Thrombotic Disorders Flashcards
(40 cards)
Confer a risk of venous/arterial thrombosis (a condition called antiphospholipid syndrome or APS)
Chronic antiphospholipid antibodies (APLAs)
Often accompany autoimmune connective tissue disorder
Chronic antiphospholipid antibodies (APLAs)
Caused by stem cell mutation that modifies membrane-anchored platelet activation suppressors
PNH
most common inherited thrombosis risk factor
FVL gene mutation
second most common inherited thrombophilia in patients with a personal and family history of deep vein thrombosis
Prothrombin G20210A gene mutation
Thrombosis is often associated with a combination of genetic defect, disease, and lifestyle influences
Thrombosis Double Hit
Comprise a family of immunoglobulins that bind protein-phospholipid complexes
Antiphospholipid Antibodies
Are sometimes called nonspecific inhibitors
Antiphospholipid Antibodies
the plasma protein most often bound to APLAs
B2-GPI
Most APLAs arise in response to a bacterial, viral, fungal, or parasitic infection or to treatment with one of a variety of drugs
Transient alloimmune APLAs
Part of the autoantibodies that arise in collagen vascular diseases, SLE, RA, scleroderma, and Sjogren’s syndrome
Autoimmune APLAs
Detection and confirmation of antiphospholipid antibodies
- Mixing study
- Lupus anticoagulant Test profile
- Dilute thromboplastin time (dtt)
Detection and confirmation of antiphospholipid antibodies
Important first step
Mixing study
Detection and confirmation of antiphospholipid antibodies
Con differentiate Lac from a factor deficiency
Mixing study
Detection and confirmation of antiphospholipid antibodies
In Lupus Anticoagulant Test Profile, what are the two commonly used test and both are required
A. Dilute Russel Viper Venom Time (DRVVT)
B. Silica-based PTT or Silica clot time (SCT)
Detection and confirmation of antiphospholipid antibodies
A confirmed positive result in one system is conclusive despite a negative result from the other
Lupus Anticoagulant Test Profile
Detection and confirmation of antiphospholipid antibodies
Also named tissue thromboplastin inhibitor (TTI)
Dilute thromboplastin time (DTT)
Review the Detection and confirmation of antiphospholipid antibodies
PTT initiates coagulation at factor XII
DRVV at factor X
DTT at factor VII
considered the more specific of the LAC assay.
DRVVT
uses low-phospholipid reagent.
DRVVT Screening
uses high-phospholipid reagent
DRVVT Confirmatory
In Factor V Leiden Mutation, there is A mutation in the factor V gene
substitutes glutamine for arginine at position 506 of the factor V molecule (FV R506Q)
named for the city it, Leiden mutation (or APC resistance)
factor V R506Q mutation
A guanine-to-adenine mutation at base 20210 of the 3’ untranslated region of the prothrombin gene has been associated with mildly elevated plasma prothrombin levels
Prothrombin G20210A