Coagulation Flashcards
Amyloidosis - Factor, test
X (decreased) Thrombin time (increased)
Conditions affecting vWF levels
Increased: Acute phase reaction, OCPs, pregnancy, neonates
Decreased: Type O blood group
Protein S
Carrier of protein C.
In turn 60% bound to C4BP.
Reptilase time
Snake venom (Batroxobin) from Bothrops snake»_space; Directly cleaves fibrinogen. Unlike thrombin time, not sensitive to heparin.
Time-dependent prolongation on mixing study
fVIII inhibitor
Platelet aggregation - Phases
1st phase: ADP release
2nd phase: More ADP release, TXA2 release.
Bernard-Soulier
CD42 (Gp1b/V/X)»_space; Macrothrombocytopenia
Impaired aggregation with ristocetin
Decreased response to thrombin-induced aggregation
Glanzmann’s thrombasthenia
GPIIb (CD41) / IIIa (CD61) abnormality. Normal platelet count.
No aggregation except with ristocetin.
Alpha-granule deficiencies
Gray platelet (retain P-selectin) Quebec platelet (excess u-PA degrades granule) Paris-Trousseau
Dense-granule deficiencies
Chediak-Higashi
Wiskott-Aldrich
Hermansky-Pudlak
Storage pool deficiencies - Testing
No second wave on aggregation.
Diminished responses to collagen, arachidonic acid
Note: Both of above also true of aspirin effect
vWD - I, III
I - Autosomal dominant. Mild, treatable with DDAVP. Normal multimers.
III - Autosomal recessive. Total absence.
vWD - 2A, 2B, plt-type
2A - Most common after I. Very few large multimers. Low activity:antigen ratio. Acquired forms resemble this.
2B - Rare. Exon 28 abnormality»_space; increased Gp1b affinity. Few large multimers. Abnormal RIPA. DDAVP will cause thrombocytopenia.
Plt-type - Gp1b on platelet has higher affinity for vWF. Abnormal RIPA. Fewer multimers. Distinguish with cryo challenge
vWD - 2N, 2M, Plt-type
2N - Abnormal fVIII binding site, presents like Hemophilia A.
2M - Decreased Gp1b binding affinity. Very low activity.
Hemophilia A - Congenital & acquired
Congenital: Usually intron 22 inversion. XLD, can be seen in women in Turner, homozyg, lyonization
Acquired: Rare, elderly with new bruising
Minor hemophilias
Hemophilia C (factor XI, jews, autosomal. No recombinant available) fIX (anaphylaxis with repletion) f12 (maybe thrombogenic?) f7 (maybe also thrombogenic?) f13
Dysfibrinogenemias
Qualitative dysfunction, only partially corrects on mixing due to competitive inhibition.
Diagnose with thrombin time (more sensitive than reptilase time)
Alpha-2 antiplasmin deficiency
Test: SLOW euglobulin clot lysis. Normal other coags.
TTP/HUS
Normal PT, PTT, fibrinogen, D-dimer. Platelet-rich clots.
Heparin resistance
AT-III deficiency
Increased heparin clearance or binding
Homocysteinemia
Endothelial dysfunction
Lens dislocation, peripheral neuropathy, maybe low folate?
Hypercoagulability of pregnancy
General increase in all factors
Decrease in protein S
APC resistance
Venous stasis
Platelet collagen receptors
GPVI (major, plays signaling role, deficiency in Japanese) Integrin a2b1 (GpIa/IIa)
Wiskott-Aldrich
XLD
Thrombocytopenia, eczema, immunodeficiency
Cytoskeletal abnormality, impaired signal transduction
Low IgM, high IgE and IgA
VASP
Cone & Plate analysis
VASP: Flow-based method, gold-standard for P2Y12 inhibitor effect.
Cone & Plate: In vitro method studying shear-induced adhesion. Affected by Hct, plt count, meh,.
May-Hegglin
Chediak-Higashi
Fechtner
May-Hegglin - Dohle-like bodies in neutrophils. MYH9
Chediak-Higashi - Peroxidase+ granules in many leukocytes
Fechtner - May Hegglin + Alport syndrome
Congenital amegakaryocytosis
MPL gene mutation
Specimen types in coag testing
Most: Citrated plasma (3.2% NaCit)
Flow: Whole blood
Plt agg: Platelet rich and poor plasma (still collect in blue top)
PFA-100: Patterns
ASA: Col/Epi prolonged, Col/ADP normal
Plavix: Variable, both may be prolonged
BSS, GT: Both prolonged