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CCT Hemotology

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Hematopoietic system componenets   Erythrocytes (RBC), Leukocytes (WBC), Platelets, Plasma  
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Red Blood Cells   Contains hemoglobin and carries oxygen, biconcave in shape, accounts for 1-2% of red cells in blood.  
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White Blood Cells   Primarily responsible for immune protection, Absolute WBC count often not highly specific of sensitive in the diagnosis of diseases.  
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WBC Differential   (Percentage of different types of WBCs) can be more helpful in determining presence of active infection.  
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Platelets   Primarily responsible for hemostasis, initial defense mechanism for acute blood loss, circulatory life span of 9-10 days, contains proteins on the surface that allow them to stick together.  
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Plasma   Yellowish, liquid component of whole blood, comprises of 55% of total blood volume, composed of 92% water by volume, contains dissolved proteins, ions, and clotting factors.  
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Anemia   An absolute or relative decrease in the number of circulating, functional RBCs  
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Polycythemia/erythrocytosis   An absolute or relative increase in the number of circulating RBCs  
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Thrombocytopenia   An absolute or relative decrease in the number of circulating, functional platelets.  
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Thrombocytosis   An absolute or relative increase in the number of circulating, functional platelets.  
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Leukopenia   An absolute or relative decrease in the number of circulating, functional WBCs.  
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Leukocytosis   An absolute or relative increase in the number of circulating functional WBCs.  
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Components of Hemostasis   Vascular integrity platelet function Coagulation factors Fibrinolysis  
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Bleeding time   measures time between skin incision and cessation of bleeding, normal= 3-8 minutes  
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Platelet Count   Reported as part of complete blood count (CBC), normal= 150,000-400,000/mm.  
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Prothrombin Time (PT)   Evaluates function of extrinsic pathway of coagulation cascade, normal control values= 11-13 seconds.  
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International Normalized Ratio (INR)   Calculated from PT ratio that would have been obtained if the world health organizations reference thromboplastin had been used in measurement of PT.  
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INR also does what?   Allows monitoring of anticoagulation effects if Coumadin.  
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INR therapeutic range depends on reson for anti-coagulation   Atrial fibrillation, biological valve replacement, prevention of DVT/VTE = 2.0-3.0 Mechanical valve replacement, recurrent systemic VTE = 2.5-3.5  
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Partial thromboplatin time (PTT)   test all factors except VII and XIII, but used primarily to evaluate intrinsic pathway  
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PTT Normal value   Normal= 25-35  
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Thrombin time   Evaluates ability of fibrinogen (Factor I) to convert into a fibrin clot  
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Thrombin time normal value   < 22 seconds, heparin therapy -inhibits fibrin formation  
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Heparin has no:   Fibrinolytic capacity CAN BE REVERSED BY PROTAMINE SULFATE  
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tPA...?   Tissue plasminogen activators (tPA, rtPA)  
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tPA does what?   Catalyzes conversion of plasminogen to plasmin - Major enzyme responsible for clot degradation Can result in bleeding in sites other than desired location  
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FFP...?   Fresh Frozen Plasma  
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FFP contains what?   Contains all coagulation factors except platelets. Used for treatment of a pt with an unknown or undiagnosed bleeding disorder with clinically significant active hemorrhage  
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Platelets indicated for what?   Indicated for pt's with bleeding due to thrombocytopenia or platelet dysfunction  
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Typical adult dose of a 6-unit pack of platelets will raise platelet count by about...?   50,000/mm3  
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Willebrands disease =   Factor VIII containing vWF (Humate-P or Koate HS)  
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Jaundice caused by what...   Buildup of bilirubin in circulatory system may also indicate increased hemolysis of RBCs  
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Anemia is what...?   an absolute decrease in the number of circulating RBCs  
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Mayor function of RBC is to...?   Transport oxygen  
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Anemia is classified into three casual categories:   Increased RBC destruction Decreased RBC production Acute or chronic blood loss  
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Causes INTRINSIC or EXTRINSIC to RBC cell membrane   INTRINSIC- Enzyme defects, Membrane abnormality, Hemoglobin abnormality EXTRINSIC- Immunologic, mechanical, environmental, abnormal sequestrations  
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Sickle Cell-   Most common hemoglobinopathy encountered in american emergency departments, affects predominantly person of african ancestry  
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Sickle cell patho-   Causes an interlocking with an adjacent hemoglobin chain in the deoxygenated state, leading to the "sickling" of the entire RBC  
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Sickled cells cause and are what?   Cause an increase in viscosity, are less deformable and do not pass through microcirculation, and are sequestered and destroyed the the liver and spleen  
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Sickle cells S&S:   Pt's most often present with symptoms related to a vaso-occlusive crisis and associated sequelae  
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Sickle cell: Vaso-occlusive Crisis-   Severe in extremities, back, joints, abdomen, or chest. Treatment is analgesics and fluid replacement if clinically dehydrated.  
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Hemolytic Crisis treatment:   Similar to vaso-occlusive crisis but includes treatment for potential shock state, may require blood transfusion if symptomatically anemic.  
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Priaprism is what?   Sustained, painful pathologic erection, occurs in 10-40% of male's with sickle cell, can result in impotence.  
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Priaprism treatment?   Immediate urologic consultation, terbutaline 0.25-0.5 mg SQ (reduces blood flow to the penis and other end-organs), Hydration, Analgesia (comfort only) and pRBC transfusion  
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Acute Chest Syndrome accounts for...   25% of premature deaths in patients with sickle cell disease.  
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Acute Chest Syndrome Tx:   No definitive therapy identified, supportive care -Hydration -Analgesia -Maintenance of oxygen and ventilation -Empiric antibiotics  
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Thrombocytopenia is what?   Decreased production of platelets - Due to bone marrow suppression from variety of causes  
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Immune thrombocytopenia is what?   Increased destruction due to an antiplatelet antibody Can be related to various other immunilogic disorders such as lupas, leukemia, lymphoma, etc.  
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Heparin-induced thrombocytopenia (HIT):   -Immune-mediated side effect Occurs in 1-5% of pts receiving heparin -Usually occurs within 5-7 days of first dose -Diagnosis is suggested by absolute thrombocytopenia or a >50% reduction in platelets after beginning therapy -Mortality up to 30%  
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Disseminated Intravascular Coagulation (DIC):   -Relatively common acquired coagulopathy -Most often encountered in critical care setting  
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DIC potential causes:   -Trauma (burns, crush injuries, TBI, etc) -Pregnancy complications -Sepsis -Transfusion of drug reaction -Envenomation -Heat stroke  
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DIC   Out of control coagulation of fibrinolytic cascade within systemic circulation  
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DIC- lifethreatening combination of:   Bleeding tendency -Loss of platelet and remaining platelet dysfuction -loss of coagulation factors -fibrinolysis Fiberin deposition -Small vessel obstruction -Tissue ischemia RBC injury and resulting anemia  
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Diseases of Hemophilia A   caused by a genetic variant of factor VIII -present in normal levels but lacks procoagulant properties -Abnormal gene carried on X chromosome  
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Hemophilia A:Female carriers can have the following possible offspring with each pregnancy   -female carrier -female without carrier trait -male with hemophilia A -male without hemophilia A  
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Hemophilia A: Males with hemophilia A can have the following possible offspring   -All daughters will be carriers -None of the sons will have hemophilia  
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Hemophilia treatment:   Desmopressin (DDAVP) can be used to increase levels of Factor VIII in pts with mild Hemophilia A and is treatment of choice -Dose is 0.3mcg/kg intranasally, IV or SQ  
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Hemophilia B:   Caused by genetic decrease in function of factor IX  
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Hemophilia B treatment:    
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Malignant Pericardial Tamponade:   -Most often caused by pericardial effusiions -Most common primary malignancies *Lung *Breast *Esophagus *Leukemia *Melanoma -Can result from radiation therapy to chest  
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Malignant Pericardial Tamponade treatment:   -Initial treatment of pericadiocentesis with (or without, but more risky) ultrasound guidance can be lifesaving -Acts as a bridge to pericardial window in operating room  
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Hernation Syndromes   -Uncal herniation -Central herniation -Tonsillar herniation  
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