The manifestation’s feature of atypical hemolytic uremic syndrome using eculizumab (clinical case)

Abstract

Background. There is a clinical case of an atypical hemolytic-uremic syndrome (aHUS), beginning with symptoms of heavy damage of the gastrointestinal tract with prolonged repeated diarrhea and abdominal pain. They were imitating a pseudomembranous colitis, which was made possible to exclude due to the negative Clostridium Difficile test results and the lack of effect from treatment with vancomycin. The uniqueness of this case is in the rare occurrence of this pathology among the adult population . Timely diagnosis of aHUS, pathogenetic treatment help to reduce mortality. Materials and methods. Analysis of the medical history and observation of the patient, who hospitalized in the nephrology department of the Republican Hospital of Saransk. Results. Fulminant development of the disease with damage of the gastrointestinal tract, kidneys, lungs, microangiopathic hemolysis, thrombocytopenia, increased blood lactate dehydrogenase and decreased activity of ADAMTS 13 metalloproteinase have enabled to diagnose aHUS. Using pathogenetic complement- blocking therapy with Eculizumab achieved regression of clinical signs of the disease, normalization of laboratory parameters. Conclusions. The course of aHUS is characterized by an unfavorable prognosis, the risk of sudden complications. The diagnosis of this disease based on clinical and laboratory signs of thrombotic microangiopathy, such as microangiopathic Coombs-negative hemolytic anemia, thrombocytopenia, multiple organ damage: acute damage to the kidneys, lungs (pneumonitis, atelectasis), intestines (erosive and ulcerative colitis), pancreas (subacute pancreatitis, transient hyperglycemia). Timely diagnosis allowed to achieve positive dynamics against the background of treatment with Eculizumab, which restores the regulation of the activity of the complement system in the blood. Therefore, patients with this diagnosis require special attention with lifelong monitoring and treatment, due to the high risk of sudden complications.

Keywords

• atypical hemolytic-uremic syndrome
• thrombotic microangiopathy
• thrombotic thrombocytopenic purpura
• thrombocytopenia
• complement-blocking