Immune Thrombocytopenia (ITP)

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Basics

Description

  • Immune thrombocytopenia (ITP) is a condition characterized by the immunologic destruction of normal platelets and/or impaired thrombopoiesis in response to an unknown stimulus.
  • It is defined as a platelet count <100 × 109/L, once other causes of thrombocytopenia have been ruled out (1).
  • ITP nomenclature:
    • Newly diagnosed (<3 months), persistent (3 to 12 months), and chronic (>12 months)
    • Primary when it presents in isolation; secondary when associated with other disorders
  • ITP is a relatively common disease of childhood that typically follows a viral infection. Onset is within 1 week, and spontaneous resolution occurs within 2 months in >80% of patients.
  • In adults, ITP is usually a chronic disease and spontaneous remission is rare.
  • Synonym(s): idiopathic thrombocytopenic purpura; immune thrombocytopenic purpura; and Werlhof disease

Epidemiology

  • Peak age
    • Pediatric ITP: 2 to 4 years
    • Chronic ITP: >50 years with incidence 2 times higher in persons 60 years than those <60 years of age
  • Predominant gender
    • Pediatric ITP: male = female
    • Chronic ITP: female > male (1.2 to 1.7:1)

Incidence

  • Pediatric acute ITP: 1.9 to 6.4/100,000 children per year
  • Adult ITP: 1.6/100,000 per year (1)

Prevalence
Limited data; in one population (in Oklahoma):

  • Overall prevalence of 11.2/100,000 persons
  • In children (<16 years): 8.1/100,000 with average age of 6 years
  • In adults (>16 years): 12.1/100,000 persons with average age of 55 years

Etiology and Pathophysiology

  • Accelerated platelet uptake and destruction by reticuloendothelial phagocytes results from action of IgG autoantibodies against platelet membrane glycoproteins IIb/IIIa. There is also cell-mediated platelet destruction by CD8+ T cells.
  • Autoantibodies interfere with megakaryocyte maturation, resulting in decreased production.
  • Fc-independent desialylated platelet clearance has been proposed as the mechanism of refractoriness to therapies that target the classic FC-dependent pathway.
  • Association in patients receiving immune checkpoint inhibitor therapy (2)

Risk Factors

  • Autoimmune thrombocytopenia (e.g., Evans syndrome)
  • Common variable immunodeficiency (CVID)
  • Drug side effect (e.g., quinidine, vancomycin, penicillin, sulfonamides)
  • Infections: Helicobacter pylori, hepatitis C, HIV, CMV, varicella zoster, measles, rubella, influenza, EBV, Whipple disease
  • Vaccination side effect. Live virus vaccinations carry a lower risk than natural viral infection: 2.6/100,000 cases MMR vaccine doses versus 6 to 1,200/100,000 cases of natural rubella or measles infections.
  • Bone marrow transplantation side effect
  • Connective tissue disease, such as systemic lupus erythematosus, antiphospholipid antibody syndrome
  • Lymphoproliferative disorders

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Basics

Description

  • Immune thrombocytopenia (ITP) is a condition characterized by the immunologic destruction of normal platelets and/or impaired thrombopoiesis in response to an unknown stimulus.
  • It is defined as a platelet count <100 × 109/L, once other causes of thrombocytopenia have been ruled out (1).
  • ITP nomenclature:
    • Newly diagnosed (<3 months), persistent (3 to 12 months), and chronic (>12 months)
    • Primary when it presents in isolation; secondary when associated with other disorders
  • ITP is a relatively common disease of childhood that typically follows a viral infection. Onset is within 1 week, and spontaneous resolution occurs within 2 months in >80% of patients.
  • In adults, ITP is usually a chronic disease and spontaneous remission is rare.
  • Synonym(s): idiopathic thrombocytopenic purpura; immune thrombocytopenic purpura; and Werlhof disease

Epidemiology

  • Peak age
    • Pediatric ITP: 2 to 4 years
    • Chronic ITP: >50 years with incidence 2 times higher in persons 60 years than those <60 years of age
  • Predominant gender
    • Pediatric ITP: male = female
    • Chronic ITP: female > male (1.2 to 1.7:1)

Incidence

  • Pediatric acute ITP: 1.9 to 6.4/100,000 children per year
  • Adult ITP: 1.6/100,000 per year (1)

Prevalence
Limited data; in one population (in Oklahoma):

  • Overall prevalence of 11.2/100,000 persons
  • In children (<16 years): 8.1/100,000 with average age of 6 years
  • In adults (>16 years): 12.1/100,000 persons with average age of 55 years

Etiology and Pathophysiology

  • Accelerated platelet uptake and destruction by reticuloendothelial phagocytes results from action of IgG autoantibodies against platelet membrane glycoproteins IIb/IIIa. There is also cell-mediated platelet destruction by CD8+ T cells.
  • Autoantibodies interfere with megakaryocyte maturation, resulting in decreased production.
  • Fc-independent desialylated platelet clearance has been proposed as the mechanism of refractoriness to therapies that target the classic FC-dependent pathway.
  • Association in patients receiving immune checkpoint inhibitor therapy (2)

Risk Factors

  • Autoimmune thrombocytopenia (e.g., Evans syndrome)
  • Common variable immunodeficiency (CVID)
  • Drug side effect (e.g., quinidine, vancomycin, penicillin, sulfonamides)
  • Infections: Helicobacter pylori, hepatitis C, HIV, CMV, varicella zoster, measles, rubella, influenza, EBV, Whipple disease
  • Vaccination side effect. Live virus vaccinations carry a lower risk than natural viral infection: 2.6/100,000 cases MMR vaccine doses versus 6 to 1,200/100,000 cases of natural rubella or measles infections.
  • Bone marrow transplantation side effect
  • Connective tissue disease, such as systemic lupus erythematosus, antiphospholipid antibody syndrome
  • Lymphoproliferative disorders

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