ESPN 51th Annual Meeting

ESPN 2018


 
Early use of eculizumab in a case of STEC-HUS before the development of clinical symptoms
MERAL TORUN BAYRAM 1 BELDE KASAP DEMIR 3 ALPER SOYLU 1 SALIH KAVUKÇU 1 FATIH ÖZALTIN 2

1- DOKUZ EYLÜL UNIVERSITY MEDICAL FACULTY
2- HACETTEPE UNIVERSITY MEDICAL FACULTY
3- KATIP ÇELEBI UNIVERSITY MEDICAL FACULTY
 
Introduction:

The treatment of complicated STEC-HUS presents a significant conundrum. Volume expansion, diuretics, antihypertensives, and dialysis form the cornerstone of treatment while plasma exchange, immunoadsorption, and eculizumab have been administered in complicated cases with a paucity of data concerning their efficacy. We report a case of STEC-HUS treated by eculizumab before clinical picture settles.

Material and methods:

 A 3.5-year-old girl developed bloody diarrhea. Stool culture yielded E.coli O157; Shiga toxin was positive. She was followed for clinical/laboratory findings of HUS. After 5 days, while she was still asymptomatic, laboratory evaluation showed thrombocytopenia , hematuria , proteinuria (protein/creatinine 2.02 mg/mg), mild BUN elevation, increased LDH, decreased haptoglobin, schistocytes in peripheral smear and normal C3.

Results:

She was given one dose of eculizumab (600 mg) upon diagnosis of STEC-HUS.Next day all laboratory parameters improved. Creatinine never increased above baseline, BUN normalized on the 1st day, platelet on the 3rd day, LDH on the 5th day. Urine protein/creatinine decreased to 1.75, 1.07, 0.33 and 0.22 on the 2nd, 3rd, 10th and 90th day of follow up, respectively. CFH mutation (exon 20, c.3226C>G/Q1076E, heterozygote) was detected in genetic analyses of complement system. The same mutation was also shown in her healthy mother. The patient is doing well without any further treatment at the moment.

Conclusions:

A lack of efficacy for eculizumab was suggested in STEC-HUS based on the results in the 2011 German STEC-HUS outbreak. However, eculizumab was given to the most severely affected patients in this series. Early use of eculizumab in a French STEC-HUS outbreak resulted in rapid hematological recovery without renal/neurological sequel or death. The course of our patient suggests that very early use of eculizumab in STEC-HUS might result in rapid normalization of laboratory parameters even before the development of clinical findings.