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16 Cards in this Set
- Front
- Back
Treat resistant immune (idiopathic) thrombocytopenic purpura.
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* Romiplostim and eltrombopag are recently approved thrombopoietin receptor agonists that are effective in the treatment of chronic immune (idiopathic) thrombocytopenic purpura.
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Clinical studies of recombinant human thrombopoietin resulted in antibody f
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Clinical studies of recombinant human thrombopoietin resulted in antibody formation to thrombopoietin and thrombocytopenia in normal subjects. Because of this, recombinant thrombopoietin is not useful in ITP or other clinical disorders.
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Romiplostim
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Romiplostim is given parenterally, once weekly; and eltrombopag is given orally, daily. Neither have sequence homology with thrombopoietin, and it is unlikely to cause antibody formation against the growth hormone. Romiplostim is effective in both splenectomized and nonsplenectomized patients and both agents allow corticosteroids or other therapies to be tapered during treatment. Serious adverse events are uncommon with either agent. There have been episodes of thrombosis and increased bone marrow reticulin reported.
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Plasmapheresis in tx of ITP
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Plasmapheresis is not effective in removing the autoantibodies in patients with ITP, because these are usually IgG antibodies that are found in both the intravascular and the extravascular compartments. The IgG that is removed from the intravascular compartment is therefore rapidly replaced after equilibration within the extravascular space.
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children,adults treatment,
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children, this disease is usually self-limiting
and requires no treatment, but adults generally require medical (through immunosuppression or dialysis) or surgical (through splenectomy) management. |
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Children with chronic ITP have
platelet counts from 20,000 to 75,000/mm3 .... treatment. |
Children with chronic ITP have
platelet counts from 20,000 to 75,000/mm3 and typically do not require treatment. Pulse or short-course corticosteroids may be used in refractory cases, but long-term daily steroid use should be avoided. |
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what cause ITP
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autoimmune AB formation againsts host plateles( IgG) they coat and damage platelets, which are then removed by splenic macrophages ( RES binds self immunoglobulins attached to the platelets
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what are 2 forms of ITP
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1) acute - seen in children precede by viral infection( in most cases)
usually self limited ( 80 % resolved in 6 months) 2)chronic form in adults mostly women 20-40 spont remission are rare |
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clinical picture of ITP
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petechia , echhymoses on the skin
bleeding of the mucous membranes no spleno megaly! |
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ds
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platelets are usually < 20.000
rest blood is normal 9 but if severe bleeding occurs>>Hb/Hct decreased and reticulocytes are increased |
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tx of ITP
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1adrenal cortoco steroids
2iv immune globulin( they saturated RES binding site for platelet bound self immunoglobulin so less platelets uptake and destruction by spleen 3 splenectomy - if chronic ITP>> remission in 70-80 % |
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if splenectomy what about immunization
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risk for pneumoc sepsis, will need antibiotic prophylaxis and surgeryis delayed untill P is 5 years old
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what problem could be with P - kid if taken prednizon
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behavioral and avascular necrosis( as long term effect)`
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why could be failure of itp to responce for spleenectomy
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additional spleen
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activities and NSIDS in KID with ITP
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restrict activitie and stop drugs
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if ITP in pregnant ( platelets < 20 000) what will be with infacts
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10 % change to have platelets <50 t
4% < 20T if pregnant need splenectome- infant would have more severe thrombocytopenia |