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Secondary ITP can also be caused by certain autoimmune disorders such as lupus, inherited immune disorders such as common variable immunodeficiency (CVID), autoimmune lymphoproliferative syndrome (ALPS), and Evans syndrome, in which antibody-mediated red cell destruction accompanies ITP. Secondary ITP can also be caused by persistent infections (such as HIV, hepatitis B or C, or CMV), in addition to the ulcer-causing stomach bacterium such as Helicobacter pylori (primarily in certain countries, such as Japan and Italy), and lymphoproliferative disorders (such as chronic lymphocytic leukemia) that impair the immune system. A few cases resembling ITP result from the use of certain drugs. Secondary ITP can also occur in children after vaccination for measles-mumps-rubella (MMR) although rare. Recent cases of ITP have also been reported infrequently after both COVID infection and vaccination to prevent it.
ITP occurs in people of all races and ethnicities. ITP can occur at any age from 3 months to over 100 years of age. About 40% of all patients diagnosed with ITP are children younger than 10 years of age. The incidence is highest between 2 to 4 years of age, and males and females are affected equally until adolescence. Between adolescence and the age of 60, ITP is more common in females. The highest prevalence is among males and females over the age of 60.
The incidence (how many people are diagnosed each year) of adult ITP in the USA is roughly estimated to be 3.3 per 100,000/year. The prevalence (how many have ITP at any time) is 9.5 per 100,000. In a study that analyzed data from the Maryland Health Care Commission, the prevalence of ITP was estimated to be 9.5 per 100,000 children ages 1-5, 7.3 per 100,000 in children ages 6-10, and 4.1 per 100,000 in children of ages 11-14. Since children with ITP usually recover, the number of children who have ITP at any one time is almost equal to those diagnosed annually. Worldwide, it is estimated that there are well over 200,000 people affected by ITP.
In 80% of children who present with ITP, the disorder is self-limiting and resolves with or without treatment (i.e., spontaneously) within 12 months, usually sooner. In contrast, the proportion of adults with ITP who have a life-long chronic condition is much higher, approximately 50-70%, although firm data is lacking. One study suggested that 60% of adults diagnosed with ITP will improve within 3 years. ITP that develops in adolescents most often follows the clinical course seen in adults.
Heparin-induced thrombocytopenia (HIT) is the most common drug-induced, antibody-mediated thrombocytopenic-thrombotic disorder. Heparin is often used to prevent blood clotting (anticoagulant). HIT generally occurs a week after first exposure to the drug and sooner with subsequent exposures. The antibodies in HIT bind to heparin in combination with a protein released from platelets called PF4. HIT antibodies activate platelets and other cell types, predisposing to formation of blood clots (thrombosis) in veins or arteries. This is a disorder recognized in hospitalized patients but not in outpatients taking low doses of heparin subcutaneously to prevent clot formation. HIT is rare in children.
In most children and some adults, therapy may not be necessary at the time they first see their physician and the disorder may resolve spontaneously. The decision to initiate treatment depends on the severity of bleeding, the severity of the thrombocytopenia (especially in adults) the age of the patient (increased risk of bleeding in the elderly), coincidental disorders that might predispose to bleeding (tendency to fall, concurrent anti-platelet drugs or anticoagulants), lifestyle (such as young and athletic or jobs such as construction) and risks, side effects and costs and time away from work required for each intervention. In addition, after careful and comprehensive investigation, treatment may also be indicated to improve quality of life. These factors contribute to deciding if treatment is indicated and which treatment to use.
The orphan drug anti-D (WinRho SDF, Rhophylac), a specific form of gamma globulin, was approved by the U.S. Food and Drug Administration (FDA) to treat ITP in individuals who are red blood cell RhD antigen positive, do not already have antibodies on their red cells and have not undergone splenectomy. The drug can be used repeatedly, including in children who have newly diagnosed, persistent or chronic ITP. However, concerns have been raised because of a small fraction of individuals who have had severe side effects from brisk red cell destruction and its consequences soon after infusion.
The criteria for determining whether second line therapy is needed are the same as those involving initiation of treatment plus patients with suboptimal and/or short-lived responses to first line approaches. As mentioned, corticosteroids should be used for the shortest duration possible (ideally less than 6 weeks) to achieve these objectives and to provide a bridge to less toxic alternatives if treatment continues to be required. Many adults and some children will need long-term management because their platelet count fell once the dose of corticosteroids is tapered and IVIG is stopped.
A third option,Tavalisse (fostamatinib disodium hexahydrate) was approved in 2018 by the FDA for the treatment of thrombocytopenia in adults with ITP who have had insufficient response to a previous treatment; because of concerns about adverse effects on the growth plate, it should not be given to children. Approximately 20% of patients who are refractory to other forms of management responded based on pre-specified criteria, but, importantly, almost 40% did so using less stringent but clinically meaningful endpoints and most did so within several weeks of initial administration. The drug has several side effects (hypertension, diarrhea, headache and abnormal liver tests). One advantage is that it has the lowest reported risk of thrombosis of any licensed treatment of ITP and has anti-inflammatory effects as well, although additional clinical data is needed to confirm these findings.
Rodeghiero F, Stasi R, Gernsheimer T, et al. Standardization of terminology, definitions and outcome criteria in immune thrombocytopenic purpura of adults and children: report from an international working group. Blood. 2009;113(11):2386-2393.
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Approaching his teenage years, Donnie is a handsome young boy. He likes to be helpful and can be talkative. He likes to talk about things he has done before. He is interested in playing soccer. He enjoys swimming, playing with walkie talkies and hanging out with children in the neighborhood. In school, he enjoys working on the computer and reading. He does well when he can work one-on-one with adults or be the leader of a small group.
Her favorite foods are pizza and ice cream. She is looking for a family with a mother and father. She feels that she does better with other children who are older than her rather than younger children. She wishes to maintain connections with family members.
She has difficulty relating with children her same age and relates more to children younger than her. She enjoys watching the Disney channel and playing outside. She does well with incentive-based rewards
Ethan is a calm and content young boy. He enjoys playing with toys that light up and make noises. He also enjoys laying in his floor saucer and watching TV. He is a lovable child and gets along well with other children. Ethan will require a family who is familiar with the medical field or is open to learning about his care and condition.
Angelina is a beautiful, petite young lady with the most adorable freckles. Angelina is outgoing, friendly and often seeks to please. She enjoys drawing, coloring, puzzles, and all types of crafts which can serve as a calming and redirecting tool when she becomes upset. Angelina also enjoys listening to music. She is articulate and good at advocating for her needs. Angelina is in need of a family with extensive experience with children with challenging behaviors. She requires one on one supervision at all times.
Research suggests that allergy may be related to persistent glue ear in some children. You can ask your GP about testing your child for allergies or referral to an NHS allergy clinic if you think this might be the cause. Allergies to fur, pollen, dust mites or some foods may cause the eustachian tube to swell and this can prevent fluid from draining from the middle ear. For more information on allergy, you may like to contact the charity Allergy UK.
Our comic for children under 10, Harvey Gets Grommets, explains why Harvey gets grommets, what happens when he visits the doctor, and what happens at the hospital when the grommets are put in.
Hearing aids are devices designed to make sounds louder. They are used on a temporary basis for glue ear while waiting either for the glue ear to resolve or for grommet surgery. For children who have repeated problems with glue ear or are unable to have grommet surgery, hearing aids can be very helpful.
Osteopathy is a system of diagnosing and treating conditions, concentrating on the body's structure. This treatment can help to realign muscle tissue, bones and joints, allowing the body to function effectively and in doing so, may relieve pain and ailments.For information about osteopathy and to find a registered osteopath who specialises in treating children in your area, contact the General Osteopathic Council. The General Osteopathic Council registers qualified osteopaths and sets standards of osteopathic practice and conduct. Their aims are to protect patients, to develop the osteopathic profession and promote an understanding of osteopathic care.Phone: 020 7357 6655 (voice)The line is open between 9am and 5pm, Monday to FridayWebsite: www.osteopathy.org.uk 2ff7e9595c