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Myelofibrosis Bone Marrow Samples

by | May 4, 2024 | News Releases | 0 comments

Bay Biosciences provides high-quality, fresh frozen bone marrow aspirate samples, peripheral blood mononuclear cells (PBMC’s), with matched fresh frozen sera (serum), plasma, bio-fluids from patients diagnosed with myelofibrosis.

The sera (serum), plasma and PBMC bio-fluid specimens are processed from myelofibrosis (MF) patient’s peripheral whole-blood using customized collection and processing protocols.

Myelofibrosis Overview

Myelofibrosis (MF) is a bone marrow cancer affecting blood cell production. It causes abnormal development and function of bone marrow cells, leading to fibrous scar tissues.

MF is part of a group of conditions called myeloproliferative neoplasms (MPNs).

Other MPNs can also progress to myelofibrosis. While some people can go years without having symptoms, others have symptoms that get worse due to scarring in their bone marrow.

Myelofibrosis is a rare blood cancer where scar tissue forms in your bone marrow (the soft, spongy tissue inside of your bones). It’s a type of chronic leukemia that involves too many abnormal blood cells being made. Eventually, these cells can replace normal cells.

Your bone marrow produces immature blood-forming cells called stem cells that may develop into red blood cells, white blood cells or platelets. With myelofibrosis, a change (mutation) in a stem cell’s DNA causes the cell to become defective, or a cancer cell, instead. The cell multiplies, passing the mutation onto new cells.

Over time, more abnormal cancer cells are produced. Some of these cells create inflammation that causes scar tissue to form in your bone marrow. The scarring and the excess cancer cells can prevent your bone marrow from making healthy blood cells.

Treatment goals mainly involve managing symptoms and conditions that arise, including anemia and an enlarged spleen.

How Common is Myelofibrosis?

Myelofibrosis is rare disease, with about 1.5 cases reported per 100,000 people each year in the United States. It affects people regardless of sex. People of any age can have myelofibrosis, although it’s more likely to be diagnosed in people over 50. Children with myelofibrosis are usually diagnosed before age 3.

Types of Myelofibrosis

Following are two types of myelofibrosis:

  • Primary myelofibrosis is myelofibrosis that occurs on its own.
  • Secondary myelofibrosis arises secondary to other blood disorders, including primary thrombocytosis orpolycythemia vera. Secondary myelofibrosis accounts for about 10% to 20% of diagnoses.


Signs and Symptoms of Myelofibrosis

Myelofibrosis tends to come on slowly, and many people don’t notice symptoms at first. However, as it progresses and starts to interfere with blood cell production, its symptoms may include:

Causes of Myelofibrosis

Researchers don’t know what causes myelofibrosis, but they know it’s associated with DNA changes in specific genes. Myelofibrosis is associated with a genetic mutation in blood stem cells. Proteins called Janus-associated kinases (JAKs) play a role in myelofibrosis.

JAKs regulate the production of blood cells in bone marrow by signaling the cells to divide and grow. If the JAKs become overactive, too many or too few blood cells will be produced.

About 60% to 65 % of people with myelofibrosis have a mutation in the JAK2 gene. Another 5% to 10% have a mutation in the myeloproliferative leukemia (MPL) gene. A mutation called calreticulin (CALR) accounts for approximately 20% to 25 % of myelofibrosis cases.

How Myelofibrosis Effects The Body?

The abnormal blood cell production can lead to a variety of conditions, including:

  • Anemia: A deficiency of red blood cells. Your red blood cells help transport oxygen throughout your body. Too few red blood cells deprive your tissues of oxygen, causing symptoms like fatigue, weakness and shortness of breath.
  • Thrombocytopenia: A deficiency of platelets. Platelets help your blood clot when you’re injured. A low platelet count may make you more susceptible to bleeding and bruising.
  • Splenomegaly: An enlarged spleen. Your spleen controls your number of blood cells and removes damaged red blood cells from your body. Too many abnormal blood cells can overwork your spleen, causing it to enlarge. Splenomegaly may feel like a sensation of fullness or discomfort in the upper left section of your abdomen.
  • Extramedullary hematopoietic: Abnormal growth of blood-forming cells outside of your bone marrow. These cells may grow in other body parts, such as your lungs, gastrointestinal tract, spinal cord, brain or lymph nodes. The cells can form masses (tumors) that press on organs or impair their function.
  • Portal hypertension: An increase in blood pressure in the vein that carries blood from your spleen to your liver. It likely results from damage to your veins related to extramedullary hematopoiesis.

  • In about 12% of all cases, primary myelofibrosis progresses to acute myeloid leukemia (AML), a very aggressive form of blood cancer.


Risk Factors of Myelofibrosis

Generally speaking, MF is rare, occurring in only about 4-6 out of every 100,000 people in the United States.

Some sources suggest that primary MF is as rare as 0.8-2.1 per 100,000 people yearly. For secondary MF, the prevalence depends on the exact condition causing it.

However, several things can increase your risk of developing it, including:

  • Age: While people of any age can have MF, it’s usually diagnosed in those over the age of 50.
  • Another blood disorder.: Some people develop secondary MF as a complication of another condition.
  • Exposure to chemicals: Myelofibrosis has been associated with exposure to certain industrial chemicals, including toluene and benzene.
  • Exposure to radiation: Individuals who’ve been exposed to radioactive material may have an increased chance of developing MF.


Diagnosis of Myelofibrosis

Myelofibrosis usually shows up on a routine complete blood count (CBC). Patients with myelofibrosis tend to have very low levels of red blood cells and unusually high or low levels of white blood cells and platelets.

Based on the results of your CBC test, your doctor might also do a bone marrow biopsy. This involves taking a small sample of your bone marrow and looking at it more closely for signs of myelofibrosis, such as scarring.

You may also need an X-ray or MRI scan to rule out any other potential causes of your symptoms or CBC results.

Treatment of Myelofibrosis

Myelofibrosis treatment usually depends on the types of symptoms you have. Many common myelofibrosis symptoms are associated with an underlying condition caused by myelofibrosis, such as anemia or an enlarged spleen.

Treating Anemia

If myelofibrosis is causing severe anemia, you may need:

  • Blood transfusions: Regular blood transfusions can increase your red blood cell count and reduce anemia symptoms, such as fatigue and weakness.
  • Hormone therapy: A synthetic version of the male hormone androgen may promote red blood cell production in some people.
  • Corticosteroids: These may be used with androgens to encourage red blood cell production or decrease their destruction.
  • Prescription medications: Immunomodulatory medications, such as thalidomide (Thalomid) and lenalidomide (Revlimid), may improve blood cell counts. They may also help with symptoms of an enlarged spleen.

Treating an Enlarged Spleen

If you have an enlarged spleen related to myelofibrosis  that’s causing problems, your doctor might recommend:

  • Radiation therapy: Radiation therapy uses targeted beams to kill cells and reduce the size of the spleen.
  • Chemotherapy: Some chemotherapy drugs may reduce the size of your enlarged spleen.
  • Surgery: A splenectomy is a surgical procedure that removes your spleen. Your doctor might recommend this if you aren’t responding well to other treatments.

Treating Mutated Genes

A drug called ruxolitinib (Jakafi) was approved by the Food and Drug Administration in 2011 to treat the symptoms associated with MF. Ruxolitinib targets a specific genetic mutation that may be the cause of myelofibrosis.

In clinical trials, it was shown to reduce the size of enlarged spleens, reduce the symptoms of MF, and improve prognosis.

Additional drugs, Inrebic (fedratinib), Vonjo (pacritinib), and Ojjaara (momelotinib), were approved to treat certain types of MF in 2019, 2022, and 2023, respectively. Like Jakafi, these drugs all fall into the category of Janus Kinase (JAK) inhibitors.

Experimental Treatments

Researchers are working on developing new treatments for myelofibrosis. While many of these require further study to make sure they’re safe, doctors have started using two new treatments in certain cases:

  • Stem cell transplant: Stem cell transplants have the potential to cure myelofibrosis and restore bone marrow function. However, the procedure can cause life threatening complications, so it’s usually only done when nothing else works.
  • Interferon-alpha: Interferon-alfa has delayed the formation of scar tissue in the bone marrow of people who receive treatment early on, but more research is needed to determine its long-term safety.


Complications of Myelofibrosis

Over time, myelofibrosis can lead to several complications. Other than AML, they include the following:

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.

This can put too much pressure on smaller veins in your stomach and esophagus, which could lead to excessive bleeding or a ruptured vein.

Tumors

Blood cells can form in clumps outside the bone marrow, causing tumors to grow in other areas of your body.

Depending on where these tumors are located, they can cause a variety of different problems, including seizures, bleeding in the gastric tract, or compression of the spinal cord.

Life Expectancy of Myelofibrosis

It’s difficult to predict the outlook for myelofibrosis, especially in cases of secondary myelofibrosis, as it depends on the root cause. In addition, there’s no staging system for myelofibrosis.

People who are low risk tend to have a median survival of 15 years or longer. Certain factors can lower this time frame, including:

  • Having certain chromosomal abnormalities
  • Being older than 65
  • Having too few red blood cells, too many white blood cells, or certain immature white blood cells
  • Needing many blood transfusions 
  • Having generalized symptoms such as fever, weight loss, or fatigue

Roughly 10-20% of myelofibrosis cases progress to AML, a difficult-to-treat form of cancer with a low survival rate.

Summary

While myelofibrosis often doesn’t cause symptoms in its early stages, it can eventually lead to serious complications, including more aggressive types of cancer.

Work with your doctor to determine the best course of treatment for you and how you can manage your symptoms.

Living with MF can be stressful, so you may find it helpful to seek out support from an organization such as the Leukemia and Lymphoma Society or the Myeloproliferative Neoplasm Research Foundation. Both organizations can help you find local support groups, online communities, and even financial resources for treatment.

Myelofibrosis Frequently Asked Questions

Is Myelofibrosis Curable?

There isn’t one single treatment that works the same for every person or every type of myelofibrosis. The only true cure involves a stem cell transplant, but this can also cause complications and needs more research on its safety.

How Painful is Myelofibrosis?

With MF, you may feel some discomfort or pain below your ribs on the left side because of the enlarged spleen. It may also cause bone pain, as well as joint pain in some cases. The degree of pain can vary between people.

Do Patients with Myelofibrosis lose weight?

Weight loss can be one of the symptoms of MF. This is usually related to a lower appetite and overall feeling of fullness caused by an enlarged spleen.

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