Download - Chronic itp
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CHRONIC ITP
By DR Falak abro
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• chronic ITP persists for more than 6-12 months.
• Chronic ITP has more insidious onset with easy bruising and petechiae
•Age 8-14 years.
• male : female ratio is 1:2
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PATHOPHYSIOLOGY
• Increased platelet destruction .
• Spleen is key organ in Pathophysiology
.platelet autoantibodies formed in white pulp
. Macrophages in red pulp destroy immunoglobulin coated platelets.
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PATHOPHYSIOLOGY
•Autoantibody coated platelets induce Fc receptor-mediated phagocytosis by mononuclear macrophages
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Clinical Signs and symptoms
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*Bruise
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Petechiae
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PurPura
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Gingival bleeding
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Epistaxsis
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INVESTIGATION
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PERIPHERAL BLOOD FILM
• ITP with low platelet count usually (<100,000 usually) with normal hemoglobin and WBC count
• thrombocytopenia with platelet count <20 x 109/L is common and platelet size may be normal or increased
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Bone Marrow Examination
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•The bone marrow in patients with ITP contains normal or increased number of megakaryocytes indicates that :
a: plateletes production is normal
b: thrombocytpenia is due to increased platelet destruction.
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TREATMENT
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• Wait and Watch if platelets are below 50,000 or there is no signs of bleeding.
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INTRAVENOUS IMMUNOGLOBULINS
•Mechanism:
blocking FC receptors of RE (reticuloendothelial) phagocytes.
• preventing them from binding and destroying IgG antibody-coated platelets.
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LNH PHARMACY
1 vial= 250 mgPrice= approx Rs: 1500
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Dose : safe dose is 400mg/kg/day using 5 days continuously or 1g/kg/day for 2 days.
Merits: IVIG is preferable to steroids because it causes faster elevation of platelete count greater than 20,000 within 24 hours.
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Demerits :• Its expensive
• Long infusion time of 6-8 hours
• Headache
• vomiting
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Corticosteroids:
Mechanism
• inhibit platelet destruction.
•Rapid action that reduces RE destruction of antibody coated platelet.
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Dose:•Oral prednisone 1-2mg/kg/day for two weeks then tapered over third week
• In chronic ITP with recurrent bleeding intravenous methyl predinisolone20-30mg/kg/day for 3 days can be given.
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Intravenous Anti D therapy :MECHANISM:• Specific red blood cell antibodies coat red blood cells, which are taken by RE system in place of antibody coated platelets.
•Anti Rh-D immunoglobulin produces mild hemolytic anemia that saturates Fc receptors of phagocytic elements of RE system.
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•Dose I/V 50ug -75ug/kg for two days platelet rise within 48 hours to 72 hours.
•Merits: Lower side effects than IVIG doesnot cause headach or vomiting
•Demerit: causes hemolysis.
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LNH PHARMACY
1 vial= 300 ugPrice = RS: 5500
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SPLEENECTOMY:
•Indicated in chronic, symptomatic ITP when other options fail.
•About 64-88% of patient with chronic Itp achieve complete remission.
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It is done because It removes:• primary site of platelete destruction and
site of antiplatelete-antibody production
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RITUXIMAB:MECHANISM:
• it is monoclonal antibody which depletes B-cells by binding to the CD-20 antigen surface •Therapy effect remains for 6-12 months as it prevents activity of autoreactive cells specially against gp-IIb/IIIa.
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Dose : 375mg/m2 per dose weekly for 4 weeks.
Price : 100mg vial approx RS: 17000 500mg vial approx RS: 85000
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Time to respond therapy : 1-7 weeks
Side-effects: fever, chills, allergy reactions which can be prevented by slow infusion and premedication with antihistamine or steroids.
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(Thrombopoeitin) TPO receptor agonists
•Mechanism of action
•Endogenous TPO made in liver which regulates platelet production by increasing the number and maturation of bone marrow megakaryocytes.
•2 TPO receptor agonists : a: romiplostim with dose 1-10ug/kg/dose subcutanously weekly.
b: Eltrombopag with dose is 50mg orally.
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•ROMIPLOSTIM is a subcutaneous thrombopoiesis stimulating FC-peptide fusion protein.
•ELTROMBOPAG is orally active non peptide agonist, it acts by stimulating platelet production.
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Side effects
• Romiplostim : headache, phyrangitis, fatigue
•Eltrombopag : nausea and vomiting, hepatic toxicity
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THANKYOU…