The autoimmune thrombocytopenia (or thrombocytopenia) (Itp) a rare coagulation disease. It has an estimated incidence of 10-20 cases per 100,000 people. characterized by a drastic reduction of platelets in the blood (due to their destruction by the spleen), without other associated pathologies. The severity of the symptoms varies from patient to patient: it can range from mucocutaneous bleeding to severe gastrointestinal or cerebral hemorrhages, which are rarer but potentially fatal. In one third of cases the disease develops asymptomatically.
Primary and secondary forms
Autoimmune thrombocytopenia is distinguished into two forms: primary (about 80% of the total) and secondary (20%). Both detect the presence of anti-platelet autoantibodies, but the causes that trigger this process are not known. In the secondary forms the disease arises as a consequence of other pathologies (for example Lupus erymatosus) or due to the intake of particular drugs. Mortality is generally low, about 1-2%, but the disease can severely affect patients’ quality of life.
Recognize the symptoms
Between symptoms more recognizable – we read on the website of the Rare Diseases Observatory – there is the appearance of petechiae and bruising in the skin (especially in the lower limbs), together with bleeding from the nose (epistaxis) or from the gums. Bleeding bubbles in correspondence of the mucous membranes, bleeding of the gastrointestinal and genitourinary tract (presence of blood in the urine) and, in women, more abundant bleeding during menstruation are also frequent. In some cases there may be secondary iron deficiency anemia, accompanied by weakness or asthenia.
How the diagnosis is made
As for the diagnosis, the reference laboratory examination of the blood count: in addition to providing the number of platelets, also indicates the mean platelet volume, which in many cases may be increased. The substantially clinical diagnosis and, together with the blood count, involves the examination of the patient, the collection of anamnestic information and the evaluation of a peripheral blood smear, to exclude other possible causes of thrombocytopenia, especially those of an infectious type or from exposure to drugs or toxic substances.
The treatments, up to the splenectomy
The main goal of treatment reduce the risk of bleeding, keeping the number of platelets at levels that are not dangerous to health. First-line therapy is essentially based on the administration of corticosteroids (hydrocortisone or prednisone). Pediatric patients with severe bleeding, or those with platelet counts below 10-20 thousand units per microliter, together with corticosteroids as possible administer intravenous immunoglobulins. In recent years, thrombopoietin receptor agonists (Tpo-mimetics) and rituximab, a monoclonal antibody that has an immunosuppressive action, have been used as second-line therapy. Ultimately, at least 12 months after diagnosis and only in severe cases, it is possible to resort to splenectomia (removal of the spleen), which offers a solid chance of recovery in two thirds of cases.
Camilla Canepa, the 18-year-old who died after AstraZeneca, suffered from thrombocytopenia: insights
June 11, 2021 (change June 12, 2021 | 07:30)
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