FA + MTB 2 Flashcards
Hemophilia deficiency and type of inherited
Hemophilia A - 8 - XR
Hemophilia B - 9 - XR
Hemophilia C - 11 - AR
Hemophilia A, B, C - treatment
A: desmopressin + factor 8 concentrate
B: factor 9 concentrate
C: Factor 11 concentrate
Hemophilia A, B, C - PTT, PT
normal PT
increased PTT
Vitamin K deficiency - PT, PTT, BT
increased PT, PTT
normal BT
Platelets abnormalities - sign symptoms labs
- Microhemorrhage (mucus bleeding , epistaxis, petechiae, purpura
- Increased bleeding time
- Possible decreased platelet count
Bernard-Soulier - PC, BT
PC: normal or decreased
BT: increased
Bernard-Soulier diagnosis
No agglutination on ristocetin cofactor assay
Bernard-Soulier pathophysiology
Deficiency of Gp1b (platelets - vWf adhesion)
Bernard-Soulier findings
- Defects in platelet plug formation
2. Large platelets (younger)
Glanzmann thrombasthenia - PC,BT
PC: -
BT: increased
Glanzmann thrombasthenia pathophysiology
GpIIb/IIIa deficiency –> defects in platelet to platelet aggregation
Glanzmann thrombasthenia labs
Blood smear shows no platelet climbing
Glanzmann thrombasthenia - agglutination
Agglutation with ristocetin
Immune thrombocytopenia pathophysiology
Anti GpIIb/IIIa antibodies –> splenic macrophages consumption of platelet antibody complex
Commonly after viral illness
Immune thrombocytopenia labs
Increased megakaryocytes on bone bone marrow biopsy
Immune thrombocytopenia treatment
- Steroids
- IV immunoglobulins
- splenectomy (for refractory)
Immune thrombocytopenia - PC, BT
PC: decreased
BT: increased
Thrombotic thrombocytopenic purpura pathophysiology
Inhibition or deficiency of ADAMTS 13 (vwf metalloprotease) –> decreased degradation of vwf multimers –> large vwf multimers –> platelets aggregation and thrombosis
Thrombotic thrombocytopenic purpura labs and symptoms
LDH increased
Schistocytes
symptoms: pentad of neurologic and renal symptoms, fever, thrombocytopenia, microangiopathic hemolytic anemia
Thrombotic thrombocytopenic purpura treatment
- Plasmapheris
2. Steroids
Thrombotic thrombocytopenic purpura - PC, BT
PC: decreased
BT: increased
Hemolytic-uremic syndrome - typically seen in/due to
children, accompanied by diarrhea and commonly caused by Shiga-toxin-producing E-coli (eg. 157:H7)
in adults does not present with diarrhea
STEC infection is not required
Hemolytic-uremic syndrome - treatment
plasmapheresis
Hemolytic-uremic syndrome - symptoms
neurologic and renal symptoms, fever, thrombocytopenia, microangiopathic hemolytic anemia
Hemolytic-uremic syndrome - characterised by
- thrombocytopenia
- microangiopathic hemolytic anemia
- acute renal failure
Hemolytic-uremic syndrome - PC, BT
decreased PC
increased BT
MC inherited bleeding disorder / mode of inheritance
Von willebrand disease (but mild)
AD
Von willebrand disease diagnosis / treatment
No agglutination with ristocetin
Desmopressin (release vWF stored in endothelium)
Von willebrand disease - PC, BT, PT, PTT
PC normal
BT increased
PT normal
PTT increased or normal
DIC - PC, BT, PT, PTT
PC decreased
BT increased
PT increased
PTT increased
Causes of DIC - results in
STOP Making New Thrombi
Sepsis (gram -), trauma, obstetric complication, acute pancreatitis, malignancy, nephrotic syndrome, transfusion
results in widespread activation of clootting –> deficiency of factors –> bleeding state
hereditary thrombosis syndromes leading to hypercoagulability - types
- antithrombin def
- V leiden
- protein S or C def
- Prothrombin gene mutation