Clinical Staging of Immune Thrombocytopenic Purpura

 

Platelet Count per µL	Clinical Bleeding	Stage
> 20,000 per µL	none	I
10,000 – 20,000 per µL	skin only	II
10,000 – 20,000 per µL	mucosal bleeding	III
< 10,000 per µL	any bleeding	IV

 

Bleeding:

(1) skin: petechiae, purpura, ecchymoses

(2) mucosal: epistaxis, gingival bleeding, hematuria, menorrhagia, melena

(3) internal hemorrhage: intracranial hemorrhage, other

 

Stage	Management Recommendation
I	observe; avoid antiplatelet agents and trauma, control blood pressure and any medical condition that increases the risk for bleeding
II	observe; treat prior to surgery or other situations where bleeding expected
III	treat
IV	treat

 

Treatment options include:

(1) Platelet transfusions: These usually are ineffectual, since the platelets are quickly destroyed. Platelet transfusions are used only as a last ditch attempt to stop life threatening hemorrhage.

(2) Intravenous steroids: Prednisone or methylprednisolone are effective for initial control of the thrombocytopenia, but patients may have recurrence of disease when steroids are discontinued.

(3) Intravenous infusion of immune globulin (IVIG) concentrates: These are effective in many patients but the therapy is expensive.

(4) Anti-D immunoglobulin (Rhogam): Infusion of anti-D immunoglobulin has been approved for use in D-positive patients with ITP, but severe hemolytic anemia may occur.

(5) Vinca alkaloids: These have a variable benefit and are not routinely used.

(6) Plasmapheresis: Some people have tried plasma apheresis to remove the causative antibody, but IgG has a large volume of distribution and is difficult to completely remove.

(7) Immunosuppressant therapy: This is usually reserved for patients with severe disease refractory to other treatments.

(8) Splenectomy: This may be used in patients resistant to other forms of therapy.

 

An International Childhood ITP Registry (www.unibas.ch/itpbasel) has been developed to follow children with ITP.