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Platelet Function Disorders

  • Platelets are cells found in the blood that help control bleeding. When a child has a platelet disorder, a person’s blood may not be able to clot normally and they may develop a bleeding disorder.

    Most platelet disorders are acquired, meaning they appear spontaneously or as a result of an external factor; children usually aren’t born with the disorder.

    How Dana-Farber/Boston Children’s Cancer and Blood Disorders Center approaches platelet function disorders

    Children with all types of abnormal platelet function are treated through the Platelet Function Disorders Program.

    • Our program includes pediatric hematologists who have considerable experience diagnosing and treating all types of childhood platelet disorders.

    • We provide comprehensive care to children and adolescents, including access to the most recent treatments and to unique clinical trials.

    • Our team includes world renowned researchers who are seeking to better understand and treat pediatric platelet disorders.

  • What is a platelet function disorder?

    Platelets are cells that circulate in the blood stream and help the blood clot. When a child has a platelet disorder, a person’s blood may not be able to clot normally and they may develop a bleeding disorder.

    Types of platelet function disorders

    Acute thrombocytopenic purpura:

    • most common form of idiopathic thrombocytopenia purpura
    • usually occurs in children, ages 2 to 6
    • symptoms may follow a viral infection, such as chickenpox
    • very sudden onset, with symptoms disappearing in less than six months and often within a few weeks
    • usually does not recur
    • risk of serious or life-threatening bleeding is very low

    Chronic thrombocytopenic purpura:

    • more common in adults but can occur at any age
    • affects girls two to three times more often than boys
    • symptoms last a minimum of six months and can persist for many years
    • may become a lifelong problem
    • regular follow-up care with a blood specialist (hematologist) is recommended

    What causes platelet function disorders?

    In most cases, the cause of idiopathic thrombocytopenia purpura (ITP) is not known.

    • Often, a child may have had a viral infection a few weeks before developing symptoms of ITP.
    • Viral infections may trigger ITP, as the antibodies the body develops to fight them could be abnormal.
    • Researchers have also explored the possibility that certain medications cause ITP but no direct link has been made.
    • ITP is not contagious, meaning your child cannot catch it from playing with another child who has ITP.

    Are platelet function disorders common?

    Platelet function disorders are not extremely common. In most cases they are acquired, or caused by an external factor like another illness or medication. In rare cases they are inherited.

    What are the symptoms of platelet function disorders?

    The most common symptoms of platelet disorders are:

    • purpura (purple color of the skin after blood has leaked under it forming a bruise, often from no known trauma)
    • petechia (tiny red dots under the skin that are a result of very small bleeds into the skin)
    • nosebleeds
    • bleeding in the mouth and/or in and around the gums
    • blood in vomit, urine or stool
    • bleeding in the head (most dangerous symptom that can be life-threatening, usually prompted by a head trauma)
  • How does a doctor know if a child has a platelet function disorder?

    Platelet function disorders can be identified through the following tests:

    • bleeding time, which measures how long it takes for a cut to stop bleeding
    • complete blood count
    • additional blood and urine tests (to measure clotting times and detect possible infections)
    • possible bone marrow testing to identify any abnormal cells
    • careful review of your child's medications

    A full bone marrow exam in which samples of the fluid (aspiration) and solid (biopsy) portions of bone marrow are withdrawn with a needle under local anesthesia may also be performed.

  • Treatment for platelet function disorders

    There are a number of treatments that can help increase platelets levels in children with idiopathic thrombocytopenic purpura (ITP), but there is no cure. The majority of children with ITP get better spontaneously in a few days or weeks, with or without treatment. When treatment is necessary, the most common forms are:

    Steroids:

    • Temporarily reduce production of abnormal antibodies and increase platelet count by slowing the rate at which they are destroyed by the spleen
    • May be taken orally
    • Side effects may include: irritability, stomach irritation, weight gain, hypertension, sugar in the urine or acne

    Intravenous gamma globulin:

    • Human blood product containing many antibodies that help slow the rate at which abnormal platelets are destroyed by the spleen
    • Given through a needle inserted into a vein over 3 to 6 hours
    • Temporary side effects include: fever, chills, headache, muscle and joint pain, hives, rash or allergic reactions

    Intravenous Rh immune globulin:

    • Human plasma product, manufactured with special processes to make it safe
    • Temporarily stops the spleen from destroying platelets
    • Child must be blood type Rh positive and have a spleen for this medication to be effective
    • Given intravenously
    • Temporary side effects include: mild anemia, fever, chills, headache, blood pressure changes or allergic reactions. Rarely, severe anemia from breakdown of blood cells can occur.

    Other treatments for ITP may include:

    • medication changes
    • infection treatment
    • surgery to remove spleen (considered more often in older children with chronic ITP)
    • hormone therapy (for teenage girls to stop their menstrual cycle if excessive bleeding occurs)

    Caring for a child with a platelet function disorder

    Preventing serious bleeding until a child's body resolves the idiopathic thrombocytopenic purpura is the most important aspect of care. Parents should consider:

    • padding child's crib
    • having child wear helmets
    • providing protective clothing for child
    • restricting certain contact sports and rough play
    • avoiding medications that may interfere with platelet function and may cause bleeding, including aspirin, ibuprofen, Motrin, Advil, naproxen, naprosyn, Aleve, and more
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