Idiopathic thrombocytopenic purpura in adults

Idiopathic (immune) thrombocytopenic purpura is a hemorrhagic disease caused by thrombocytopenia, not associated with a systemic disease. It usually has a chronic course in adults, but is often acute and transient in children. Spleen size is normal. For the diagnosis it is necessary to exclude the presence of other diseases by means of selective tests. Treatment includes the appointment of glucocorticoids, splenectomy and with life-threatening bleeding transfusion of platelets and intravenous immunoglobulin.

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Causes of the idiopathic thrombocytopenic purpura

Idiopathic thrombocytopenic purpura (ITP) is usually the result of the appearance of antibodies directed against structural platelet antigens (autoantibodies). In children, idiopathic thrombocytopenic purpura may be due to the addition of viral antigens to megakaryocytes and platelets.

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Symptoms of the idiopathic thrombocytopenic purpura

Symptomatic manifestations of petechiae and bleeding of mucous membranes. The spleen retains its normal size until it increases due to a viral infection. Idiopathic thrombocytopenic purpura is suspected in patients with unexplained thrombocytopenia. Parameters of peripheral blood within the limits of the norm, except for the decrease in the number of platelets. A bone marrow examination is performed if, in addition to thrombocytopenia, abnormalities are detected in the smear of peripheral blood or changes in the levels of other cellular elements. In the study of bone marrow there are no abnormalities or there is an increase in the number of megakaryocytes on the background of a normal bone marrow. Since there are no specific diagnostic criteria, for the diagnosis it is necessary to exclude other thrombocytopenic diseases according to clinical and laboratory data. Due to the fact that HIV-associated thrombocytopenia can be indistinguishable from idiopathic thrombocytopenic purpura, HIV testing is performed in patients with risk factors for HIV infection.

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Treatment of the idiopathic thrombocytopenic purpura

The starting therapy is the intake of glucocorticoids inside (for example, prednisolone 1 mg / kg per day). In patients who responded to therapy, the platelet count rises to normal within 2 to 6 weeks. Then the dose of glucocorticoids decreases. However, in most patients there is no adequate response or there is a relapse after the onset of gradual elimination of glucocorticoids. Splenectomy allows you to achieve remission in about 2/3 of these patients. Since further treatment may be ineffective in patients refractory to glucocorticoid therapy and splenectomy, and the natural course of idiopathic thrombocytopenic purpura is often benign, additional therapy is not indicated, except for a decrease in platelet counts below 10,000 / μL or the occurrence of active bleeding; in this case, more intensive immunosuppressive therapy (cyclophosphamide, azathioprine, rituximab) is prescribed.

In children, as a rule, only symptomatic treatment is used, as most of them usually have a spontaneous recovery of thrombocytopenia within a few days or weeks. Even after several months and years of thrombocytopenia, children can acquire spontaneous remission. If there is bleeding in the mucous, glucocorticoids or intravenous immunoglobulin are prescribed. Data on the use of glucocorticoids or intravenous immunoglobulin for initial treatment are inconsistent, as they, by raising the level of platelets, may not lead to an improvement in the clinical outcome. Splenectomy is rarely used in children. However, with severe thrombocytopenia and the presence of symptoms for more than 6 months, splenectomy can be effective.

In children and adults with life-threatening bleeding, an intravenous immunoglobulin of 1 g / (kg x day) for 1 or 2 days is prescribed for rapid blockade of phagocytosis. Usually the number of platelets rises on the second or fourth day, but only for 2-4 weeks. High doses of methylprednisolone (1 g / day intravenously for 3 days) is a cheaper and easier to apply treatment method than the use of intravenous immunoglobulin, but this method is also effective. In patients with idiopathic thrombocytopenic purpura and the presence of life-threatening bleeding, thrombocyte mass is used. For prophylactic purposes, however, platelet weights are not used

The intake of glucocorticoids inside or intravenous immunoglobulin is prescribed, if necessary, by temporarily raising the platelet level during extraction of teeth, labor, surgical interventions or other invasive procedures.