Types
By tradition, the term idiopathic thrombocytopenic purpura has been used when the cause is idiopathic, or unknown. The specific trigger for most cases remains unknown. [4] Whatever the trigger, the condition is now considered to be immune-mediated and the term Immune Thrombocytopenic Purpura is more usual. Either of these terms may be abbreviated as ITP. A consequence of the severe reduction in platelet count includes purple spots on the skin, or Purpura, gum bleeding, easy bruising, or hemorrhage. [2] If the symptoms resolve within 6 months, it is more specifically termed acute ITP. Acute ITP is commonly seen in children, especially after a viral illness (i.e. Chickenpox) or after starting certain drugs. If the attack lasts longer than 6 months, it is termed chronic ITP. Chronic ITP affects adults more than children and women more than men. [5]
Another form is thrombotic thrombocytopenic purpura.This may be abbreviated as TTP. [6] TTP can be immune mediated or due to an acquired genetic mutation. The non-immune subtype and can be termed congenital TTP. Immune TTP is most commonly seen in female adults, whereas congenital TTP is diagnosed early in childhood or during pregnancy. [3]
Diagnosis
Diagnosis of ITP includes a detail history and physical and focuses on ruling out other causes of thrombocytopenia (i.e. Leukemia, Lupus, aplastic anemia). Other than sudden onset of bleeding due to abnormally low platelet count, patients will appear and act normal. First line workup includes a Complete blood count with a peripheral smear. To rule out bone marrow disorders, a bone marrow biopsy can be performed. In ITP, this biopsy is typically normal outside of increased number of megakaryocytes. Because many viral infections can trigger ITP, it is routine to perform viral serologies to identify and treat the underlying trigger. Autoantibody tests can also be performed; however, this test is not routine and a positive or negative result alone cannot be used to diagnose ITP. [7] [8]
Treatment
Most children with ITP recover spontaneously and do not require pharmacologic intervention. Clinical observation is preferred when platelet counts are greater than 20,000-30,000/μL in acute ITP. First-line interventions when treatment is required includes corticosteroids, like Prednisone. The use of prednisone is, however, limited given the extensive side effects with prolonged use at high doses. Other treatments include intravenous immunoglobulin and anti-D immunoglobulin. [7]
If ITP persists despite first-line treatments and becomes chronic ITP, more intensive medications can be pursued. These interventions include Rituximab and thrombopoietin receptor agonists. Splenectomy can be considered if all other treatments fail. [7]
Treatment of TTP may involve plasma exchange and a chemotherapy medication Vincristine. [9]
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