Idiopathic thrombocytopenic purpura

The symptoms of ITP are first mentioned in the writings of Hippocrates . In 1735, P. Verlgof described the disease , observing cases of spontaneous recovery. In the XIX century, it was found that the cause of hemorrhagic syndrome in ITP is a decrease in the number of platelets in the blood. The immune genesis of ITP was established in the course of the experiment of William Harrington and James Hollingsworth in St. Louis in 1950. American doctor Harrington poured himself 500 ml of ITP patient's blood, after which he observed a decrease in the level of platelets in his own blood, which persisted for 5 days ^{[3 ]} . Bark's bone marrow biopsy showed normal megakaryocyte levels. In 1916, Polish physician Paul Katsnelson described a case of attaining ITP remission in a young woman after splenectomy ^[4] . From the middle of the 20th century, glucocorticosteroids began to be used in ITP therapy.

The prevalence of ITP varies from 1 to 13 per 100,000 people. Most of the patients are young and middle-aged women ^[5] . The disease is not inherited.

ITP is a chronic wave-like disease. It is characterized by thrombocytopenia , the frequent presence of giant platelets in the bloodstream, normal or increased number of functionally inactive megakaryocytes in the bone marrow . In about half of the cases, antiplatelet antibodies are detected in the blood, for example, against glycoprotein IIb-IIIa and Ib-IX . With a significant decrease in the level of platelets in the blood (usually less than 30x10 ⁹ / l), hemorrhagic syndrome of varying severity may be observed. The latter is usually represented by skin hemorrhages ( petechiae , purpura , ecchymosis ), hemorrhages in the mucous membranes, and (less often) nasal, gingival and uterine bleeding . Sometimes there is melena , hematuria , hemorrhagic strokes . Ophthalmological manifestations: hemorrhages under the skin of the eyelids and conjunctiva, sometimes in the fiber of the orbit with the formation of exophthalmos; deterioration of visual acuity due to the formation of hyphema (blood in the anterior chamber of the eye) and hemophthalmus (blood in the vitreous body).

The pathogenesis of the disease is not well understood. At the heart of ITP is an autoimmune reaction directed against its own platelets. There are no reasons for secondary autoimmune thrombocytopenia (5-10% of all immune thrombocytopenia), such as lymphoproliferative diseases , HIV infection , hepatitis C , APS , SLE, and Willebrand's disease . Antibodies to platelets in the bloodstream are not always determined. In children, ITP is often preceded by acute respiratory viral infections.

Adrift acute (lasts less than 6 months) and chronic forms of ITP. Acute exacerbation (crisis) and remission are distinguished by the period of the disease.

ITP treatment usually begins with a decrease in platelet levels of less than 30x10 ⁹ / l, or when a clinically significant hemorrhagic syndrome appears, regardless of the platelet count. The standard first-line therapy option is systemic glucocorticosteroids (GCS) ^[6] . In a 2015 randomized trial, a four-day intravenous infusion of 40 mg of dexamethasone was more effective and less toxic than a long course of prednisone (1 mg / kg orally for 30 days) ^[7] . In cases of insufficient efficacy of GCS, splenectomy , rituximab , fostamatinib are used , as well as specific thrombopoietin receptor agonists ( romiplostim , eltrombopag ). In rare cases, other immunosuppressants are used ( azathioprine , cyclosporine , etc.). If necessary, a rapid increase in the level of platelets (for example, before surgery or in the case of life-threatening bleeding), preparations of intravenous immunoglobulin and transfusion of the platelet concentrate are used .

• Heparin-induced thrombocytopenia
• Gestational thrombocytopenia
• Thrombotic thrombocytopenic purpura (TTP)
• Romiplostim
• Eltrombopag
• Fostamatinib