Coagulation disorders Flashcards
What factors are in the intrinsic pathway?
8, 9, 11, 12
What factors are in the extrinsic pathway?
TF (3), 7
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?
Von Willebrand disease
What do these labs indicate:
Normal PT, prolonged aPTT
BT: long
PFA-100: prolonged?
Von Willebrand disease
How do you treat Von Willebrand disease?
DDVAP
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?
hemophilia
What are the two types of hemophilia and their subsequent factor deficiencies?
a - VIII “classic”
b - IX “christmas”
How can you differentiate between types of hemophilia?
measure clotting factors
What do these labs indicate:
Normal PT, prolonged aPTT
BT normal?
hemophilia
How do you manage hemophilia?
replace missing clotting factors – recommend monitoring at treatment center
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?
Factor V leiden disorder
What do these labs indicate:
Normal PT and PTT
Activated protein C resistance assay
confirmed w/ diagnostic testing?
factor V leiden disorder
How do you treat factor V leiden disorder?
high risk - indefinite anticoagulation (warfarin)
moderate - consider prophylaxis during procedures only
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?
heparin-induced thrombocytopenia
What causes heparin-induced thrombocytopenia?
immune mediated RBC destruction against platelet factor 4 + heparin complex, activating platelets
How can you diagnose heparin-induced thrombocytopenia?
ELISA for heparin/PF4 complex antibodies OR funcitonal assay
What are the 4Ts of heparin-induced thrombocytopenia?
thrombocytopenia, timing, thrombosis, absence of other causes
How do you manage heparin-induced thrombocytopenia?
stop heparin products + agatroban
always check for DVTs, and do NOT give platelet transfusion
What bleeding disorder should you consider with bleeding tendencies - blood very thin, hematuria, blood in stool…widespread bleeding throughout?
disseminated intravascular coagulation (DIC)
What do these labs indicate:
CRC>2%
BT: high
Hgb: low
platelets: low
Prolonged PT and aPTT
Elevated FDP/D-dimer
fibrogen: low?
disseminated intravascular coagulation (DIC)
How can you manage disseminated intravascular coagulation?
supportive w/ platelet transfusion, FFP, crypprecipitate to replace fibrinogen
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?
hemolytic uremic syndrome
What do these labs indicate:
blood smear: shistocytes
Platelets: low
PT, aPTT, fibrinogen: normal
BUN + creatinine: high
+ shiga toxin ?
hemolytic uremic syndrome
How do you treat hemolytic uremic syndrome?
plasma exchange and aggressive antibiotic therapy
What the heck is factor XI deficiency?
Hemophilia C, common in Ashkezansi Jewish population, and with MILD bleeding
What is defined by the following 5 symptoms: fever, renal failure, neurologica findings, thrombocytopenia w/ mucocutaneous bleeding, and microangiopathic hemolytic anemia (MAHA) like splenomegaly?
thrombotic thrombocytopenic purpura
What can help you remember thrombotic thrombocytopenic purpura?
FAT RN
fever
anemia
thrombocytopenia
renal
neuro
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?
thrombotic thrombocytopenic purpura
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
thrombotic thrombocytopenic purpura
How do you treat thrombotic thrombocytopenic purpura?
plasma exchange and monitoring CBC< creatinine, LDH, RDC daily + retuximab may reduce relapse
What is defined by “wet” bleeding and NOT with splenomegaly, and could be induced by a viral illness??
idiopathic thrombocytopenia purpura
What does this PE and lab values indicate:
- NO splenomegaly
normal CBC
smear: large platelets, NO shistocytes
platelets: low
bone marrow: megakaryocytes?
idiopathic thrombocytopenia purpura
How do you manage idiopathic thrombocytopenia purpura?
1) corticosteroids
2) IVIG/anti-D immunoglobulins
3) rituximab
4) prevent recurrence: splenectomy
transfusion in life-threatening situations!
