Question 1 · Hard
A 38-year-old woman has confusion and petechiae. Results: Hb 7.9 g/dL [79 g/L], platelets 9 × 10^3/µL [9 × 10^9/L], schistocytes 8%, LD 1,650 U/L (reference 100-225 U/L), haptoglobin < 10 mg/dL [< 0.1 g/L], DAT negative, PT and APTT normal, and creatinine 1.1 mg/dL [97 µmol/L]. Plasma exchange was started at once. The ADAMTS13 test was requested later and run on a citrate sample drawn after the first exchange: activity 46% (reference > 60%). Plasma from the citrate tube used for her PT at admission, before any treatment, was separated and frozen. The best action is:
- ATest ADAMTS13 on the admission EDTA tube
- BRepeat ADAMTS13 after the next exchange
- CReport that TTP has been excluded
- DTest the stored pretreatment plasma
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Answer: D · Test the stored pretreatment plasma
Schistocytes, severe thrombocytopenia, a high LD, absent haptoglobin, a negative DAT, normal clotting times and near-normal kidney function strongly suggest immune TTP, which is confirmed by ADAMTS13 activity below 10%. Plasma exchange replaces the patient's plasma with donor plasma that contains normal ADAMTS13, so a sample taken after the first exchange can give a falsely reassuring result. ADAMTS13 should therefore be measured on plasma collected before any plasma therapy, and the stored pretreatment citrate plasma is the right specimen. EDTA is unsuitable because it chelates the metal ions the enzyme needs, and a sample after the next exchange would be even more diluted with donor plasma. A post-exchange result cannot exclude TTP.
- A: EDTA chelates the zinc and calcium the enzyme needs, so EDTA plasma is not suitable.
- B: A later sample would contain even more donor plasma.
- C: A result after plasma exchange reflects donor ADAMTS13 and cannot exclude TTP.