Idiopathic thrombocytopenic purpura

Hematologic diseases autoimmune hemolytic anemia, idiopathic thrombocytopenic purpura, pernicous anemia Kidney disease Goodpasture syndrom, lipoid nephroses, minimal change glomerulonephritis Diseases of the gastrointestinal tract autoimmune chronic active hepatitis, autoimmune atrophic gastritis, Crohn s disease, ulcerative colitis... 

Hematologic von Willebrand disease Idiopathic thrombocytopenic purpura Factor VII defect causing impaired platelet adhesion and increased bleeding time Decrease in circulating platelets—can be acute or chronic... 

HBP High blood pressure ITP Idiopathic thrombocytopenic purpura... 

Primary immunodeficiency states, including both antibody deficiencies and combined deficiencies / Idiopathic thrombocytopenic purpura... 

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). 

Autoimmune disorders Development or exacerbation of autoimmune disorders, including myositis, hepatitis, idiopathic thrombocytopenic purpura, psoriasis, rheumatoid arthritis, interstitial nephritis, thyroiditis, and systemic lupus erythematosus have been reported in patients receiving alpha interferon. 

ITP idiopathic thrombocytopenic purpura IV intravenous IVP intravenous push JRA juvenile rheumatoid arthritis K/K potassium KVO ke vein open L/d liters per day LA long acting LDL low-density hpoprotein LFT liver function test LH leuteinizing hormone LHRH luteinizing hormone releasing hormone... 

McMillan, R., The pathogenesis of chronic immune (idiopathic) thrombocytopenic purpura. 

Jantunen E, Nousiainen T. Pulsed dexamethasone for refractory idiopathic thrombocytopenic purpura. Am J Hematol 1996 53 281-2. 

Vesely SK, Perdue JJ, Rizvi MA, Terrell DR, George JN. Management of adult patients with persistent idiopathic thrombocytopenic purpura following splenectomy a systematic review. Ann Intern Med 2004 140 112-20. 

Idiopathic thrombocytopenic purpura Membranous glomerulonephritis Polymyalgia rheumatica Polymyositis Psoriatic arthropathies Rheumatoid arthritis Systemic lupus erythematosus Ulcerative colitis Uveitis... 

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. 

It is indicated in acute leukaemias, lymphomas, Ewing s sarcoma, neuroblastoma, Wilm s tumour and idiopathic thrombocytopenic purpura. 

Intravenous gamma globulin preparations are available for replacement therapy for patients with congenital agammaglobulinaemia and hypogammag-lobulinaemia, idiopathic thrombocytopenic purpura and Kawasaki syndrome. It is also used for prophylaxis of infection following bone marrow transplantation. 

It is indicated in renal transplantation, severe active rheumatoid arthritis unresponsive to other therapy, certain autoimmune diseases, chronic active hepatitis, idiopathic thrombocytopenic purpura and acquired haemolytic anaemia. 

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) Prednisone,1 vincristine, occasionally cyclophosphamide, mercaptopurine, or azathioprine commonly high-dose gamma globulin, plasma immunoadsorption or plasma exchange Usually good... 

Azathioprine and mercaptopurine appear to be of definite benefit in maintaining renal allografts and may be of value in transplantation of other tissues. These antimetabolites have been used with some success in the management of acute glomerulonephritis and in the renal component of systemic lupus erythematosus. They have also proved useful in some cases of rheumatoid arthritis, Crohn s disease, and multiple sclerosis. The drugs have been of occasional use in prednisone-resistant antibody-mediated idiopathic thrombocytopenic purpura and autoimmune hemolytic anemias. 

The effectiveness of immunosuppressive drugs in autoimmune disorders varies widely. Nonetheless, with immunosuppressive therapy, remissions can be obtained in many instances of autoimmune hemolytic anemia, idiopathic thrombocytopenic purpura, type 1 diabetes, Hashimoto s thyroiditis, and temporal arteritis. Improvement is also often seen in patients with systemic lupus erythematosus, acute glomerulonephritis, acquired factor VIII inhibitors (antibodies), rheumatoid arthritis, inflammatory myopathy, scleroderma, and certain other autoimmune states. 

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. 

Danazol is a weak androgen and can thus exert the adverse effects of androgens, but because it inhibits LH and FSH secretion it can also elicit menopausal-like symptoms. However, from time to time it has other types of adverse effect. It has been used with some success in the management of pelvic pain associated with endometriosis (1), heavy menstrual bleeding (2), and idiopathic thrombocytopenic purpura (3). It is ineffective in unexplained subfertility (4). 

A patient developed pulmonary fibrosis after having taken danazol for 2 months for idiopathic thrombocytopenic purpura (6). He developed bilateral pneumothoraces and pneumomediastinum and died. An association between danazol and lung fibrosis has been reported only once before here it could have been coincidental or a reflection of a pathological process underlying the originally diagnosed disorder. 

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. 

Andres E, Zimmer J, Noel E, Kaltenbach G, Koumarianou A, Maloisel F. Idiopathic thrombocytopenic purpura a retrospective analysis in 139 patients of the influence of age on the response to corticosteroids, splenectomy and danazol. Drugs Aging 2003 20(ll) 841-6. 

Pakhale S, Moltyaner Y, Chamberlain D, Lazar N. Rapidly progressive pulmonary fibrosis in a patient treated with danazol for idiopathic thrombocytopenic purpura. Can Resp J 2004 11 55-7. 

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. 

Azathioprine is an effective agent in suppressing the immune system in patients undergoing renal transplantation and in patients suffering from acute glomerulonephritis, the renal component of systemic lupus erythematosus, prednisone-resistant idiopathic thrombocytopenic purpura, and functioning autoimmune hemolytic anemia. Azathioprine depresses bone marrow functioning, which is its chief side effect. 

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. 

---