idiopathic thrombocytopenic purpira

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Idiopathic thrombocytopenic purpura

Definition:

It is an autoimmune bleeding disorder in which antibodies are formed against platelets causing the destruction and premature removal by monocyte macrophage system. Exact etiology is not known but is associated with some risk factors mentioned below

Post infection

ITP may follow infection with rubella –HIV-HBV (infectious mononucleosis)

Vertical transmission

Mother with ITP, her infant also affected because of platelet transport across the placenta.

post transfusion

Following administration of the platelet antigen positive blood to patient who lacks this specific platelet antigen develops ITP.

Associated with Autoimmune disorders

Examples included SLE, lymphoproliferative disorder.

Clinical features

· Acute type occurs in children of 2-6 yrs .appears suddenly following non specific viral infection

· 70 % causes revolve spontaneously within few weeks.

· Chronic occurs in adults predominantly in female .onset is insidious with relapses and remission.

· 10% of cases resolve by itself.

Features

Petechial hemorrhages on skin.
Epistaxis
Bleeding from gums
History of bruising in chronic cases
Splenomegaly
Increases in bleeding after trauma.

Investigations

A) Blood examination

Platelet count decreased
Bleeding time prolonged
Clotting time normal
Prothrombin time normal or increased
Clot retraction poor
Capillary fragility is greatly increased(rumple-leede test)

B) Bone marrow aspiration

a) Purpose: to diagnose ITP, to rule out leukemia and aplastic anemia.

b) Increased megakaryocytes: With following characteristics

Not surrounded by platelets
abnormal in shape
single nuclei
after vacuoles seen

Treatment:

General measures

Patient should avoid trauma, elective surgery, tooth extraction etc.
Also avoid unnecessary medications for example aspirin.

Corticosteroids

Prednisolone tablet 10-20 mg qti

Dose should be continued until platelet returns to normal then gradually decrease the dose.

Indications of Splenectomy:

Cases that relapse 2-3 times after steroid therapy

Cases not responding to steroid treatment

Platelet count rise quickly following splenectomy and becomes double within 24 hours and …….

Immunosuppressive therapy

Case that not responding to steroid treatment
Case when splenectomy failed t increase platelets
Drug IV vincristine -1.4 mg for 4-6 weeks

IV immunoglobulin

In acute crisis
Preoperatively in patient requiring surgery.
Dose – IV immunoglobulin 400 mg /kg for five consequent days.

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