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September 23, 2013- Platelet Disorders 1.

Hemostasis depends on the interaction of vasculature, platelets, plasma coagulation factors, fibrinolytic system a. Primary Hemostasis- problems with platelets i. Mucosal/Cutaneous Bleeding 1. Easy bruising 2. Epistatis 3. Gingival bleeding 4. Prolonged bleeding with tooth extraction 5. Menorrhagia 6. Purpura 7. Petechiae Qualitative Platelet Disorders- normal in number, non-functioning 8. Glandzmann’s Thrombastheniaa. Autosomal recessive gene b. Normal platelet count c. Normal adhesiveness and retention- no aggregation except with ristocetin d. Abnormal clot retraction- unique to this disease e. + tourniquet test f. Increased bleeding time g. Bleeding- severe, early onset h. Spontaneous/aggravated by trauma i. Glycoprotein IIb IIIa missing from platelet membrane and decreased thrombasthenin (causes abnormal clot retraction) j. Treat with platelet concentrate 9. Von Willebrand’s Diseasea. Autosomal dominant gene on chromosome 12 (controls synthesis of factor VIII from megakaryocytes and endothelial cells) b. Heterozygote- usually mild- begins in second decade c. Homozygote- moderately severe- begins early, severity decreases with age d. Decreased active VIII:C (need for intrinsic coag) and VIII:VWF (necessary for ristoceitin aggregation, and adhesion of platelets to subendothelium) e. Treatment- cryoprecipitate (has both VIII:C and VIII:VWF, fibrinogen and XIII)- or DDAVP (deamino-8-d-arginine vasopressin- causes increased synthesis but must have ability to synthesize VIII:VWF) f. Platelet count normal g. Bleeding time increased- decreased VIII:VWF so can’t adhere to collagen h. APTT increased- because they’re not activating VIII:C which is essential to intrinsic coagulation

i. long term nutritional deficiency . Thrombocytopenia-DECREASED AMOUNTS OF PLATLETS a. Basophilic inclusions in granulocytes. Excessive destruction c. Homozygous: moderate to severe bleeding c. Problem is that the platelets lack glycoprotein Ib on membrane.look sort of like Dohle bodies but know they don’t have infection e. No aggregation with ristocetin (other agents it’s normal) 10. Giant platelets – size of lymphocytes f. Abnormal aggregation with ristocetin (other agents normal) i. Acquired= i. Normal number of megakaryocytes in bone marrow.platelet concentrate if bleeding 11. Down to 20. Thrombocytopenia (mild to moderate) e.can’t bind to VIII:VWF to attach to collagen and can’t utilize ristocetin. Relatively rare c. Congenital= rare b. Most patients clinically normal. Autosomal recessive – 1/1 million people b.something happening to platelets once outside BM g. Deficient production in bone marrow of platelets b. Bernard-Soulier Syndrome a. Increased bleeding time g. May Hegglin Anomaly a. Most common hemostatic problem come across with patients d. Decreased platelet adhesion j.000/uL can spontaneously hemorrhage so must do preventative measures 2. Autosomal dominant gene b. Heterozygous: mild to moderate bleeding d. Clot retraction normal j. Thrombocytopenia f. Treatment. Amegakaryocytic Thrombocytopeniaa. Decreased platelet adhesiveness h. but can attach to fibrin because doesn’t require Ib k.¼ have mild bleeding QUANTITAIVE ABNORMALITIES ii. Bizarre giant platelets d.

Insidious onset c. Thrombotic Thrombocytopenic Purpura (TTP) a. Mucosal/Cutaneous Bleeding. Treatment= steroid/splenectomy 5. Acute or chronic i. Decreased ADAMTS13 vWF cleaving protease. auto-immune d. toxins. Antibody to platelets b. Usually benign g. No activation of coagulation factors c. Strinking thrombocytopenia ii. Chemotherapy iv. Internal Bleeding. PT normal 6. Follows acute or long history b. rubella. Positive tourniquet test iv. Two types: i. Remissions/relapses d. kidney’srenal failure e. drugs. Idiopathic Thrombocytopenic Purpura. Sudden onset e. APTT normal v. 10% progress to chronic 4.more serious f. certain infections f. certain medications. Mostly in children c. Chronic ITP a.“white clots” (platelet clumps) b. History: respiratory infection. Bleeding time increased iii. Aplastic anemia iii.without have large molecules of vWF in blood will cause platelets to clump i. Platelet thrombi throughout vasculature. Treatment= steroids h. pregnancy.(ITP) a. Acute Idiopathic Thrombocytopenic Purpura (ITP) a. Etiology: associated with viral disease.milder ii. Ionizing radiation 3. Regional tissue damage: CNS (most markedly effected)-strokes. Decreased levels associated with estrogen. mumps d.ii. Familial (inherit a defective enzyme) or acquired (developing an auto-ab to the protease) . infections.

Polycythemia vera (increase in RBC. transfusion increases platelets about 10. Primary problem (below) ii. Thrombocytopenia. platelets) 5. h. CGL 6. Short-lived. Separate plasma from RBCs with light spin 2000 rpm for 3 minutes ii. schistocytes. Secondary to well defined clinical states: 1. j. will either bleed or throw clots 4. Mostly in children b. WBC.will increase 25-150% after surgery 3.(PRP)i. Platelet Rich Plasma.microangiopathic hemolytic anemia Increased bleeding time Treatment. Underlying malignancy b. Post splenectomy 2.b/c that’s where platelet clumps reside Thrombocytosis and Thrombocythemia. Severe bleeding or thrombosis 3. Hemolytic Uremic Syndrome. Increased large molecules of vWF in blood. activate platelets Thrombocytopenia.000/uL in about an hour . Schistocytes e. often symptomless ii. Maintain at room temperature maximum of 72 hours with gentle agitation 2.of platelets in solution iii. If greater than 1 million can’t predict if platelets will function correctly. Increased megakaryocytes therefore increased platelets iii.INCREASED NUMBER OF PLATELETS 1. Acute hemorrhage 4.g. Reduced renal function. best compatible HLA type a.(HUS) a. i.000/uL 2. Platelets have qualitative defects also Platelet Transfusions 1. 90% mortality rate if not treated 7. Yields ~250mL.b/c have platelet clumps d. corticosteroids (didn’t work very well however) k. ABO Compatible. Platelet thrombi specific to renal vasculature c. Platelet counts greater than 500. ~90% platelets in solution yielded iv. plasma exchange (best option). Treatment= chemotherapy a. Thrombocytosisi. Major surgery.fresh frozen plasma. Thrombocythemiai.

Use within 24 hours . Heavy spin (5000 rpm for 5 min) iii. Take PRP ii. Blood returned to patient iii. Use heparin ii. Left with platelet concentrate -Yields ~20-50 mL plasma with platelets c. Plateletpharesisi.b. Remove platelet poor plasma (supernatant) put in satellite bag iv. Platelet Concentratei.