Coagulation disorders

Question 1

What factors are in the intrinsic pathway?

Answer 1

8, 9, 11, 12

Question 2

What factors are in the extrinsic pathway?

Answer 2

TF (3), 7

Question 3

What type of clotting disorder can have some symptoms that appear as nose bleeds, easy bruising and bleeding gums, postop bleeding, heavy menstrual bleeding, endometriosis, miscarriages, and potentially postpartum hemorrhages as the most common inherited bleeding disorder?

Answer 3

Von Willebrand disease

Question 4

What do these labs indicate:
Normal PT, prolonged aPTT
BT: long
PFA-100: prolonged?

Answer 4

Von Willebrand disease

Question 5

How do you treat Von Willebrand disease?

Answer 5

DDVAP

Question 6

What type of clotting disorder involves bleeding episodes primarily around the ankle, knee, elbow, as well as retroperitoneal, intramuscular, or intracranial bleeding and is an X-linked recessive disorder?

Answer 6

hemophilia

Question 7

What are the two types of hemophilia and their subsequent factor deficiencies?

Answer 7

a - VIII “classic”
b - IX “christmas”

Question 8

How can you differentiate between types of hemophilia?

Answer 8

measure clotting factors

Question 9

What do these labs indicate:

Normal PT, prolonged aPTT
BT normal?

Answer 9

hemophilia

Question 10

How do you manage hemophilia?

Answer 10

replace missing clotting factors – recommend monitoring at treatment center

Question 11

What type of clotting disorder involves increased incidence of DVT, PE, hepatic/cerebral vein thrombosis, associated w/ miscarriage, preeclampsia, placental abruption, and stillbirth and is from a point mutation in G1691A factor V gene?

Answer 11

Factor V leiden disorder

Question 12

What do these labs indicate:

Normal PT and PTT
Activated protein C resistance assay
confirmed w/ diagnostic testing?

Answer 12

factor V leiden disorder

Question 13

How do you treat factor V leiden disorder?

Answer 13

high risk - indefinite anticoagulation (warfarin)

moderate - consider prophylaxis during procedures only

Question 14

What type of clotting disorder involves platelet counts dropping at least 50% 5-10 days after treatment with heparin, increasing risk of thrombosis, thrombocytopenia, and DVT?

Answer 14

heparin-induced thrombocytopenia

Question 15

What causes heparin-induced thrombocytopenia?

Answer 15

immune mediated RBC destruction against platelet factor 4 + heparin complex, activating platelets

Question 16

How can you diagnose heparin-induced thrombocytopenia?

Answer 16

ELISA for heparin/PF4 complex antibodies OR funcitonal assay

Question 17

What are the 4Ts of heparin-induced thrombocytopenia?

Answer 17

thrombocytopenia, timing, thrombosis, absence of other causes

Question 18

How do you manage heparin-induced thrombocytopenia?

Answer 18

stop heparin products + agatroban

always check for DVTs, and do NOT give platelet transfusion

Question 19

What bleeding disorder should you consider with bleeding tendencies - blood very thin, hematuria, blood in stool…widespread bleeding throughout?

Answer 19

disseminated intravascular coagulation (DIC)

Question 20

What do these labs indicate:
CRC>2%
BT: high
Hgb: low
platelets: low
Prolonged PT and aPTT
Elevated FDP/D-dimer
fibrogen: low?

Answer 20

disseminated intravascular coagulation (DIC)

Question 21

How can you manage disseminated intravascular coagulation?

Answer 21

supportive w/ platelet transfusion, FFP, crypprecipitate to replace fibrinogen

Question 22

What bleeding disorder is defined by the trio of 1) renal injury, 2) anemia and 3) thrombocytopenia withOUT neuro symptoms or a fever and can be from toxins, infections, or drugs?

Answer 22

hemolytic uremic syndrome

Question 23

What do these labs indicate:
blood smear: shistocytes
Platelets: low
PT, aPTT, fibrinogen: normal
BUN + creatinine: high
+ shiga toxin ?

Answer 23

hemolytic uremic syndrome

Question 24

How do you treat hemolytic uremic syndrome?

Answer 24

plasma exchange and aggressive antibiotic therapy

Question 25

What the heck is factor XI deficiency?

Answer 25

Hemophilia C, common in Ashkezansi Jewish population, and with MILD bleeding

Question 26

What is defined by the following 5 symptoms: fever, renal failure, neurologica findings, thrombocytopenia w/ mucocutaneous bleeding, and microangiopathic hemolytic anemia (MAHA) like splenomegaly?

Answer 26

thrombotic thrombocytopenic purpura

Question 27

What can help you remember thrombotic thrombocytopenic purpura?

Answer 27

FAT RN
fever
anemia
thrombocytopenia
renal
neuro

Question 28

What disease revolves around ADAMTS13 deficiency where th Von Willebrand does not separate and instead bursts cells, and can be caused by quite a few medications?

Answer 28

thrombotic thrombocytopenic purpura

Question 29

What do these lab values indicate:
CRC>2
blood smear: schistocytes
AST: elevated
LDH: elevated
Haptoglobin: low
bilirubin: high
platelets: low
PT, PTT, fibrinogen: normal
ADAMTS13: low

Answer 29

thrombotic thrombocytopenic purpura

Question 30

How do you treat thrombotic thrombocytopenic purpura?

Answer 30

plasma exchange and monitoring CBC< creatinine, LDH, RDC daily + retuximab may reduce relapse

Question 31

What is defined by “wet” bleeding and NOT with splenomegaly, and could be induced by a viral illness??

Answer 31

idiopathic thrombocytopenia purpura

Question 32

What does this PE and lab values indicate:
- NO splenomegaly
normal CBC
smear: large platelets, NO shistocytes
platelets: low
bone marrow: megakaryocytes?

Answer 32

idiopathic thrombocytopenia purpura

Question 33

How do you manage idiopathic thrombocytopenia purpura?

Answer 33

1) corticosteroids
2) IVIG/anti-D immunoglobulins
3) rituximab
4) prevent recurrence: splenectomy
transfusion in life-threatening situations!