[Nephrological aspects of complement-mediated thrombotic microangiopathy].

AIM: To analyze cases of complement-mediated thrombotic microangiopathy (C-TMA) in the daily practice of a hospital nephrology department, relative to the general aspects of the clinical course, therapy, and short-term outcomes.

SUBJECTS AND METHODS: The prospective study conducted in the period June 1, 2015 to June 1, 2016 included 10 patients with C-TMA newly diagnosed during hospitalization. Standard demographic parameters, generally accepted clinical data showing the severity of TMA and the involvement of vital organs were recorded at admission. Short-term outcomes of therapy (plasma therapy and/or eculizumab therapy), such as achievement of hematological remission and the need for maintenance therapy and renal replacement therapy (RRT), were evaluate.

RESULTS: The median time from the onset of symptoms of the disease to diagnosis in the analyzed cases was 5 months (range 1 week to 26 months). The incidence of C-TMA among the inpatients of nephrology departments was 6.4 per 1,000 patients per year. Five patients were admitted to a clinic with symptoms of obvious extrarenal organ disorders; 4 of them had multiple organ dysfunction. Evident clinical symptoms of renal dysfunction were detected in all the patients, which necessitated RRT in 8 out of the 10 patients. Renal biopsy was performed in 7 patients. All were found to have typical morphological manifestations of acute and chronic TMA. All the patients received plasma therapy; most of them had plasma exchanges; 4 patients took eculizumab. There were no fatal outcomes. All achieved hematological remission. Three of the 8 cases could achieve varying recovery of kidney function and stop dialysis.

CONCLUSION: Analysis of this series of cases confirms the idea about the severity of TMA due to multiple organ dysfunction and the high risk of end-stage renal failure at late diagnosis. The key points of better renal prognosis are the earliest detection and treatment of the disease.