Myelofibrosis

The myelofibrosis (OMF ), and myelofibrosis, bone marrow fibrosis or osteofibrosis called (English osteomyelofibrosis ) is a myeloproliferative disorder and is a progressive disease of the bone marrow; they can be used as an independent disease (primary or idiopathic myelofibrosis, PMF, IMF) occur or as a result of another disease that alters the bone marrow, for example, in polycythemia vera, manifest secondary.

Frequency

The myelofibrosis is a rare disease that affects women more frequently. The onset peaks between the ages of 50 and 60. The incidence is approximately 1,5:100.000 / year or approximately 1200 cases per year in Germany.

Causes

Possible causes for the OMF are:

  • In response to effects of chemical or physical noxious agents, such as ionizing radiation.
  • Acquired genetic defects.
  • Secondary disease as a result of another disease that alters the bone marrow, for example, in polycythemia vera.
  • Other unknown causes.

Pathogenesis

In the OMF leads to a progressive fibrosis ( obliteration ) of the blood-forming bone marrow tissue. The blood production is disturbed. Probably set free the altered blood cells and their precursors substances that may cause an increased fiber formation in the bone marrow tissue. The blood formation takes place more and more in the liver and spleen, as the bone marrow due to increasing fiber formation can no longer adequately fulfill its task. Consequence may be an enlarged spleen and / or liver.

Clinic / symptoms

At the beginning of the disease the patients remain asymptomatic, only after a long time the disease is diagnosed.

A deficient or defective blood formation may provoke the following symptoms:

  • Various forms of anemia
  • In the beginning, there is a tendency to thrombosis
  • Later bleeding can occur due to thrombocytopenia.
  • Leukocytoses
  • Leftward shift of the blood picture (occurrence of immature precursors of blood cells in the peripheral blood)
  • Increased leucocyte alkaline phosphatase
  • Erythrocytes in a blood smear showing a " Teardrop " ( = Dakryocyten, Teardrop poikilocytosis ).

Other symptoms:

  • Fatigue
  • Anorexia
  • Fatigue
  • Diarrhea
  • Night sweats
  • A greatly enlarged spleen ( splenomegaly) or liver ( hepatomegaly ) can cause abdominal pain.
  • Joint pain
  • Blurred vision

Diagnostics

  • History: fatigue and weight loss (see Symptoms )
  • Blood picture shows a left shift ( occurrence of immature precursors of blood cells in the peripheral blood; see also erythrocyte distribution width )
  • Palpation and ultrasound examination of the spleen and liver ( upper abdominal pain )
  • X-ray: any calcification ( = sclerosis) of the bone observable.
  • Diagnosis by bone marrow aspiration ( KMP ) and distinguish it from other myeloproliferative disorders.

Differential diagnoses

  • Chronic myeloid leukemia ( CML)
  • Knochenmarkkarzinose

Therapy

The OMF is a chronic and advanced disease and should be monitored and treated carefully by experts ( hematologist ). Therefore, only roughly:

  • Bone marrow transplantation (BMT )
  • Possibly drugs such as α - interferon, androgens, hydroxyurea, thalidomide, erythropoietin, Ruxolitinib
  • Blood transfusions

Forecast

Since the prognosis depends on many factors (primary or secondary disease, other diseases) is, there are no generalized representable results. The median survival after diagnosis concerns depending on the study 4-7 years, although long- term survivors have been described up to 20 years. The main causes of death are severe infections (due to bone marrow failure) and transformation into an aggressive form of acute leukemia.

International Classification of Diseases for Oncology Leukocytosis Blood film Medical history Red blood cell distribution width
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