MAHA and Thrombocytopenia Flashcards
classic hematological finding on the PBS of maha
schistocytes. caused by damaged vascular endothelium.
reasons behind MAHA
TTP, HUS, DIC, HELLP, and hypertensive crises.
what indicate MAHA on the CBC
thrombocytopenia, low Hb.
Markers of hemolysis anemia
increased LDH, increased bilirubin, low haptoglobin
What must you rule out to diagnose MAHA
Negative DAT/coombs test which rules out autoimmune hemolysis
TTP is caused by a deficiency of ____ needed to cleave ultralarge ____ multiverses. These multiverses aggregating can cause uncontrolled platelet agglutination.
Acquired ___ deficiency is typically due to inhibition by an ____
TTP is caused by a deficiency of ADAMTS13 needed to cleave ultralarge WVF multiverses. These multiverses aggregating can cause uncontrolled platelet agglutination.
Acquired deficiency is typically due to inhibition by an autoantibody.
Primary clinical diagnosis when a patient presents with thrombocytopenia and MAHA without an alternative explanation
Lab findings of TTP
MAHA: schistocytes Increased LDH Thrombocytopenia Increased bilirubin Decreased haptoglobin. Decreased ADAMTS13 activity. PT, PTT normal
T/F in TTP, PT and PTT are elevated
False. Nothing is wrong with clotting, it’s causing aggregation of VWF and platelet agglutination (factors aren’t actually doing anything).
PT/INR and PTT is normal
treatment of TTP
It’s a medical emergency.
Daily plasma exchange is required, and steroids or immunosuppressive therapy is used in addition.
Platelet transfusions are done in TTP?
No. Platelet transfusions are avowed in TTP and may be dangerous as it provides fuel for the development of thrombosis.
HUS caused by ____
Shiga toxin, or some types of ecolI
Consider HUS if person has diarrhea and also thrombocytopenia/maha
Just like TTP, HUS needs plasma exchange?
FALSE. HUS is supportive. It will go away, unlike TTP which has an inherent problem with ADAMTS13, shiga toxin might temporarily bind to ADAMTS13 to make it look like TTP but antibiotics will help
_____ is a syndrome of unregulated systemic coagulation, which results in widespread deposition of fibrin clots in blood vessels. Results in consumption of platelets and coagulation factors, resulting in ___ ___ as well as compromised blood flow due to ____ which can ultimately lead to tissue ____ and organ damage.
DIC is a syndrome of unregulated systemic coagulation, which results in widespread deposition of fibrin clots in blood vessels. Results in consumption of platelets and coagulation factors, resulting in EXCESSIVE BLEEDING as well as compromised blood flow due to THROMBOSIS which can ultimately lead to tissue ISCHEMIA and organ damage.
DIC can be considered as a 3 step process.
- Release of large amounts of tissue factor stimulates the extrinsic pathway by activating
- Excessive thrombin production and defective physiologic anticoagulant mechanisms.
There is a reduced production and increased consumption of antithrombin, protein C,
protein S and tissue factor pathway inhibitor.
- Suppression of fibrin clot degradation due to inhibition of fibrinolysis