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Immune Thrombocytopenic Purpura (ITP)

It is an autoimmune disorder due to the presence of auto- antibodies directed against platelet membrane glycoprotein IIb and IIIa resulting in premature removal of platelet by macrophage monocyte system.
Cause of Autoimmune Thrombocytopenia
  1. Idiopathic
    1. Immune thrombocytopenic purpura (ITP)
  2. Secondary
    1. Connective tissue disorders (Specially SLE)                
    2. Lymphoproliferative disorders (Lymphoma, CLL)
    3. Infections (example HIV infection)                                    
    4. Drugs
    5. Solid tumors
Extra Edge:
Laboratory testing is performed to evaluate for secondary causes of ITP and should include testing for HIV infection and hepatitis C (and other infections if indicated); serologic testing for SLE, serum protein electrophoresis, and immunoglobulin levels to potentially detect hypogammaglobulinemia; selective testing for IgA deficiency or monoclonal gammopathies and, if anemia is present, direct antiglobulin testing (Coombs test) to rule out combined autoimmune hemolytic anemia with ITP (Evans syndrome). 

Clinical Features
  1. In children: Typically presents 2 – 3 weeks after a viral infection with sudden onset of purpura, nasal or oral bleeding. 
  2. In adults: ITP involves females more commonly and has insidious onset. Symptoms and signs of collagen vascular disorders (like rheumatoid arthritis) may be present. The course is chronic with remissions and relapses. 
  3. Splenomegaly and bleeding in the joints are not the feature of ITP.
                                           Acute ITP                                      Chronic ITP
Age                                      Children 2-6 years                         Adults 20-40 years
Sex ratio                               None                                            F:M 3:1 female more
Antecedent infection common   (1-2 week prior)                          Unusual
Onset                                     Abrupt                                        Insidious
Bullae in mouth                       Present                                       None
Platelet count                          <20,000                                     30000-80000
Eosinophilia & lymphocytosis    Common                                     Rare
Duration                                2-6 weeks                                     Months or years
Spontaneous remission          Present in 80%                               Uncommon
Extra Edge: In ITP, BT is increased (LQ 2012) but CT is normal.

Note: In ITP, BT is prolonged but CT is normal. 

  1. In children, the disease is usually self – limiting within a few weeks. 
  2. Drugs therapy
    1. Prednisolone        
    2. Intravenous immunoglobulin
  3. Splenectomy
Extra Edge:
  1. Platelet transfusion is not effective because in ITP, thrombocytopenia is caused by immune mediated destruction of platelet.
  2. Most common cause of death- cerebral hemorrhage. 
Recent Advances:
  1. Two agents thrombopoietin receptor agonists TPO receptor agonist, have shown response in many patients with refractory ITP.
  1. Romiplostim (Given subcutaneously)
  2. Eltrombopag (Oral) 
  1. Rituximab

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