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HemeOnc Quiz 3
HemeOnc Quiz 3
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Graduate
12/07/2009

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Term
What platelet level is indicative of thrombocytopenia?
Definition
Less than 50,000/mL
Term
Which clotting pathway is fast and slow? What is the final results of both pathways?
Definition
Fast is the extrinsic, slow is the intrinsic. Final result of both is activation of prothrombin to thrombin which converts fibrinogen to fibrin threads..
Term
What is the most common cause of abnormal bleeding?
Definition
Thrombocytopenia
Term
Common causes of thrombocytopenia?
Definition
RT/chemo, chemicals, vital hepatitis, drugs, malignancy or idiopathic
Term
Which lab test measures the extrinsic and common pathway only for coagulation?
Definition
PT
Term
Which lab test is used to monitor heparin? Coumadin?
Definition
Heparin = PTT
Coumadin = PT
Term
Which lab test evaluates the intrinsic and common pathway?
Definition
PTT
Term
Which lab test evaluates platelet function?
Definition
BT - bleeding time
Term
What blood disorder is an autoimmune IgG disorder and is often preceded by a viral URI?
Definition
ITP
Term
How will ITP affect platelets, PT and PTT?
Definition
Platelets will be decreased, PT and PTT will be increased.
Term
Which blood disorder is seen with pregnancy, marrow transplant complication or drugs and has neurologic systems?
Definition
TTP
Term
How will TTP look on CBC. smear and coagulation tests?
Definition
It will appear as a hemolytic anemia and thrombocytopenia with schistocytes on smear. It will have normal coagulation tests.
Term
Which blood disorder presents similarly to TTP but does not have any neurologicl symptoms?
Definition
HUS
Term
Which blood disorder primarily affects kids and is associated with E. coli infection leading to bloody diarrhea, acute renal failure, thrombocytopenia and anemia?
Definition
HUS
Term
Which two blood disorders involve non-immunologic destruction of platelets?
Definition
TTP and HUS
Term
What are the two main distinguishable differences between TTP and HUS?
Definition
HUS is will have renal failure but no neurologic symptoms. TTP will not have renal failure but will have neurologic symptoms.
Term
Describe how DIC occurs.
Definition
Widespread intravascular damage leads to widespread intravascular fibrin formation which occludes small vessels and depletes platelets and coagulation factors leading to bleeding. The microvascular thromosis leads to organ failure and the depletion of coag factors leads to bleeding.
Term
What are some common causes of DIC?
Definition
septicemia, crush injury, viral infection, chemo, pregnancy, blood transfusion rxn, snake venom, burns and liver disease.
Term
How will DIC present on PT, PTT, fibrinogen, bleeding time and platelet count?
Definition
Increased PT and PTT. Decreased fibrinogen. Increased bleeding time and decreased platelet count.
Term
How do you treat DIC?
Definition
Treat the underlying cause and replace factors and platelets.
Term
What is the most common congenital coagulopathy?
Definition
vW disease
Term
What is the pathology of vW disease?
Definition
inadequate platelet aggregation and a defective factor 8.
Term
Symptoms of vW disease?
Definition
mucosal bleeding, easy bruising and menorrhagia.
Term
Do you have platelets with vW disease? What is the problem?
Definition
You have platlets but they cannot make a good plug due to factor absence
Term
How will vW disease present on PTT and bleeding time?
Definition
They will both be increased.
Term
Describe the deficiency seen with Hemophilia A and B.
Definition
A is a factor 8 deficiency. B is a factor 9 deficiency.
Term
Which pathway is affected in Hemophilia A?
Definition
Intrinsic pathway
Term
Which hemophilia is more mild?
Definition
B is more mild
Term
S&S of Hemophilia?
Definition
spontaneous bleeding, hemarthroses, intracranial bleeding, bleeding into soft tissues and gingiva.
Term
How will Hemophilia A affect PTT, PT, bleeding time and platelet levels?
Definition
PTT will be increased(Factor 8 is intrinsic), PT, bleeding time and platelet count are normal.
Term
How do you treat Hemophilia A? B?
Definition
A = IV heat treated factor 8 concentrate.
B = Fresh frozen plasma.
Term
What kind of acquired coagulopathy is seen with post-op patients that arent eating well and may be on broad spectrum antibiotics?
Definition
Vit K deficiency
Term
What disorder has been seen in a higher prevalence in Greek and northern European decent and leads to an increased risk of thromboembolic disease?
Definition
factor V Leiden
Term
How is Heparin delivered? What reverses the effects of heparin?
Definition
It is only administered IV and is reversed by protamine sulfate.
Term
What is the drug of choice for those at risk of thromboembolic disease during pregnancy?
Definition
Low Molecular Weight Heparin (LMWH)
Term
Which anticoagulant is started simultaneously with Heparin but takes 3-4 days for its effect to take place so they can go home with this drug?
Definition
Warfarin (Coumadin)
Term
How is Warfarin (Coumadin) OD reversed?
Definition
Vitamin K
Term
: excessive iron loading of the tissues as a result of genetic defect, liver disease or certain anemias.
Definition
Hemochromatosis
Term
S&S of hemochromatosis?
Definition
asymptomatic, fatigue, loss of libido, bronzing of the skin, diabetes, arthralgia, cardiac problems, cirrhosis and abdominal pain.
Term
How much iron in the body do you need to see symptoms with hemochromatosis?
Definition
7-8 grams
Term
What is the gold standard test for hemochromatosis?
Definition
genetic testing for HFE1 gene
Term
Hemochromatosis Tx?
Definition
Phlebotomy, chelation (removes heavy metals from the body) and avoidance of iron in diet.
Term
Complication of hemochromatosis?
Definition
Diabetes, cirrhosis, liver cancer, pancreatic damage and cardiac complications.
Term
What levels are indicative of lead poisoning for children and adults?
Definition
Children = above 50 ug/dL.
Adults = above 80-100 uf/dL.
Term
Explain the pathophys of lead poisoning on the blood.
Definition
It inhibits 3 enzymes (PBG synthase, heme synthase and coproporphyrinogen oxidase) which injures the RBC membrane and inhibits the synthesis of the alpha and beta globin chains, making them defective.
Term
What is pathopneumonic of lead poisoning on peripheral smear?
Definition
ringed sideroblasts (RBCs that are ringed and have ferritin deposits in them) and RBC stippling (small dots at the periphery)
Term
What are some S&S of lead poisoning in children and adults?
Definition
Children = speech and learning deficits.
Adults = blue-black line on gingiva, GI distress, motor neuropathies, CNS damage and fatigue.
Term
:caused by inherited defects in the heme biosynthetic pathway resulting in the excess production of porphyrin precursors.
Definition
Porphyria
Term
What are the common symptoms of all porphyria?
Definition
photosensitivity reactions and nervous system dysfunction
Term
What are the S&S of the two main types of Porphyria, PCT (porphyria cutanea tarda) and EPP (erythropoietic porphyria)?
Definition
PCT = bullous eruptions, plaquelike scar formation, hyperpigmentation and excess hair to face.
EPP = childhood onset, itching, burning, erythema, swelling of the exposed skin areas.
Term
How is Porphyria diagnosed?
Definition
measure the levels of porphyria precursors in the urine and stool.
Term
:Hepatolenticular degeneration due to an autosomal recessive disorder leading to excess amounts of copper accumulating in the brain, liver, kidneys and cornea due to low levels of ceruloplasmin.
Definition
Wilson's disease
Term
What are the 3 ways that Wilson's disease will typically present?
Definition
Intravascular hemolytic anemia, hepatic dysfunction or neuropsychiatic illness.
Term
What are some S&S of Wilson's disease?
Definition
hepatomegaly, elevated LFTs, fatty liver, and decreased serum ceruloplasmin.
Term
What is pathopneumonic of Wilson's disease on physical exam?
Definition
Kayser-Fleischer rings on slit lamp (brown rings at corneal margins)
Term
Treatment for Wilson's disease?
Definition
eliminate copper from diet and chelation therapy (removal of heavy metals from the body)
Term
At what level of blood volume loss is heart failure and death imminent without volume replacement?
Definition
50%
Term
Describe the pathology of hypovolemic shock.
Definition
RBC and volume loss leads to a loss of tissue perfusion which leads to tissue hypoxia which leads to hypovolemic shock.
Term
Which way is the oxygen dissociation curve shifted with acidosis?
Definition
Acidosis shifts to the right (favors O2 unloading to the tissues)
Term
Replacement of what is necessary to maintain blood homeostasis in a massive bleed?
Definition
platelets and coagulation factors
Term
What are the normal components of normal saline solution?
Definition
Just NaCl
Term
Components of diarrheal Tx solution?
Definition
NaCl, K and lactate/acetate
Term
Components of Ringers solution?
Definition
NaCl, K, Ca, and lactate/acetate
Term
What kind of solution is preferred to manage large volume losses? Why?
Definition
Colloid solutions because they contain proteins.
Term
What are the advantages of giving FFP to someone with a massive bleed?
Definition
It contains coagulation factors
Term
What are the components of whole blood transfusions?
Definition
Whole blood cells and coagulation factors minus platelets.
Term
What kind of blood transfusion is commonly used to treat most anemias?
Definition
PRBCs (packed red blood cells)
Term
Do PRBCs contain WBCs or platelets?
Definition
Yes they do, but leukodepleted packed RBCs do not.
Term
What kind of blood transfusion is used commonly in those with transplants, malignancies and aplastic anemias?
Definition
Leukodepleted RBC transfusion
Term
What are Washed RBCs (Frozen) used for?
Definition
for long term storage of those with rare blood types
Term
What are Washed RBCs (Irradiated red cells) used for?
Definition
for immunocompromised patients
Term
What are Washed RBCs (CMV-negative cells) used for?
Definition
for CMV negative recipients having bone marrow transplants
Term
How much will each unit of PRBCs increase Hgb and Hct by?
Definition
Hgb by 1g/dL and Hct by 3%.
Term
What are the two examples of blood transfusions that are used to replace coagulation factors?
Definition
FFP and Cryoprecipitate
Term
What are the indications for FFP transfusion?
Definition
massive blood loss, emergency reversal of warfarin, DIC, liver disease and inherited coagulopathies
Term
What are the indications for cryoprecipitate transfusion?
Definition
factor 13 deficiency and fibrinogen replacement
Term
With repeated transfusions, what can develop that will shorten the lifespan of transfused platelets?
Definition
HLA alloantibodies
Term
What are some S&S of a transfusion reaction?
Definition
fever/chills, chest and low back pain, hypotension and a feeling of impending doom.
Term
What should you do if you suspect a blood transfusion reaction?
Definition
discontinue the transfusion stat and draw a venous blood sample stat.
Term
Describe the antigens and antibodies found with the ABO blood grouping system.
Definition
Type A = person has A antigen and B antibodies.
Type B = person has B antigen and A antibodies.
Type AB = person has A and B antigens and no antibodies.
Type O = person has no antigens and A and B antibodies.
Term
Which blood type is the universal donor and recipient?
Definition
Donor = Type O.
Recipient = Type AB.
Term
Describe the Rh system and how it can be fatal to newborns.
Definition
If an Rh negative mother has an Rh positive baby and she forms antibodies against the blood of her fetus during labor as the placenta detaches from the uterus. When she has another pregnancy, if that baby is Rh positive, the antibodies that she made from her first pregnancy will cross the placenta and cause hemolysis of the fetal blood and the baby will die.
Term
What is given before and shortly after birth to prevent a Rh negative mother from forming Rh antibodies during delivery?
Definition
RhoGAM
Term
What are the 3 most common etiologies of TTP?
Definition
pregnancy, bone marrow transplant and drugs.
Term
Is factor 8 part of the intrinsic or extrinsic pathway?
Definition
Intrinsic
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