Condition\Diseases Flashcards
Hemolytic transfusion reactions summary
Def: ABO incompability due to human error
SS:
- fever
- flank pain
- red/brown urine
Txt: medical emergency stop transfusion saline 100-200 ml/hr Vasopressors
Febrile nonhemolytic transfusion summary
Def: reaction due to interleukins and TNF alpha substance
SS: Fever Chills Rigors Mild Dyspnea
Txt:
stop transfusion
Tylenol/benadryl
symptomatic
Delayed Hemolytic Transfusion summary
Def: reaction caused by atypical antibody usually 2-10 days after
SS: slight fever falling hematocrit mild increase unconjugated bilirubin mild increase spherocytes
Txt:
None for this transfusion
future trans: antigen negative blood products
Disorders of primary hemostasis
Platelet: quantitative (thrombocytopenia) and qualitatie (functional defects)
Von Willebrand Disease
Disorders of secondary hemostasis
Congenital: hemophilia A and B, rare factor deficiences
Acquired: Vit. K deficiency, liver disease
von Willebrand disease
Most common inherited bleeding disorder
Def: mutations in the gene coding for von willebrand factor results in dysfunction of platelet adhesion or the dysfunction of carrying of factor VIII and not protecting it
S/S: epistaxis, dental bleeding, menorrhagia, bruising, prolonged bleeding (primary hemostasis), severly affected pts may have joint and soft tissue hemorrhages
Dgx: vW panel: low vW antigen, low factor VIII, low ristocetin cofactor activity (protein doesn’t func); other tests: abnormal platelet func testing, NORMAL INR, aPTT, and platelet count
Tx: avoid drugs that inhibit plt func, DDAVP, antifibrinolytics, plasma derived VIII/vWF, cryoprecipitate
Hemophilia
Def: congenital bleeding disorder caused by deficiency of coag factor VIII (type A, classic) or IX (type B, Christmas) (both located on X chromosome)
S/s: clinical phenotypes are indistinguishable; severity of bleeding depends on the factor level; joint, soft tissue, deep muscle, and intracranial bleeding
Dgx: Markedly prolonged PTT, NORMAL INR and plt count and func. measure specific factor levels
Tx: avoid drugs that impair plt func, factor VIII or IX concentrates, prophylactic factor replacement
Vitamin K deficiency
Required for the synthesis of coag factors II, VII, IX, and X and protein C and S.
Infants: doesn’t cross placenta, low levels in breast milk, low amount of colonic bacteria
Adults: malnutrition, impaired malabsorption, antibiotics
Liver disease
associated with defects in primary and secondary hemostasis and in fibrinolytic system
Thrombocytopenia, decreased synthesis of clotting factors, dysfunctional fibrinogen, excess fibrinolysis
Can cause anticoagulation or coagulation problems because it contributes to both but the fulcrum of the scale is small so it could tip in any direction
Venous thromboembolism
occurs in veins
Dx: D-dimer, compression US, v/Q perfusion, CT scan
S/S: pain, swelling, tenderness, discoloration, warmth, asymmetry, dyspnea, chest pain, angina, hemoptysis, cough
Genetic thrombophilia
Mechanisms of thrombosis: failure to control thrombin generation (factor V Leiden, prothrombin gene mutation, protein C and S deficiency)
impaired neutralization of thrombin (anti-thrombin deficiency)
Genes + Triggers = clot
Everyone has a thrombosis threshold and genes, triggers, and age bring you closer to that threshold until you hit it and clot
Tx: heparin, life long anti coag
Anaphylactic Transfusion Reaction Summary
Def: body develops anaphylactic reaction to transfusion blood products
SS: shock hypotension angioedema respiratory distress
Txt: stop transfusion epinephrine maintain airway saline vasopressors
Urticarial Transfusion Reaction Summary
Def: blood products cause histamine release from mast cells and basophils causing hives and urticaria
SS:
hives/urticaria
Txt:
benadryl
continue transfusion
Post Transfusion Purpura Summary
Def: sensitazation to foreign antigen from previous platelet transfusion. Very rare. MC - HPA-1a
SS:
severe thrombocytopenia 5 - 10 days post transfusion
Lasts days to weeks
Txt:
high dose corticosteroids
exchange transfusion
subsequent transfusions = washed cells or HPA-1a negative cells
Transfusion Related Acute Lung Injury Summary
Def: Rare. Believed to be immune response in pulmonary
SS: sudden onset respiratory distress during or after transfusion Fever tachypnea tachycardia HOTN
Txt:
supportive
mechanical ventilation
Comps with RH- Preggers
Def:
- Mother Rh-
- Has baby1 who is Rh+ and baby1 Rh+ blood get into Mom bloodstream.
- Nothing bad happen to baby1. Mother now has Rh antibodies in her system
- Mom get preg again. If have Rh+ baby, her body attacks the fetus and tries to kill it.
Txt:
300 mcg IM Rhogam/fetal blood 15cc during and after preg
Splemomegaly Summary
Def: Enlarged spleen
Cause: Liver dx Hematologic malignancy Infection CML Lymphoma Parasitic infection Gaucher dx
