Immune thrombocytopenia (ITP) is an autoimmune disorder defined as a platelet count less than 100 × 109/L with the exclusion of other causes of thrombocytopenia. The primary pathology is thought to be autoimmune-mediated destruction of platelets and/or inhibition of platelet production by megakaryocytes by autoantibodies or T cells, although the lack of diagnostic test leads to many misdiagnoses. ITP is a heterogeneous disease that can occur at any age and affects both sexes, with peaks under 10 (equal sex distribution), women of childbearing age (female predominance) and then increases in older age (male predominance). ITP can also be secondary to other causes such as autoimmune diseases, hematological malignancies and immunodeficiency states (where it is a poor prognostic factor). Patients are at risk of bleeding and thrombosis and have impaired quality of life with fatigue and cognitive impairment. There are many available treatments and others in clinical trial. While most agents aim to increase platelet count to prevent bleeding, there are major unmet needs to reduce fatigue, improve quality of life, to be able to individualize care, and to have a non-toxic curative treatment.

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Immune Thrombocytopenia (ITP)

  • James Bussel,
  • Nichola Cooper

摘要

Immune thrombocytopenia (ITP) is an autoimmune disorder defined as a platelet count less than 100 × 109/L with the exclusion of other causes of thrombocytopenia. The primary pathology is thought to be autoimmune-mediated destruction of platelets and/or inhibition of platelet production by megakaryocytes by autoantibodies or T cells, although the lack of diagnostic test leads to many misdiagnoses. ITP is a heterogeneous disease that can occur at any age and affects both sexes, with peaks under 10 (equal sex distribution), women of childbearing age (female predominance) and then increases in older age (male predominance). ITP can also be secondary to other causes such as autoimmune diseases, hematological malignancies and immunodeficiency states (where it is a poor prognostic factor). Patients are at risk of bleeding and thrombosis and have impaired quality of life with fatigue and cognitive impairment. There are many available treatments and others in clinical trial. While most agents aim to increase platelet count to prevent bleeding, there are major unmet needs to reduce fatigue, improve quality of life, to be able to individualize care, and to have a non-toxic curative treatment.