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Introduction
How ITP Occurs
Symptoms of ITP
Diagnosis
Treatment of Acute ITP
Treatment of Chronic ITP
Conclusion
 

Introduction
Immune thrombocytopenic purpura (ITP) is a childhood disorder of platelets, which are the blood cells in our bodies responsible for clotting. Though it can be frightening for parents, ITP in children is usually curable and rarely does it cause serious medical problems. The mortality rate for this disease is less than 1%. Most frequently, it resolves within six months whether or not the child receives treatment. However, if you suspect that your child has ITP, you should see your pediatrician. You can then, in consult with a pediatric hematologist, determine an appropriate treatment regimen.
 

How ITP Occurs

After a viral illness, children produce antibodies that protect them from future infections with that same virus (vaccinations against viral illnesses serve the same defensive role). To fight infections in the blood, antibodies work in conjunction with the spleen. The spleen is a large, infection-fighting gland that filters our blood of invading organisms and foreign proteins. There are scavenger cells in the spleen that recognize antibodies and devour anything attached to that antibody. In ITP, antibodies produced to fight infections also attach themselves to platelets in a process called autoimmunity. These platelets are then recognized as “bad” and are destroyed in the spleen. The bone marrow responds by producing more platelets, but it can hardly keep pace with the platelet destruction in children with ITP. Once the platelet count gets low enough, symptoms appear. Children may bruise easily and cuts may take longer to clot. Although children often get bruises along the bony surfaces of their arms, legs and back, the number of bruises and their abnormal location in ITP alert parents that something is wrong.
 

Symptoms of ITP

Two to three weeks after a viral infection, children with ITP typically get bruises all over their bodies, bleeding gums, and/or a bloody nose. This is often how the disorder is first spotted. Bleeding gums and/or a bloody nose, also known as wet purpura, are especially worrisome since some pediatric hematologists (blood specialists) believe that these children may be at an increased risk for bleeding inside the brain. Otherwise, the physical examination is entirely normal. Abnormal physical findings often alert your physician that an alternate diagnosis must be considered.
 

Diagnosis

The diagnosis of ITP is usually straightforward. A child presents with bleeding, a preceding viral illness, an essentially normal physical examination with the exception of bruises, and a low platelet count (thrombocytopenia). When a sample of blood is examined using a microscope, a less-than-normal number of large, “immature” platelets is seen. The other blood cell types are normal in appearance in ITP (if these other cells look abnormal, a different diagnosis is likely).
 

Treatment of Acute ITP

Acute ITP is defined as ITP lasting less than 6 months. My treatment philosophy for acute ITP is as follows:

• If your child has a platelet count above 10,000 to 15,000 (normal is in the 150,000-400,000 range) and only has bruises, I opt for close observation (the child can stay at home) and repeat blood counts.

• If your child has a platelet count of less than 10,000 or has bleeding from the gums or nose (“wet purpura”), I opt to treat the ITP with one of the therapies described below.

Unfortunately, IVIG is expensive and has several side effects. First, IVIG causes fever, chills and inflammation so that children must be given Tylenol, Benadryl (an antihistamine), and steroids (anti-inflammatory medication) before being given IVIG. Second, IVIG must be administered intravenously over 8 to 12 hours, usually in a hospital setting. Third, because it comes from human blood, there is a small risk of viral contamination (from HIV and hepatitis B and C most notably) and therefore, a risk of infection. Lastly, IVIG can cause swelling of the airway in people with a fairly uncommon genetic disorder known as Immunoglobulin A deficiency. Despite these drawbacks, IVIG is a useful treatment for ITP.

Anti-D (Winrho)
Anti-D is also composed of pooled human blood and contains antibodies against the D antigen present on red blood cells. This medication is given intravenously in an outpatient setting (no hospitalization required) over 1 to 2 minutes and does not require any medications to be given beforehand. Anti-D antibodies bind onto certain red blood cells, which are preferentially destroyed instead of the platelets. In the majority of cases the red blood cell destruction is minimal and only rarely are red blood cell transfusions needed. As with all pooled human blood products, the risk of viral contamination is present, and I do discuss this important issue with parents. Anti-D is probably as expensive as IVIG but since most children can be treated as outpatients, the overall medical costs are reduced and the child is spared the trauma of hospitalization.

Steroids
Steroids are anti-inflammatory medicines that have been used to treat ITP (and many other disorders) for decades. Although not completely understood, these drugs are felt to decrease the amount of anti-platelet antibodies produced by your child’s immune system and decrease the power of the scavenger cells within the spleen. Steroids have many side-effects. They can cause sugar and salt metabolism problems, weak bones, changes in body shape, and a weakened immune system. Moreover, they are quite bitter and your child may not like their taste. Nevertheless, they are inexpensive and useful in the treatment of ITP (especially for the treatment of chronic ITP).
 

Treatment of Chronic ITP

Chronic ITP is defined as ITP that lasts more than 6 months. I use the same treatment strategy for chronic as I do for acute ITP.

• If your child has a platelet count above 20,000 and has no symptoms, I believe that careful observation and platelet count-monitoring is safe after a discussion with the parents about what symptoms can be tolerated (bruises). As with acute ITP, I take bleeding gums and noses seriously and in such cases would want to examine your child immediately.

• For patients with platelet counts of 10,000 or less and/or symptoms I like to use the treatment method that worked during the acute ITP phase. Sometimes the treatments that worked in the past become ineffective, in which case I offer another form of therapy.

Surgical treatment
Surgical removal of the spleen (known as splenectomy) is usually reserved for emergency situations (bleeding into the brain in either acute or chronic ITP) or for children with chronic ITP that will not respond to other treatments. With the advent of minimally invasive surgical techniques, children have less pain, better cosmetic results and are in the hospital for shorter periods of time.

After splenectomy, children have an increased risk for developing certain infections (Haemophilus influenzae and Streptococcus pneumoniae) that may be life threatening, and these children need to receive a special vaccine against a bacteria that causes pneumonia (Pneumovax). In addition, roughly 10 to 15% of children fail to respond to splenectomy with normalization of their platelet counts. Clearly the decision to perform a splenectomy is a difficult one for parents and for me. However, after weighing the risks and benefits of splenectomy versus persistently low platelet counts, most parents and their children agree that splenectomy is the way to go. In fact, I have two children under my care that did not achieve a normal platelet count after splenectomy, but they are stable and have not required additional therapy.
 

Conclusion

ITP in children is usually a curable disease that rarely causes serious medical problems. The treatments are fairly well tolerated with few, if any, long-term side effects. Almost all pediatricians can recognize the signs and symptoms of ITP and hematologists are available to help plan treatment strategies and manage difficult cases.