schistocytes, polychromasia and low plt
Critical to differentiate between TTP and atypical HUS
1. TTP: worse tpenia, HTN less common, kidney impairment not so prominent
2. aHUS: more HTN, plt usually> 30K, renal function much worse
If plt > 30K, new HTN, creatinine > 2--think aHUS--> eculizumab
Upfront test for Shiga toxin if diarrhea, send ADAMTS 13 level AND inhibitor for ADAMTS 13 ( if less than 10% confirms)
Look for secondary causes: drugs, transplant, pregnancy, HELLP, DIC, infection
PLASMIC and FRENCH scores to rule out TTP, have lower sensitivity in patients over 60 yr
Rx of TTP- PEX, Steroids, rituxan
Clinical remission versus partial or complete ADAMTS 13 remission.
Partial ( level > 20% but less than LLN)
Complete ADAMTS 13 remission level higher than LLN
Clinical : normal counts, without PEX for over 30 days
What is an ADAMTS 13 relapse: if level drops to less than 20% during the 3 month follow up, give pre-emptive rituxan
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