KidsMD Health Topics

White Blood Cell Disorders

  • White blood cells are the parts of blood that help the body fight infection. Also called leukocytes, they are made up of five different subtypes: basophils, eosinophils, lymphocytes, monocytes and neutrophils.

    A number of disorders can impact these important cells and typically occur when:

    • White blood cell count is low
    • White blood cell count is high
    • White blood cells are not functioning properly

    How Dana-Farber/Boston Children's approaches white blood cell disorders

    Patients with White Blood Cell Disorders are treated through the Blood Disorders Center at Dana-Farber/Boston Children's Cancer and Blood Disorders Center, an integrated pediatric hematology and oncology partnership between Dana-Farber Cancer Institute and Boston Children's Hospital.

  • What are white blood cells?

    Whereas red blood cells carry oxygen, white blood cells, also called “leukocytes” help fight infections. Each type of leukocyte has specific roles in protecting our bodies. Many disorders affect white blood cells - causing their counts to be too low, too high, or causing malfunctions of the cells. Leukocytes include neutrophils (which fight bacteria and fungus infections), lymphocytes (which fight viruses, produce antibodies, and regulate the immune system), monocytes ("professional" germ eating cells), eosinophils, (which help fight parasitic infections and are related to allergic responses), and basophils.

    What causes benign white blood cell disorders?

    Benign white blood cell disorders occur when:

    • white blood cell count is low
    • white blood cell count is high
    • white blood cells are not functioning properly

    What are some examples of benign white blood cell disorders?

    Examples of disorders occurring when white blood cell count is low:

    • Neutropenia (low number of neutrophils in the blood)
    • Shwachman-Diamond syndrome
    • Kostmann syndrome (inherited disorder in which the blood lacks neutrophils)

    Examples of disorders occurring when white blood cells malfunction:

    • Chronic granulomatous disease (inherited disorder in which immune system cells called phagocytes do not function properly)
    • Leukocyte adhesion deficiency (rare inherited disorder in which the white blood cells are unable to produce a protein called CD18that helps them travel to the site of an infection)
    • Myeloperoxidase deficiency (disorder in which the enzymes that help the immune system fight bacteria are deficient)
    • Chediak Higashi syndrome (rare inherited disorder affecting the immune system)

    Are benign white blood cell disorders common?

    White blood cell disorders of lymphocytes and neutrophils are the most common. Disorders that involve monocytes and eosinophils are less common and disorders involving basophils are rare.

    What are the symptoms of white blood cell disorders?

    Symptoms depend on the type of white blood cell disorder a child has. Common symptoms include:

    • Frequent infections
    • Skeletal abnormalities (growth plate changes, short ribs, curvature of the spine)
    • Skin abscesses
    • Severe periodontal disease
    • Delayed wound healing 
    • Chronic, often greasy diarrhea
    • Invasive fungal infections
  • Depending on the type of white blood cell disorder a child has, the doctor may order:

    • Blood work to evaluate white blood cells
    • Stool sample
    • Bone marrow testing

    Specific lab tests for leukocyte function. After all tests are completed, hematologists will be able to outline the best treatment options.

  • Treatment largely depends on the type of white blood cell that is present and may include:

    • Oral or intravenous antibiotics to fight infection
    • Treatments to suppress autoimmune causes of low neutrophils
    • Colony-stimulating factor to stimulate bone marrow production of white blood cell
    • Blood product replacement for children at high risk of anemia or bleeding
    • Bone marrow or stem cell transplant

    Children with white blood cell disorders are treated through the Blood Disorders Center at Dana-Farber/Boston Children's Cancer and Blood Disorders Center, an integrated pediatric hematology and oncology partnership between Dana-Farber Cancer Institute and Boston Children's Hospital.

  • Research

    Thanks to ongoing research, treatment for patients with low white blood counts has markedly improved over the past 20 years. Genetic testing for Shwachman Diamond syndrome and other forms of severe congenital neutropenias has allowed better prognostication. Treaments can prolong life now for patients with even the most severe of the neutropenias.

    Clinical Trials

    For many children with rare or hard-to-treat conditions, clinical trials provide new options.

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