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Chronic eosinophilic leukemia (CEL): What to know

Chronic eosinophilic leukemia (CEL) is a type of blood cancer that causes in an overabundance of eosinophils in the body. Eosinophils are a type of white blood cell that helps the body fight infection, but having too many of them can be hazardous.

CEL is uncommon, but researchers do not know how common it is in the United States.

This page examines the symptoms and causes of CEL, as well as treatment options and outlook.

What is it?

blood test

CEL is an uncommon form of myeloproliferative neoplasm, which is a type of blood cancer caused by myeloid stem cells in bone marrow producing an abnormally large number of red and white blood cells and platelets.

CEL causes the body to overproduce eosinophils, a type of white blood cell. Eosinophils secrete substances to protect the body from infections or allergic reactions.

Eosinophils are high in the bone marrow, blood, and other tissues of people with CEL. This can cause to issues including organ damage.

Symptoms

People may not have symptoms in the early stages of the disease. A regular blood test can also detect CEL in people who have no symptoms.

Other people may get severe symptoms as a result of high eosinophil levels.

Among the signs of CEL are:

  • unexplained weight loss
  • shortness of breath
  • swollen lymph nodes
  • anemia
  • fatigue
  • fever
  • cough
  • swelling under the skin around the eyes, lips, throat, hands, or feet
  • muscle aches or pains
  • itchiness
  • diarrhea
  • night sweats

CEL is most commonly diagnosed in people between the ages of 20 and 50, but it can also affect youngsters and the elderly. CEL is more frequent in men than in women.

Causes

The cause of CEL is unknown. There is no link, for example, between CEL and a mutation in genes or chromosomes, according to researchers. CEL may be caused by environmental factors such as smoking or exposure to radiation or certain chemicals in rare situations.

Diagnosis

In most circumstances, doctors try to rule out all other potential causes of the patient’s symptoms. If they then rule out CEL, this is referred as as a diagnosis of exclusion.

A doctor evaluates any symptoms, performs a physical examination, and may subsequently run a variety of tests, including:

  • a bone marrow biopsy and aspiration, which involves removing a small sample of bone marrow for testing
  • blood chemistry tests, which show how well organs are functioning
  • a complete blood count, which gives the amount and quality of white and red blood cells and platelets

Outlook

CEL is typically slow to progress and can persist for many years.

However, a person’s condition can quickly deteriorate if CEL progresses to acute myelogenous leukemia.

A 2020 study of CEL patients in the United States discovered a median survival period of 2 years following diagnosis. CEL progressed to acute myelogenous leukemia in many of these patients.

Researchers behind a different 2020 study discovered that the outlook for CEL varies, and they called for greater research on the subject.

Other studies indicated that people who got stem cell transplantation as a treatment had survival rates ranging from 8 months to 5 years. Despite the fact that this strategy can be effective, clinicians do not typically accept it as a credible treatment for CEL.

Treatment

The appropriate approach for each person with CEL may differ.

Corticosteroids and interferon alfa (Intron A, Wellferon), for example, may be used to treat the disease. Doctors may also administer chemotherapeutic drugs such as:

  • hydroxyurea (Hydrea), possibly in combination with steroids
  • cyclophosphamide (Cytoxan, Procytox)
  • vincristine (Oncovin)

A doctor may advise you to use targeted chemotherapy medications, which target certain genes or parts of leukemia cells.

In addition, treatment with the medication imatinib (Glivec) may result in long-term remission in some people with CEL. This medication prevents the formation of excessive eosinophils.

People with aggressive CEL may benefit from stem cell transplants if stem cells from a donor with similar genes are available.

Follow-up treatment

A doctor may offer further approaches to relieve CEL symptoms, such as:

  • Leukapheresis: An electrophoresis machine is used to separate out extra white blood cells from the blood, lowering the number of eosinophils.
  • Blood-thinning medication: CEL can cause blood clots, and these medications can help avoid them.
  • Splenectomy: High eosinophil levels can cause the spleen to expand, resulting in significant abdominal pain. In this instance, a doctor may advise removing the spleen by surgery.
  • Cardiac surgery: If a person has heart disease, surgery to remove scarring of the heart muscle or replace heart valves may assist improve heart function and extend life.

Hypereosinophilic syndrome vs. CEL

Hypereosinophilic syndrome (HES) is a group of blood diseases characterized by elevated amounts of eosinophils. CEL is a type of HES.

An overabundance of eosinophils can cause problems in a variety of places of the body, most notably the:

In many situations, the cause of HES is unknown. It may be caused by an increase in the synthesis of a specific protein in some white blood cells. A person has lymphocytic HES in this case.

An hereditary genetic mutation can also cause the illness. A person in this situation has familial HES.

The high quantity of eosinophils in someone with CEL is caused by alterations in the bone marrow. CEL is a form of HES that is myeloproliferative.

HES symptoms may include:

  • problems with the nervous system, such as¬†vertigo¬†or tingling sensations
  • heart problems
  • anemia
  • deep vein thrombosis
  • skin rashes or swelling
  • breathing difficulties
  • stomach pain or upset
  • muscle and joint pain

Certain medications, such as imatinib mesylate (Glivec) and mepolizumab, may aid in the treatment of HES (Nucala).

Conclusion

CEL is a form of blood cancer that is extremely rare. It causes the body to overproduce eosinophils, a type of white blood cell.

Medication, such as chemotherapy, and other approaches, such as stem cell transplants or surgery, may be used in treatment.

Many people’s CEL develops slowly, and treatment may result in long-term remission.

Sources:

  • https://seer.cancer.gov/seertools/hemelymph/51f6cf58e3e27c3994bd53fc/
  • https://www.cancer.gov/types/myeloproliferative/hp/chronic-treatment-pdq
  • https://onlinelibrary.wiley.com/doi/full/10.1002/ajh.23664
  • https://www.medicalnewstoday.com/articles/what-is-chronic-eosinophilic-leukemia
  • https://rarediseases.info.nih.gov/diseases/2804/hypereosinophilic-syndrome
  • https://www.cancerresearchuk.org/about-cancer/cancer-in-general/treatment/cancer-drugs/drugs/imatinib
  • https://www.sciencedirect.com/science/article/pii/B9780323085939000760
  • https://onlinelibrary.wiley.com/doi/10.1002/ajh.25906

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