module 4 disorders of hemostasis Flashcards
Coagulopathy s/s (5)
prolonged pt/ptt joint bleeding tissue hematoma large, spontaneous, centrally located ecchymosis bleeding delayed
Thrombocytopenia
mucosal bleeding
bleed immediately
petechiae maybe
adequate plt count
rarely develop purpura even w trauma
asymptomatic, risk for excessive bleed w trauma
spontaneous bleeds, maybe petechiae
> 50,000
30,000-50,000
10,000-30,000
<10,000
causes of thrombocytopenia (5)
hemodilution (esp Post-op) splenomegaly (ca/cirrhosis) HIV, BM failure Hemolysis Medications
Medications causing thrombocytopenia
Heparin sulfa, vanc, piperacillin, PCN, rifampin quinine linezolid valproic acid, carbamazapine, phenytoin
Idiopathic Thrombocytic Purpura
mostly in ______
Primary
Secondary
mostly in women
Primary: acquired d/t destruction of plts
secondary: d/t SLE, CML, antiphospholipid, meds
Acute vs chronic Idiopathic thombocytic pupura
acute w kids 1-2 months d/t viral infections, sle, drug allergies
chronic- idiopathic (primary) and progessive
Platelet destruction in Idiopathic thrombocytic pupura starts w
___ by ___ who ___ to ____=____
___ binds to ____=destroyed plt
phagocytosis by macrophages who present digested plt peptides to CD4 cells-> = immune response
IgG bind to glycoproteins ->plt destroyed
Neonatal thrombocytopenia is when
can be 2 reasons
Risk of
Moms IgG’s cross over to fetus’ plts=destroyed
Can be bc mom has Idiopathic thrombocytic pupura or just an alloimmune response
intracranial hemorrhage risk when plt <20,000
Idiopathic thrombocytic pupura symptoms
petechiae, purpura, bruising
GI/GU, nose bleeds
intracranial hemorrhage
older adults more severe bleeds bc of comorbidities (htn)
How can Thrombotic Thrombocytopenic purpura cause platelet consumption and organ ischemia
thrombocytic microangiopathy caused when platelets aggregate and occlude the microcirculation
=plt consumption/organ ischemia in brain, heart, kidneys
Acquired vs Familial Thrombotic Thrombocytopenic purpura
familial- affects children, appears chronic, recurring episodes q3wks, responsive to treatment
acquired- acute/severe, females 30+
Thrombotic Thrombocytopenic purpura causes
autoimmune, family, HIV, CA, post-op, meds (antiplt, quinine, mitomcyin, cisplatin, bleomycin)
Patho of Thrombotic thrombocytopenic purpura
gene mutation causing _______=________ ->_____ = _____ in _____ = organ death
^_______
vADAMS13 (d/t mutation in gene making adams13)=^Von willebrand factor
=hypercoaguable =microthrombi in organs
^plasminogen activator inhibitor
Thrombocytic Thrombocytopenic purpura
s/s
may have neuro symptoms (seziure, hemiplegia, ams)
fatigue w anemia (microangiopathic d/t rbc lysis)
petechiae, pallor, jaundice
fever
dark urine from hemoglobinurua
n/v/d abd pain, GI symptoms common
CP, HF, shock ,death
thrombocytopenia, NORMAL pt/ptt, fibrinogen
^cr, BUN, LDH, bili
blood smear=shistocytes
Hemophilia sites of bleeding
knees, elbows, ankles
GI
Spontaneous=severe disease
Hemophilia diagnostic testing
normal platelets
normal pt
prolonged ptt
Hemophilia children diagnosed when _
will see__
diag in children when they start crawling/standing
Bruising, swelling, joint redness, mouth bleeding
Disseminated intravascular coagulation
activation of ____ and ___ d/t ___
activation of coagulation cascade and fibrinolytic cascade d/t underlying disease
Disseminated intravascular coagulation triggered by
CA, sepsis, obstetrical complications, trauma/surguries
ards, rabdo, bites, acidemeia, transfusion rxcn
Patho of Disseminated intravacular coagulation
____= exposed ____= ______
______ broken down->______ prevents adhesion =_____
may cause ____ d/t ______
endothelial damage=exposed TF=coagulation cascade
widespread fibrin deposition broken down-> products adhere to plts, prevent adhesion=bleeding
may cause organ failure d/t micro thrombi
Disseminated intravascular coagulation
s/s
bleeding from wounds, venipuncture
stroke, PE, necrotic skin lesions
hemorrhaging into closed compartments
multisystem failure= neuro, oligura, hematuria, hypotension, cyanosis of toes/fingers
Disseminated intravasular coagulation pt/ptt fibrinogen d diner plt
prolonged pt/ptt
low fibrinogen
^d-dimer
plt <100,000
arterial vs venous thrombus
main complication w either
arterial: high blood flow, fibrin/plts (stroke)
venous: low blood flow, rbcs/fibrin few plts (PE)
virchows triad (dvt) 3:
injury to blood vessel (atherosclerosis, htn, smoking)
abnormalities in blood flow (turbulent w sickle cell, ^viscocity)
hypercoagulable states (chf, MI, bed rest)
Unusual sites for thombus (4)
mesenteric vein, renal vein, hepatic, cerebral
arterial thrombosis associated w 3 deficiencies and ___
protein c
protein s
antithrombin
lupus anticoagulants
Factor 5 Leidan Mutation (hereditary hypercoagulable)
common cause of ___
causes factor ____ to be resistant to _______= _______ by APC= ^_________
common cause of thrombophilia in caucasians
factor Va resistant to activated protein c (APC)
= factor Va not broken down=^thrombin
=thrombophilic state
Protein S deficiency (hereditary hypercoagulable)
activates ____ which inactivates ___ and ____
why does inflammation=risk for clots
activates protein C which inactivates Va and VIIIa
some attaches to complement
during inflammation=vProtein S=^coagulable
Protein C deficiency (hereditary hypercoagulable)
what kind of mutation causes this
activated by protein S to form activated protein c
APC inactivates Va and VIIIa
autosomal dominant mutation in PROC gene