Heme Onc

Question 1

What is are the molecules responsible for primary hemostasis?

Answer 1

vWF released from endothelium which binds G1b in a process called adhesion allowing fibrinogen to link platelets via glycoprotein IIb/IIIa through a process called aggregation

Question 2

What occurs in seconodary hemostasis?

Answer 2

Clotting factors (intrinsic and extrinsic) turn prothrombin to thrombin which activates fibrinogen leading to fibrin mesh

Question 3

Intrinsic and extrinsic pathways

Answer 3

• Intrinsic: factors 8-12 but not 10
• Extrinsic: Exclusively 7

Question 4

How do you know the difference between a bleeding disiorder of primary or secondary hemostasis?

Answer 4

• Deep bleeding like hemarthrosis, hematomas, and prolonged bleeding are usually seen as disorders of secondary hemostasis
• Superficial bleeding like epistaxis, gingival bleeding, and menorrhagia is seen as disorders of primary

Question 5

What does PT, PTT, bleeding time, mixing studies, vWF, D dimer tests tell us?

Answer 5

• PT: Extrinsic pathway
• PTT: Intrinsic pathway
• Bleeding time: Plug formation
• Factor levels: Direct measure of factors
• Mixing study: Difference between study
• vWF: direct measure
• D dimer: indirect measure of fibrinolysis

Question 6

If you suspect a disorder of primary hemostasis (superficial bleeding) then what do you do? What does this study show?

Answer 6

• Platelet count studies
• If decreased, check smear
  
  • Decreased production in aplastic anemia
  • Sequestration
  • Increased destruction in ITP, TTP, DIC
• If normal: It is a platelet fixation issue
  
  • vWD
  • Glanzmanns
  • Bernard Soulier
  • Uremia
  • Drugs

Question 7

What do you check if you suspect deep bleeding and a disorder of secondary hemostasis?

Answer 7

Factor studies, PTT, PT, INR mixing study

• Positive mixing study
  
  • Factor deficiency: vWD, Hemophilia A and B
• Acquired disease: Vit K deficiency, liver disease, DIC

Question 8

What occurs in DIC? What labs will we see?

Answer 8

Significant systemic disease leads to chewing up clotting factors and platelets leading to no platelets and clotting factors. Labs will show increasd PT, PTT, decreased fibrinogen and positive D dimers

Question 9

What are some things that lead to vitamin K deficiency?

Answer 9

Antibiotics killing intestinal bacteria or warfarin

Question 10

Type A and B hemophilia invove what clotting factors? How is it treated?

Answer 10

8A and 9B. Transfuse factor 8 only when patient is actively bleeding

Question 11

How do you treat von willebrand disease

Answer 11

Desmopressin to increase vWF if severe give ccryoprecipitate or factor 8 acutely

Question 12

What is the differential for thrombocytopenia?

Answer 12

TTP, HIT, DIC, ITP

Question 13

What are the lab values seen in anemia of chronic disease?

Answer 13

Decreased TIBC (no available storage), Decreased Fe (in blood), increased ferritin (increased storage)

Question 14

What are the labs seen in Iron deficiency anemia

Answer 14

Decreased Fe in blood, increased TIBC, decreased ferritin stores

Question 15

What is the difference between alpha and beta thal? What are the labs we would expect?

Answer 15

Alpha is seen with 2 or 3 genes deleted and beta is seen with 1 or 2. Normal iron studies with anemia

Question 16

How is thalassemia treated?

Answer 16

Major requires blood transfusion

Question 17

What is the cause of sideroblastic anemia? What are the labs we would expect?

Answer 17

• Elevated iron and microcytic anemia. Blood smear shows ringed sideroblasts.
• Caused by
  
  • Lead
  • EtOH
  • Isoniazid
  • Myelodysplasia/AML

Question 18

What is a possible side effect of frequent transfusion and how do you treat it?

Answer 18

Iron overload treated by deferoxamine

Question 19

What are the labs that would clue you in to a folate deficiency? B12?

Answer 19

• Folate: decreased folate and elevated homocysteine and normal MMA
• B12: Decreased B12 with elevated homocysteine and elevated MMA

Question 20

What does megaloblastic mean?

Answer 20

Multilobed nuclei. Production DNA issue leading to large, multilobed cells

Question 21

What labs make us suspect hemolysis?

Answer 21

• Decreased haptoglobin
• Increased bilirubin
• Increased LDH
• Retic count

Question 22

What is the thing you should worry about if you see a normocytic anemia of decreased reticulocyte count

Answer 22

Cancer crowding out the cells that produce RBCs

Question 23

What isi the pentad for TTP?

Answer 23

FAT RN

• F: Fever
• A: Anemia
• T: Thrombocytopenia
• R: Renal failure
• N: Neurologic symptoms

Question 24

What is the treatment for TTP

Answer 24

Never give platelets, but do give plassma exchange or a plasma transfusion with ADAMTS-13 only

Question 25

What is the treatment for heparin induced thrombocytopenia

Answer 25

Argatroban bridge to coumadin long term

Question 26

ABVD

Answer 26

• Adriamycin/Doxorubicin
• Bleomycin
• Vinblastine
• Dacarbazine