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
Scully et al. Guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies. British TTP Guidelines; 2012