Hematology Question 18 Instructor

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?

CBC Patient Reference Range
RBC 2.20E12/L (3.80-5.20)E12/L
Hgb 63g/L (115-155)g/L
Hematocrit 0.219 (0.345-0.450)/L
MCV 85fL (82.0-97.0)fL
MCH 26pg (27.0-32.0)pg
RDW 18% (11.0-15.0)%
Platelets 8E9/L (140-400)E9/L

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
  • Urinalysis
  • 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.

  1. Immediate transfer to a plasmapheresis center
  2. Frozen plasma to be administered immediately to replete deficient ADAMTS13
  3. Daily plasmapheresis (Repletes ADAMTS13 and removes Antibody targeting ADAMTS13)
  4. 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

Subscribe to Pearls

Uncle Sam wants you to subscribe to Medical Pearls

Subscribe today! It’s always free, and you can easily unsubscribe at any time. We will never share your email address. Subscribe to...

Multiple Pearls
Cardiology Pearls
Endocrinology Pearls
Hematology Pearls
Nephrology Pearls
Rheumatology Pearls
Transplant Pearls
General Internal Medicine Pearls
Instructor Pearls

Subscribe to receive your pearls today—it's free!