IL9 : Hemostatic Disorder Flashcards
(20 cards)
what is hemophilia and cause
rare x-linked recessive bleeding disorder caused by pathogenic variants in factor 8 and 9
hallmark manifestation of hemophilia
bleeding tendency, hemarthrosis, intramuscular hematoma
what type of mutation is in hemophilia
Lenovo mutation (around 20-30%)
what laboratory found in patient w hemophilia
prolonged APPT and low factor 8 and 9
what is Von Willebrand disease
excessive mucosal bleeding / bleeding after invasive procedures
Von Willebrand disease caused by
qualitative and quantitative defect of VWF
in Von Willebrand disease, bleeding symptoms vary among what
age and sex
disease subtype
level of residual VWF and factor 8
Von Willebrand disease is inherited autosomal bleeding disorder true/false
true
Von Willebrand disease Lab test =
prolonged aPTT
decreased level of VWF
Immune thrombocytopenia caused from
increase platelets clearance
impaired megakaryocyte
what is the pathophysiology of B cell in Immune thrombocytopenia
- B cell produce autoantibodies against platelet glycoproteins
- antibody bind to platelet and mark for clearance by splenic macrophage -> accelerated platelet clearance
- antibody also bind to megakaryocytic (platelet precursor) to stop maturation
- activate complement or desialylation
what is the pathophysiology of T cell in Immune thrombocytopenia
helper T cell help stimulate autoantibody production
1. cytotoxic T cells can directly attack platelet or megakaryocyte -> decreasing platelet number
2. Regulatory T cell defect -> can’t suppress auto immune -> loss of self tolerance and over activation of B or T cells
can patient w immune thrombocyclopenia be asymptomatic
yes and mostly
what is ITP
= platelet count below 100,000x10^9
- diagnosis of exclusion
1. to rule out other causes of thrombocyclopenia
2. to evaluate for secondary causes of ITP
what screening tool can be used to identify patients w VWF
BAT ( ISTH- Bleeding Assessment Tool)
- can also use to detect other bleeding disorder as well
- decrease blood testing
- limitation only people w bleeding symptoms
cirrhosis cause alteration in what
primary hemostasis, coagulation, fibrinolysis
clinical finding in DIC
thrombocytopenia, prolonged PT and aPTT, hyperfibrinogenemia, elevated D dimer, microangiopathic hemolytic anemia
laboratory finding in liver disease
thrombocytopenia, prolong PT (early liver impairment), prolonged aPTTT (advanced liver impairment) hypofibrinogenemia
laboratory in Vitamin K deficiency
prolonged PT and APTT
low factor 2, 5 ,7
