What is myelofibrosis with myeloid metaplasia?

What is myelofibrosis with myeloid metaplasia?

Purpose: Myelofibrosis with myeloid metaplasia (MMM) is a chronic myeloproliferative disorder characterized by bone marrow fibrosis and extramedullary hematopoiesis.

How does myelofibrosis cause portal hypertension?

Myelofibrosis is a hematologic condition that predisposes to the formation of large and small portal venous clots. Portal injury is believed to underlie the mechanism of development of noncirrhotic portal hypertension in this population.

What are the final stages of myelofibrosis?

Myelofibrosis Prognosis and Survival

  • Shortened survival.
  • Increased risk of leukemic transformation.
  • Severe anemia, often creating the need for frequent red blood cell transfusions.
  • Severe constitutional symptoms.
  • Marked leukocytosis or thrombocytosis.

Does myelofibrosis cause high blood pressure?

Over time, myelofibrosis can lead to several complications, including: Increased blood pressure in your liver. Increased blood flow from an enlarged spleen can raise the pressure in the portal vein in your liver, causing a condition called portal hypertension.

What is the life expectancy of a person with myelofibrosis?

Transcript:Srdan Verstovsek, MD, PhD: Myelofibrosis is one of the myeloproliferative neoplasms, a chronic disease of the bone marrow. It is, unfortunately, the aggressive type. It does affect the life expectancy of the patients. The average survival is about 5 to 7 years.

Can myelofibrosis cause liver damage?

Growths: Blood cells are supposed to form inside your bone marrow. But myelofibrosis might cause them to grow in other parts of your body, like your lungs, liver, spleen, and digestive tract.

What is the life expectancy for myelofibrosis?

Life expectancy in PMF Primary myelofibrosis, also known as idiopathic myelofibrosis or myelofibrosis with myeloid metaplasia, is a rare disease19, 20 usually affecting elderly people. Median survival ranges from 4 to 5.5 years in modern series6, 7, 8, 9, 10, 11, 12, 13, 14 (Figure 1).

How long will I live with myelofibrosis?

What are symptoms of myeloid metaplasia with myelofibrosis?

Myeloid metaplasia with myelofibrosis may present in a variety of ways. Constitutional symptoms may relate to the catabolic aspects of this disease and include cachexia, fatigue, weight loss, low-grade fever, and night sweats (Table 20-13 ). TABLE 20-13. Myeloid Metaplasia with Myelofibrosis

Are there any disorders similar to primary myelofibrosis?

Symptoms of the following disorders can be similar to those of primary myelofibrosis. Comparisons may be useful for a differential diagnosis. Polycythemia vera is a rare, chronic disorder involving the overproduction of blood cells in the bone marrow (myeloproliferation).

How is primary myelofibrosis treated in asymptomatic patients?

Since the cause of primary myelofibrosis is unknown, treatment is directed toward the specific symptoms present in each patient. In asymptomatic individuals, physicians may recommend that no therapy be given until symptoms appear (watch and wait). Affected individuals receive regular checkups to detect progression of the disease.

How is autologous transplantation used to treat myeloid metaplasia?

A study of 68 patients with symptomatic myelofibrosis and myeloid metaplasia revealed overall response rates of 22% for anemia, 33% for splenomegaly, and 50% for thrombocytopenia. [ 39] This modality, combined with autologous transplantation, has been shown to slow disease progression.