An 18 year old female re-presents to the emergency department after 3 days of severe migraines. Her CBC is shown below. What other investigations are pertinent in her diagnosis?
Points of discussion: It is imperative that any diagnosis of microangiopathic hemolytic anemia be diagnosed immediately due to the inherent mortality risk with disorders such as TTP or DIC. Investigations include:
- Peripheral blood film to assess for fragmentation
- CBC, LDH, bilirubin, haptoglobin, reticulocyte count
- Group and screen, direct antiglobulin test
- Lytes, Cr, calcium panel (Ca, Mg, phosphate, albumin), liver enzymes
- PT, PTT, fibrinogen
- Add septic workup including stool sample in presence of diarrhea.
- Consider adding HIV, HCV, and autoimmune screen if suspicion of TTP
- ADAMTS13 screening if suspicion of TTP
- Beta HCG for all women of childbearing age
What is the management of TTP?
Points of discussion: This is a clinical diagnosis with a mortality of >90% without treatment. Do not wait for ADAMT13 testing to support a diagnosis.
- Immediate transfer to a plasmapheresis center
- Frozen plasma to be administered immediately to replete deficient ADAMTS13
- Daily plasmapheresis (Repletes ADAMTS13 and removes Antibody targeting ADAMTS13)
- Folic acid, iron (if deficient), role of prednisone and ASA is controversial