reduce the effects of the disease, which are repeated at regular intervals to maintain quality of life for the patient.


Notes

  1. ^ Older terms include "myelofibrosis with myeloid metaplasia" and "agnogenic myeloid metaplasia". The World Health Organization utilizes the name "chronic idiopathic myelofibrosis", while the International Working Group on Myelofibrosis Research and Treatment calls the disease "primary myelofibrosis".

  Results from FactBites:
 
Myelofibrosis (1082 words)
Myelofibrosis is a rare disease of the bone marrow in which collagen builds up fibrous scar tissue inside the marrow cavity.
Myelofibrosis occurs with equal frequency in women and men, but in children it affects girls twice as often as it does boys.
Myelofibrosis is caused by an abnormality in a single stem cell, which causes it to grow out of control.
Myelofibrosis (146 words)
Myelofibrosis (also called agnogenic myeloid metaplasia) is a myeloproliferative disorder in which the bone marrow is initially over-active but then develops scar tissue (fibrosis).
The term idiopathic means without known cause and differentiates this form of myelofibrosis from secondary myelofibrosis which may complicate other bone marrow diseases.
In myelofibrosis this network is coarsened and thickened so that normal blood cell production is progressively reduced.
  More results at FactBites »

 
 

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