Coagulophathies Flashcards
Coagulopathy categories?
Clotting
Platelet
Hypercoag
Clotting disorders? (2)
Hemophilia A (factor 8) Hemo B (factor 9)
Petechiae?
< 2mm
Focal SQ bleed
Don’t blanch
Purpura?
2-10mm
Palpable
Don’t blanch
Ecchymoses?
> 1cm
Initial labs for bleeding disorder? (4)
CBC w/ platelet count
Periphial smear
PT/INR, aPTT
+/- bleed time
Platelets levels:
thrombocytopenia?
prolonged bleed from injury?
spontaneous bleed?
< 100k
40k
<20k
Bleed time measures?
Screens for?
Prolonged if?
Time for hemostasis
Microvasculature and platelet fxn
Platelet disorder
Hemostatic response to vessel injury?
1st vasospasm
2nd platelet aggregation
3rd coag cascade
Extrinsic Pathway includes what Factors?
Requires what for activation?
What test measure Extrinsic path?
What med interrupts Extrinsic path?
Factor VII
Tissue Factor
PT (also measures Common)
Warfarin
Intrinsic Pathway includes what Factors?
Requires what for activation?
What test measures Intrinsic path?
What med interrupts Intrinsic path?
VIII, IX, XI, XII
Injury
PTT (also measures Common)
Heparin
Common Pathway includes what Factors?
What test measures Common path?
What med interrupts Common path?
I (fibrinogen), II (prothromin), V, X
PTT and PT
Lovanox
Prothrombin Time (PT) monitors what Path?
What meds?
What conditions? (2)
Extrinsic path
Warfarin
Liver fxn/damage
DIC
Partial Thromboplastin Time (PTT) or Activate Partial (aPTT) monitor what Path?
What meds?
What conditions? (5)
Intrinsic
Unfractionated Heparin (NOT LMWH)
Hemophilia, vW, liver damage, Vit K def, DIC
International Normalized Ratio (INR) monitors what?
Same as PT but more specific measures dependent on condition
INR target ranges:
DVT prophy?
DVT (acute), VTE, AFib?
Heart valves?
1.5 - 2
2 -3
2.5 - 3.5
PTT mixing study tests what?
Coag factor deficiency vs inhibitor problem
Mix pt blood w/ blood w/ normal factors,
If corrects, then pt missing factors
If not, pt has inhibitor problem
Thrombin Time (TT) tests what?
Fibrinogen
Clotting Disorders are?
Congenital or acquired
Excess or repetitive bleed at UNUSUAL sites w/ normal activity
U joints, mm, GI, GU
Hemophilia A/B details?
A = VIII (most common) B = IX (Christmas)
X-linked recessive
M»_space; F
Hemophilia A/B presentation?
Asympt until 1st bleed U by 2yo
Severe pain in weight-bearing joints
Hemo A tests?
Platelet count = N PT = N aPTT = long but corrects w/ PTT mix study Factor VIII assay
Platelet Disorders: Low
Questions? (5)
Sustained ↓? Recent infection? Meds that ↓ platelets? Bleed/Not bleed OFP to platelets? Evidence of: -platelet consumption synd? -other autoimm? -chronic liver dz?
Platelet Disorders: High
Questions? (6)
Cause of significant inflamm? Current infection? Peripheral smear abnormal? Fe def anemia? Inapprop clotting/bleeding? Signs of: -essential thrombocytosis? -CA
Platelet Disorders: Dysfxn
Caused by? (2)
Congenital (rare)
Acquired (drugs/EtOH, uremia, myelopro dz)
Platelet Disorders: Dysfxn Lab tests?
Count = N
Morphology = N
Bleed Time = long
Platelet Disorders: Splenic Sequestration?
Hyper/Megaly = cytopenia Ectomy = cytosis
Idiopathic Thrombocytopenic Purpura (ITP) result of? (3)
Acute?
Chronic?
Platelet destruction
↓ prodxn
Sequestration
A = U kids post URI,
Heparin (most C inpt cause)
C = a/w autoimm, HIV, 20-50yo
ITP presentation? (3)
Petechia, purpura, bullae on skin/muc memb
Epistaxis, oral bleeding
Subarachnoid/Intracranial hemorrhage
ITP labs? (2)
Coag studies = N Platelet count = ↓↓
ITP tx? (4)
No NSAIDs/ASA
Prednisone
IV IG
Platelet transfusion
DIC (Disseminated Intravascular Coag) is?
a/w?
Widespread coag ->
massive platelet, fibrin, factor consumption ->
uncontrolled bleeding
underlying systemic illness
Illness a/w DIC? (4)
Sepsis
Trauma
CA
Preggo complications
MOST ????? Malaria Osteomyelitis Sepsis Trauma
DIC presentation?
Variable onset
Multi organ systems
Hemorr
DIC labs? (6)
Platelets = ↓ Fibrinogen = ↓ Fibrinolysis = ↑ PT/PTT = ↑ D-dimer = ↑ Smear = schistocytes
DIC tx? (4)
Transfusion w/: Cryoprecipitate (fibrinogen replacement) FFP (coag factors) Platelets PRBCs
Thrombotic Thrompocytopenia Purpura (TTP) is?
Caused by? (4)
Rare, U fatal
Microangiopathic hemolytic anemia + thrombocytopenia ->
endothelial injury/small vessel thrombi
E. coli O157:H7
CA
Preggo
Drugs
TTP common in who?
20-50yo
F>M
+HIV
TTP presentation? (6)
Toxic Abd pain HA Mental ∆s Pallor, petechiae Jaundice
Hemolytic Uremic Synd is?
Presentation?
Labs? (4)
Tx?
U from e. coli O157:H7
Abd pain w/ bloody D days prior
Like TTP less CNS, more renal
Hemolytic anemia
Thrombocytopenia
Schistocytes
Renal fail
Adults: plasmapheresis
Kids: IV hydration w/ e-
TTP-HUS labs?
Microangiopathic hemolytic anemia Thrombocytopenia Smear = Schistocytes Coag = N (diff from DIC) Renal insuff
TTP-HUS tx? (4)
Steroids
Plasmapheresis (plasma exchange)
Rituximab if refractory
High mortality
SUMMARY: TTP triad?
Hemolytic anemia
Thrombocytopenia
Neuro deficits
(ADAMTS13 mutation)
SUMMARY: HUS triad?
Hemolytic anemia
Thrombocytopenia
Renal insuff
Von Willebrand’s Dz epidemiology?
Auto dominant
M=F
Most C coag
vWF causes?
Type I?
↓ Factor VIII ANTIGEN -> deficient or defective vWF
Most C
Acquired (bone marrow CA, valproic acid, SLE)
↓ Factor VIII
vWF presentation? (4)
Bleed (nose, vag, GI)
Hemarthrosis/Soft tissue bleed less likely
Bleed ↑ w/ ASA
Bleed ↓ E2/preggo
vWF labs?
Bleed time = long (N in hemophilia)
INR = N
vWF = low
vWF tx?
Desmopressin (releases stores vWF from endo cells)
Factor VIII infustion
Humate-P (VIII and vWF)
Hypercoag States: Congenital?
U auto-dominant
Deficiencies in:
Antithombin (III)
Prot C or S
Mutations in:
Factor V (Leiden)
Prothrombin (II)
Antithrombin III deficiency epidemiology?
Causes what?
What med acts on III?
Congenital or acquired (DIC, HUS)
Thromboembolic dz
Lack of III means no inhibition of coag cascade
Heparin (↑ effects of III)
Antithrombin III Deficiency presentation?
Labs?
Tx? (2)
FHx of DVT, PE
U 20-30yo
U asympt
Acute thrombosis or embolism
III = low
IV heparin
Long-term Warfarin
Protein C Deficiency epidemiology?
Causes what?
Presentation?
Auto dominant
Prot C is Vit-K dependent
DVTs
Lack of C means no fibrinolysis ∴ no clot lysis
FHx
Spontaneous or provoked DVT
Protein C Deficiency labs?
Tx? (3)
Prot C = low
IV heparin
Long-term Warfarin
No oral contraceptives or hormone replacement
Protein S Deficiency epidemiology?
Causes what?
Presentation?
Auto domin or
Acquired (liver dz, vit K def)
VTE, thromboembol
Lack of S means no Prot C activation
VTE U <40yo
Spontaneous abortion
Protein S Deficiency labs?
Tx?
Prot S antigen = low
Same as Prot C
+ prophy w/ preggos
Factor V Leiden Mutation epidemiology?
Causes what?
Hereditary thrombophilia
Resists activated Prot C ->
venous thrombosis, thromboembolism
Factor V Leiden Mutation labs:
aPTT
PTT
aPTT = screen for activated Prot C (APC) resistance
PTT = measure w/ APC and w/o APC,
If resistance +, PTTs will be equal
If resistance -, PTT longer w/ APC
If abnormal, test for mutation
Factor V Leiden Mutation tx?
same