Autoimmune disease, thrombocytopenic purpura

For the first time, autoimmune thrombocytopenic purpura was described in 1735 by Verlhof, who observed the disease in a ten-year-old girl. Currently, this name combines autoimmune forms of thrombocytopenia, the cause of autoaggression in which it is not possible to establish. They manifest hemorrhagic diathesis with isolated platelet deficiency. Isolate the acute and chronic form of this disease.

Causes of development

Acute thrombocytopenic purpura is mainly affected by children aged 2 to 9 years. Thrombocytopenic purpura in children is characterized by a sudden onset and rapid recovery. The development of clinical manifestations is often promoted by a viral infection of the upper respiratory tract or digestive tract. In 10% of cases, the development of the disease is associated with varicella-zoster viruses, Epstein-Barr, prophylactic vaccinations, BCG immunotherapy. The destruction of platelets is due to the immune response of the body, and not the action of the virus.

Chronic forms of thrombocytopenia lasting more than 6 months are called idiopathic thrombocytopenic purpura. The cause of the disease is unknown. Thrombocytopenic purpura often begins during adolescence, during puberty. Women are more often ill.

Clinical manifestations appear gradually. Chronic-recurrent or protracted course of the disease is characteristic. Pathogenetic basis of the disease is increased and accelerated destruction of platelets by autoantibodies against them.

Clinical picture

On the skin of patients appear symptoms of hemorrhagic microcirculatory type in the form of small-point petechial rash, small subcutaneous hematomas, hemorrhagic rash on the mucous membrane of the oral cavity. Typical bleeding of the mucous membranes of the nose, gums. Changes on the skin are more often located on the front surface of the trunk, upper and lower extremities. Typically, the formation of large hemorrhages at the injection sites. Occasionally there may be hemorrhages in the sclera or retina of the eye, bleeding from the digestive canal. Women have hemorrhages in the ovaries. In severe forms of the disease, kidney bleeding can occur.

Thrombocytopenic purpura can lead to dangerous complications in the form of cerebral hemorrhage and subarachnoid hemorrhages. Characteristic is the positive cuff test of Konchalovsky-Rumpele-Leide: the formation of hemorrhages in the areas of squeezing the skin. Lymph nodes, liver and spleen are not enlarged.

Diagnostics

The main laboratory feature is the decrease in the number of platelets. Time of folding of blood is not broken. The heparin plasma time is increased. In many cases, the increase in platelet immunoglobulin G is determined. After significant bleeding, acute posthemorrhagic anemia is identified.

Treatment

If thrombocytopenic purpura is diagnosed, the treatment of patients begins with the appointment of corticosteroids. In the course of treatment, patients have a steady increase in platelets until complete remission is achieved. With insufficient effectiveness of corticosteroids, prescribe delagil and immunomodulators. If the conservative therapy is ineffective for 6 months, a splenectomy is recommended.

Another direction of therapy - the appointment of high doses of immunoglobulins for intravenous administration in the course of 5 days. These drugs inactivate antibodies, bind and remove viral, bacterial and other antigens.

In 80-90% of patients within 1-4 months, spontaneous recovery occurs. In some cases, thrombocytopenic purpura can last up to 12 months. The course of the disease is chronically recurrent: periods of bleeding alternate with periods of remission.