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ITP Medical Malpractice - Idiopathic Thrombocytopenic Purpura
Idiopathic thrombocytopenic purpura or ITP is also known as primary immune thrombocytopenic purpura. It is an autoimmune disease caused by the deposition of IgG autoantibodies on platelets, which are then destroyed by immune cells. The bone marrow is normal and the number of platelets formed is normal. It is the destruction of platelets that is increased. There are two types of clinical syndromes which represent ITP: 1) there is an acute form that occurs in children; and 2) there is a chronic form found in adults. There is an obvious decrease in the number of platelets circulating in the bloodstream One has to rule out a toxic exposure or other disease before the diagnosis of idiopathic thrombocytopenic purpura is made. The condition may be made worse by having bone marrow failure.
Acute ITP in children usually follows some kind of infection and lasts only about two months. Chronic ITP lasts for at least six months and has no known cause behind getting it. In North America, the incidence of ITP is about 66 cases per million people per year. In children, the incidence of acute ITP is about 50 per million children per year. Chronic ITP that does not resolve occurs in 10 people per million per year. The most serious complication is hemorrhage, particularly an intracranial hemorrhage.
The mortality rate for kids with intracranial hemorrhages is about 1 percent, while the mortality rate for adults with intracranial hemorrhage is about 5 percent. Those over sixty who have chronic disease have a mortality rate of almost fifty percent over time. In kids, about 80 percent spontaneously resolve but spontaneous resolution in adults is not common. There are almost three times as many females involved as men, particularly in adults, in whom 72 percent are female.
The peak age for acute ITP in kids is about 2-4 years old. The peak age for adults with chronic disease is about 20-50 years of age. Common signs and symptoms include an abrupt onset (in kids) or a gradual onset (in adults). There are bruises, called purpura, heavy menstrual bleeding, bloody noses, bleeding from the gingiva, and recent live viral immunization in kids or recent viral infection in kids.
Physical findings on examination include petechiae, particularly on dependent regions of the body, hemorrhagic bullae (blisters) on mucus membranes, purpura (bruised areas), GI bleeding evidence, gingival hemorrhaging, heavy menstrual bleeding, retinal hemorrhages, evidence of bleeding within the brain and possible neurological symptoms. The spleen is usually not able to be felt. The platelet count is often less than 20,000, which leads to spontaneous bleeding.
The cause of idiopathic thrombocytopenic purpura, as mentioned, is the formation of autoantibodies against platelet receptors so that the immune system destroys the platelets. A CBC can be performed which shows the low platelet count in the absence of low WBCs or RBCs. If there are giant platelets on the peripheral smear, this suggests congenital thrombocytopenia and not ITP. A bone marrow study is usually normal and coagulation studies are normal. The bleeding time is not a useful test in this condition.
If there is life-threatening bleeding, you need immediate critical care in an ICU. The treatment involves giving high doses of glucocorticoids and IV immunoglobulin to trick the immune system so it doesn't attack the platelets. Platelet transfusions might be necessary but this is a temporary measure as these platelets get chewed up, too. Platelets survive longer if they are transfused immediately after giving an IV injection of IgG. If medical therapy fails, doctors can remove the spleen and this may improve the platelet count. It is indicated as an emergency if bleeding becomes life threatening. Remember that ITP is a diagnosis of exclusion so you need to make sure that the patient truly has ITP before going about treating the disease as ITP.LEGAL HELPLINE: ☎ 855 804 7125
The author of the substantive medical writing on this website is Dr. Christine Traxler MD whose biography can be read here