54 year old male, 75 lb weight loss. Had multiple investigations, CT, Laparotomies, serology all negative for SLE, vasculitis etc. Normal bone marrow etc. A diagnosis of "Hollow Viscus Syndrome" was made.
Transferred to our hospital August 27 to GI service for bowel rest and TPN. On Admission noted to have TMA, Hbg ~75, fragments, Plts ~45, high LDH, Undetectable haptoglobin. PLEX stared on Aug 28 day. ADAMST-13 normal. No offending agents. Renal function deteriorating at anephric rate. Nephrology consulted Aug 30, when creatine was 350 umol/L. U/A shows HG casts. Renal biposy Aug 31, shows : TMA, with glomerular thrombi, and small vessel thrombi. All very acute. Negative IF, no crescents. mild ATN.
Patient now anephric, in ICU with pulmonary edema, responding to dialysis. Still getting PLEX
1) Has anyone seen aHUS present with such profound GI manifestations (there was no pancreatitis)?
2) Clear indication for soliris?