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CCT Hemotology

QuestionAnswer
Hematopoietic system componenets Erythrocytes (RBC), Leukocytes (WBC), Platelets, Plasma
Red Blood Cells Contains hemoglobin and carries oxygen, biconcave in shape, accounts for 1-2% of red cells in blood.
White Blood Cells Primarily responsible for immune protection, Absolute WBC count often not highly specific of sensitive in the diagnosis of diseases.
WBC Differential (Percentage of different types of WBCs) can be more helpful in determining presence of active infection.
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.
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.
Anemia An absolute or relative decrease in the number of circulating, functional RBCs
Polycythemia/erythrocytosis An absolute or relative increase in the number of circulating RBCs
Thrombocytopenia An absolute or relative decrease in the number of circulating, functional platelets.
Thrombocytosis An absolute or relative increase in the number of circulating, functional platelets.
Leukopenia An absolute or relative decrease in the number of circulating, functional WBCs.
Leukocytosis An absolute or relative increase in the number of circulating functional WBCs.
Components of Hemostasis Vascular integrity platelet function Coagulation factors Fibrinolysis
Bleeding time measures time between skin incision and cessation of bleeding, normal= 3-8 minutes
Platelet Count Reported as part of complete blood count (CBC), normal= 150,000-400,000/mm.
Prothrombin Time (PT) Evaluates function of extrinsic pathway of coagulation cascade, normal control values= 11-13 seconds.
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.
INR also does what? Allows monitoring of anticoagulation effects if Coumadin.
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
Partial thromboplatin time (PTT) test all factors except VII and XIII, but used primarily to evaluate intrinsic pathway
PTT Normal value Normal= 25-35
Thrombin time Evaluates ability of fibrinogen (Factor I) to convert into a fibrin clot
Thrombin time normal value < 22 seconds, heparin therapy -inhibits fibrin formation
Heparin has no: Fibrinolytic capacity CAN BE REVERSED BY PROTAMINE SULFATE
tPA...? Tissue plasminogen activators (tPA, rtPA)
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
FFP...? Fresh Frozen Plasma
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
Platelets indicated for what? Indicated for pt's with bleeding due to thrombocytopenia or platelet dysfunction
Typical adult dose of a 6-unit pack of platelets will raise platelet count by about...? 50,000/mm3
Willebrands disease = Factor VIII containing vWF (Humate-P or Koate HS)
Jaundice caused by what... Buildup of bilirubin in circulatory system may also indicate increased hemolysis of RBCs
Anemia is what...? an absolute decrease in the number of circulating RBCs
Mayor function of RBC is to...? Transport oxygen
Anemia is classified into three casual categories: Increased RBC destruction Decreased RBC production Acute or chronic blood loss
Causes INTRINSIC or EXTRINSIC to RBC cell membrane INTRINSIC- Enzyme defects, Membrane abnormality, Hemoglobin abnormality EXTRINSIC- Immunologic, mechanical, environmental, abnormal sequestrations
Sickle Cell- Most common hemoglobinopathy encountered in american emergency departments, affects predominantly person of african ancestry
Sickle cell patho- Causes an interlocking with an adjacent hemoglobin chain in the deoxygenated state, leading to the "sickling" of the entire RBC
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
Sickle cells S&S: Pt's most often present with symptoms related to a vaso-occlusive crisis and associated sequelae
Sickle cell: Vaso-occlusive Crisis- Severe in extremities, back, joints, abdomen, or chest. Treatment is analgesics and fluid replacement if clinically dehydrated.
Hemolytic Crisis treatment: Similar to vaso-occlusive crisis but includes treatment for potential shock state, may require blood transfusion if symptomatically anemic.
Priaprism is what? Sustained, painful pathologic erection, occurs in 10-40% of male's with sickle cell, can result in impotence.
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
Acute Chest Syndrome accounts for... 25% of premature deaths in patients with sickle cell disease.
Acute Chest Syndrome Tx: No definitive therapy identified, supportive care -Hydration -Analgesia -Maintenance of oxygen and ventilation -Empiric antibiotics
Thrombocytopenia is what? Decreased production of platelets - Due to bone marrow suppression from variety of causes
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.
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%
Disseminated Intravascular Coagulation (DIC): -Relatively common acquired coagulopathy -Most often encountered in critical care setting
DIC potential causes: -Trauma (burns, crush injuries, TBI, etc) -Pregnancy complications -Sepsis -Transfusion of drug reaction -Envenomation -Heat stroke
DIC Out of control coagulation of fibrinolytic cascade within systemic circulation
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
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
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
Hemophilia A: Males with hemophilia A can have the following possible offspring -All daughters will be carriers -None of the sons will have hemophilia
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
Hemophilia B: Caused by genetic decrease in function of factor IX
Hemophilia B treatment:
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
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
Hernation Syndromes -Uncal herniation -Central herniation -Tonsillar herniation
Created by: robillard.james