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Heme Onc Step 1

QuestionAnswer
What types of cells does a myeloblast give rise to? neutrophils, basophils, eosinophils
What are two enzymes released from eosinophils? histaminase and arylsulfatase, help limit reaction following mast cell degranulation
What effects does bradykinin have? incr vasodilation, permeability, pain
What is the pathogenesis of Glanzmann's disease? absent GpIIb/IIIa recepts - no platelet aggregation w/ fibrinogen
Which diseases have abnormal ristocetin cofactor assay? Class vWD and Bernard-Soulier disease (absent GpIb receptors)
What causes Heinz bodies? Oxidation of ferrous to ferric iron leads to denature Hgb precipitation and membrane damage
What are some causes of nonmegaloblastic macrocytic anemia? liver disease, alcoholism, reticulocytosis, orotic aciduria, drugs: 5FU, AZT, Hydroxyurea
What is the pathogenesis of Anemia of Chronic Disease? liver synthesis and release of hepicidin; prevents release of Iron to Transferrin; decr Fe, TIBC, incr ferritin
What enzyme defect results in Acute Intermittent Porphyria? Porphobilinogen deaminase (uroporhyrinogen-I-synthase)
What causes ITP? anti-GpIIb/IIIa Ab
What causes TTP? deficiency of ADAMTS13 (vWFmetalloproteinase) decr degradation of vWF multimers
Where is EPO synthesized? renal cortex by interstitial cells in the peritubular capillary bed
Why do men have higher Hgb levels? testosterone stimulates erythropoiesis; also lack of cyclic bleeding
How do Heinz bodies form? peroxide oxidized Hgb which precipitates
What activates factor VII in the extrinsic coagulation system? tissue thromboplastin (factor III) which is released from injured tissue
What does the prothrombin complex consist of? Factor Xa, factor V, PF3 (phospholipid), Ca
What causes post-transfusion purpura? pt receives blood and has Ab against platelet specific antigens that are on the donor platelets
What are the clinical findings of TTP? fever, thrombyocytopenia, renal failure, microangiopathic hemolytic anemia, CNS deficits
If a mixing study does not correct prolonged PT or PTT, what is the most likely cause of disease? circulating antibodies causing immune destruction (most commonly anti-VIII post-partum)
What are the causes and clinical findings of primary fibrinolysis? open heart surgery and diffuse liver disease (decr alpha2-antiplasmin), prostatectomy (release of urokinase); severe bleeding with (+) FDPs and (-) D-dimer assay
What is involved in a major crossmatch? pt serum (check for Ab against foreign antigens on donor RBCs) added to donor RBCs
What type of blood products must an IgA deficient pt receive? blood or blood products that lack IgA (to prevent severe anaphylactic reaction due to IgE Ab)
What can protect a mother from developing Rh sensitization? ABO incompatibility (fetal RBCs will be destroyed immediately upon entering mother's circulation)
Created by: ash0403
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