Idiopathic thrombocytopenic purpura treatment

Sequential determination of platelet counts in patients receiving vincristine during early studies unexpectedly occasionally revealed thrombocytosis, which could not be accounted for by systemic response to treatment alone 10,11). Ultimately shown to most likely be the result of increased megakaryocytic endomitosis II), the observation led to the use of vincristine, and later vinblastine, both alone and bound to platelets, in a variety of thrombocytopenic disorders. These include idiopathic thrombocytopenic purpura, thrombotic thrombocytopenic purpura, and chemotherapy-induced microangiopathic hemolytic anemia. 

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. 

In patients with thrombocytopenia associated with a variety of conditions, 17 of 22 improved (7i). Though these results have not been confirmed with this degree of success 14), vincristine remains an important agent in the treatment of refractory idiopathic thrombocytopenic purpura (ITP). 

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. 

Treatment of primary immunodeficiency states in which severe impairment of antibody forming capacity idiopathic thrombocytopenic purpura (IPT) bone marrow transplantation pediatric HIV infection... 

Romiplostim Genetically engineered protein in which the Fc component of a human antibody is fused to two copies of a peptide that stimulates the thrombopoietin receptors approved for treatment of idiopathic thrombocytopenic purpura ... 

Danazol has also been used in the treatment of fibrocystic disease of the breast and hematologic or allergic disorders, including hemophilia, Christmas disease, idiopathic thrombocytopenic purpura, and angioneurotic edema. 

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. 

Braendstrup P, Bjerrum OW, Nielsen OJ, Jensen BA, et al. 2005. Rituximab chimeric anti-CD20 monoclonal antibody treatment for adult refractory idiopathic thrombocytopenic purpura. Am... 

Danazol is a weak androgen and also has a series of other hormonal and anti-hormonal properties. It inhibits pituitary gonadotropin and has been used in the treatment of endometriosis, fibrocystic disease of the breast, idiopathic thrombocytopenic purpura, and hereditary angioedema. Its hepatotoxic effects include reversible rises in serum transaminases and cholestatic hepatitis a few cases of hepatocellular tumors have been reported. 

A 37-year-old woman with refractory idiopathic thrombocytopenic purpura, who had responded dramatically to danazol 600 mg/day after the failure of other forms of treatment, developed a multinodular well-differentiated trabeculovesicular hepatocellular tumor after 5 years. There were no metastases and no invasion of the lymph nodes, and after chemoembolization she received an orthoptic liver transplant. A year later she remained in good condition. 

Prednisone is used in the treatment of autoimmune diseases, both organic-specific autoimmune diseases such as myasthenia gravis and idiopathic thrombocytopenic purpura and nonorgan-specific autoimmune diseases such as lupus erythematosus, rheumatoid arthritis, and periarteritis nodosa. In addition, prednisone is used in patients receiving organ transplants and is of paramount value in countering the problems associated with organ rejection. 

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. 

Kappers-Klunne MC, van t Veer MB. Cyclosporin A for the treatment of patients with chronic idiopathic thrombocytopenic purpura refractory to corticosteroids or splenectomy. Br J Haematol 2001 114(l) 121-5. 

Dammacco F, lodice G, Campobasso N. Treatment of adult patients with idiopathic thrombocytopenic purpura with intravenous immunoglobulin effects on circulating T cell subsets and PWM-induced antibody synthesis in vitro. Br J Haematol 1986 62(l) 125-35. 

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. 

Vincristine (1.4 to 2.0 mg/m ) is indicated in the treatment of acute leukemia and in combination therapy for Hodgkin s disease, non-Hodgkin s malignant lymphomas (lymphocytic, mixed-cell, histiocytic, undifferentiated, nodular, and diffuse types), rhabdomyosarcoma, neuroblastoma, and Wilm s tumor. In addition, vincristine has been used in the treatment of idiopathic thrombocytopenic purpura, Kaposi s sarcoma, breast cancer, and bladder cancer. The intrathecal administration of vincristine is fatal. 

Kimura F,Itoh H,Ambiru S,et al. (2002) Long-term results of initial and repeated partial splenic embolization for the treatment of chronic idiopathic thrombocytopenic purpura. AJR Am J Roentgenol 179 1323-1326... 

Anti-D immunoglobulin is used as an alternative to intravenous immunoglobulin to treat idiopathic thrombocytopenic purpura. Patients treated with anti-D immunoglobulin (WinRho ), in contrast to earlier published reports, had more adverse reactions, particularly chills and rigors, despite pretreatment with paracetamol and diphenhydramine [66 ]. Two patients needed treatment for severe anemia and one for severe hemoglobinuria. Pretreatment with glucocorticoids or the use of subcutaneous anti-D immunoglobulin may reduce transfusion-related adverse reactions. However, the... 

For treatment of encephalitis convulsions, idiopathic thrombocytopenic purpura, atherosclerosis, viral myocarditis, cancer, secondary erythroderma, nitrite poisoning, bronchialasthma and acute viral hepatitis ... 

Whilst vincristine is normally mildly platelet suppressive, when it is used in such conditions as chronic idiopathic thrombocytopenic purpura, a rise in the platelet count may be seen. This thromhocythaemic effect has been suggested as a treatment for resistant cases of ITP (22). In these patients the effect has usually been to raise the platelets from a low value to within normal limits, but in most cases the platelets return to the previous lower levels in spite of the continued use of vincristine (22, 83). 

The best results of this treatment came in the first reported series of patients. As is often the case, this highly selected group of patients responded better than subsequently studied series. In the original report, 21 patients with idiopathic and 22 with secondary thrombocytopenic purpura were given 2 mg doses of intravenous vincristine (1 mg in children) every 7-10 days. Improvement in platelet count was seen in 16 patients (76%). 

Niaudet P, Habib R (1994) Cyclosporine in the treatment of idiopathic nephrosis. J Am Soc Nephrol 5 1049-1056 Niaudet P, Habib R (1998) Methylprednisolone pulse therapy in the treatment of severe forms of Schoenlein-Henoch purpura nephritis. Pediatr Nephrol 12 238-243 Noris M, Ruggenenti P, Perna A et al (1999) Hypocomple-mentemia discloses genetic predisposition to hemolytic uremic syndrome and thrombotic thrombocytopenic purpura Role of factor H abnormalities. J Am Soc Nephrol 10 281-293... 

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