#3740 AFTER ALL A TYPICAL HUS

Abstract Background and Aims Typical Hemolytic Uremic Syndrome (HUS) is a thrombotic microangiopathy (TMA) characterized by the triad of acute renal failure, hemolytic anemia thrombocytopenia. TMAs are classified into: Inherited or primary acquired (atypical HUS), secondary infection associated. Infection with Shiga-toxin–producing Escherichia coli (E. coli), mainly serotype O157:H7, most common cause typical HUS, but other serotypes have been rarely associated such as Enteroagregative (EAEC) O104:H4. Method Case Report Results We report case 51-years-old male past medical history arterial hypertension (treated Perindopril) IgGk Multiple Myeloma (MM) diagnosed 10 years prior submitted to two bone marrow autotransplants (9 4 prior) multiple chemotherapy agents including Carfilzomib-Pomalidomide-Dexamethasone (last cycle days before onset symptoms). One week after returning from trip Brazil, he was admitted emergency department two-day non-bloody diarrhea (4-5 liquid stools per day) asthenia, similar positive familiar epidemiology. He denied abdominal pain, fever, nausea vomiting. At physical examination, hypertensive (177/90 mmHg), anuric (12,5 ml/h), dehydrated apyretic. Abdominal palpation painless there were no signs peritoneal irritation. Laboratory tests showed hemoglobin 10.5 g/dl, new thrombocytopenia (261 000/uL » 21 000/uL), kidney disfunction (serum creatinine (sCr) 0.74 mg/dL 9 serum urea (sU) 41 260 mg/dL), hyperkaliemia (6 mEq/L) metabolic acidosis (pH 7,39 bicarbonate 18.4 mEq/L). LDH 1949 U/L, C-reactive protein 30.6 mg/L, procalcitonin 3.77 ng/mL, haptoglobin <0.07 g/L normal complement levels (C3 C4). Schistocytes present in peripheral blood smear. The diagnosis Acute Kidney Injury related TMA established patient transferred Nephrology ward urgent hemodialysis started. Exhaustive study performed showing ultrasound, non-nephrotic proteinuria (1.6 g/g), urinalysis leukocyturia erythrocyturia. Autoimmune PR3-ANCA, MPO-ANCA, anti-GBM antibody, Anti-dsDNA anti-beta 2-glycoprotein I antibodies, anticardiolipin anti-ADAMTS13 antibodies ADAMTS13 activity, well C5, C'3, C'4, factor B, H I, anti-factor ac, AH 50 CH50b unremarkable. Blood urine culture antistreptolysin O titers negative. Specific stool cultures for E. O157, Shigella, Salmonella, Yersinia Campylobacter Stool evaluation parasites also Viral serology HIV, HBV, HCV Plasmodium PCR required due high suspicion E.coli infection. Carfilzomib induced raised, while molecular techniques results awaited. MM stable treatment held until recovery function. During hospitalization recovered diuresis decreased. On 11th day hospitalization, discharged still depended on hemodialysis. Finally, identified aatA aggR genes EAEC O104:H4 allowing infectious HUS rather than drug-related TMA. discharge, dialysis stopped sCr 3 mg/dL. Four months later, resumed function remained 0.9 mg/dL, despite sustained 1.2 g. Conclusion authors emphasize importance considering causes when O157 excluded, namely (as Carfilzomib) less frequent This reveals requesting even test particular relevance immunosuppressed patients. allowed continuing treatment.