R2 Talk: Dr. Nathan Hocker
Mentor: Lisa Antes
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Thrombotic Microangiopathy
Criteria
* Microangiopathic Hemolytic Anemia
* Thrombocytopenia
* End-organ damage – AKI, CVA, MI, PE, Encephalopathy, Symptoms of underlying cause. AKI most common.
* No coagulopathy (as opposed to DIC)
Classification – Primary vs Secondary
* Primary – genetic or antibody-mediated
o TTP
o Complement-Mediated
* Secondary
o Infection – STEC-HUS
o Cancer
o Pregnancy
o Transplant – solid organ or bone marrow
o Medications
* Quinine
* Calcineurin inhibitors
* Chemotherapy
Thrombotic Thrombocytopenic Purpura – treat first, ask questions later
* Most common cause of TMA in adults
* 90% mortality without treatment, 10% mortality with treatment
* Plasma exchange is primary treatment modality
* Neurologic symptoms are more common than AKI (as opposed to other TMAs)
* Diagnose with ADAMTS13 activity – <10% activity is confirmatory.
o Always treat while test is pending
Hemolytic Uremic Syndrome
* More common in children, less common in adults
* Typical cause is Shiga-Toxin E. Coli (O157:H7) – STEC-HUS
o Presents with dysentery and AKI
* Diagnose via enteric pathogen panel
* Can lead to CKD/ESRD, death is less common
* Treatment is supportive
Complement-Mediated HUS/TMA
* Consider after ruling out TTP and STEC-HUS
* Check complement levels – C3, C4, CH50
o Typically low but not always
* Treat with eculizumab – monoclonal C5 antibody
o Require prophylaxis and vaccination for N. meningitidis
