Immune thrombocytopenic purpura treatment

Recommend treatment approach for the initial treatment of immune thrombocytopenic purpura (UP). 

Intravenous Immune Globulin (IGIV) IGIV is a product derived from blood plasma from a donor pool similar to the immune globulin (IG) pool, but prepared so it is suitable for intravenous use. IGIV does not transmit infectious diseases. It is primarily used for replacement therapy in primary antibody-deficiency disorders, for the treatment of Kawasaki disease, immune thrombocytopenic purpura, hypogammaglobulinemia in chronic lymphocytic leukemia, and in some cases of HIV infection. 

Tsiouris J, Tsiouris N. Hemiplegia as a comphcation of treatment of childhood immune thrombocytopenic purpura with intravenously administered immunoglobulin. J Pediatr 1998 133(5) 717. 

Immune thrombocytopenic purpura. For the treatment of hemorrhage associated with immune (or idiopathic) thrombocytopenic purpura (ITP), doses of 1 g/kg daily for 2 to 3 days plus high-dose methylprednisolone are indicated. 

Innovative variations in the engineering of BPs have been developed in an effort to create new therapeutics to treat human disease. For example, peptibodies, which are peptides fused to an IgG Fc molecule, have emerged. The only marketed peptibody, romiplostim (NPLATE ), was approved under a Biologic License Application (BLA) by the United States Food and Drug Administration (FDA) in 2008 and by European Medicines Agency (EMA) in 2009 for the treatment of immune thrombocytopenic purpura (Shimamoto et al. 2012). 

Vancine-Califani SM, De Paula EV, Ozelo MC, Orsi FL, Fabri DR, Annichino-Bizzacchi JM. Efficacy and safety of dapsone as a second-line treatment in non-splenectomized adults with immune thrombocytopenic purpura. Platelets 2008 19(7) 489-95. 

Kumar S, Diehn FE, Gertz MA, Tefferi A (2002) Splenectomy for immune thrombocytopenic purpura long-term results and treatment of postsplenectomy relapses. Ann Hematol 81 312-319... 

Viral infection The authors report a case in which a child became infected with Epstein-Barr virus developed immune thrombocytopenic purpura, and treatment with anti-D Ig caused intravascular haemolysis as well as acute kidney injury [118 ]. Although some degree of extravascular haemolysis is expected, this case is tmusual because of tile severity and intravascular nature. The authors speculate that the use of intravenous anti-D triggered an xmusual virus-induced immune response that resulted in the pattern of haemolysis seen, which has been reported previously. 

Podolanczuk A, Lazarus AH, Crow AR, Grossbard E, Bussel JB (2009) Of mice and men an open-label pilot study for treatment of immune thrombocytopenic purpura by an inhibitor of Syk. Blood 113 3154-3160... 

Idiopathic thrombocytopenic purpura is an immune-mediated disease in which immunoglobulin, either as antibody directed against platelet antigens or nonspecifically bound to platelets, is present in increased quantities on platelets. This leads to increased destruction of platelets and, in many instances, megakaryocytes. Standard treatment consists of corticosteroids and splenectomy (72). When these measures fail, treatment may include androgenic steroids, administration of intravenous 7-globulin, or injection of vinca alkaloids. 

Immune globulin, given intramuscularly or intravenously, is recommended in the treatment of primary humoral immunodeficiency, congenital agammaglobulinemias, common variable immunodeficiency, severe combined immunodeficiency, idiopathic thrombocytopenic purpura, and autoimmune hemolytic anemia. There are six licensed preparations of immune globulin. 

Idiopathic thrombocytopenic purpura (ITP) Immune globulin (IV)2 Consult the manufacturer s dosing recommendations for the specific product being used. Response in children with ITP is greater than in adults. Corticosteroids are the treatment of choice in adults, except for severe pregnancy-associated ITP. 

Although this treatment has been available for idiopathic thrombocytopenic purpura for several years, its mechanism of action is not understood. Removal of IgG and IgG-containing immune complexes does not explain its effects in rheumatoid arthritis. The most recent hypothesis for this treatment s mechanism of action is down-regulation of B cell function through the release of small amounts of staphylococcal protein A complexed with immunoglobulins. 

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