P0315SUCCESFUL TREATMENT OF A STRETOCOCCUS-PNEUMONIAE-ASSOCIATED HAEMOLYTIC URAEMIC SYNDROME BY A SHORT COURSE OF ECULZIMAB

Abstract

Abstract Background and Aims Hemolytic uraemic syndrome (HUS) is a known rare complication of Streptococcus pneumonia infections (SP-HUS) and is characterized by microangiopathic hemolytic anemia, thrombocytopenia and renal failure. In adults it is associated with a higher mortality than HUS due to other etiologies. Method Case Report Results A 33-years old female patient with a history of alcohol abuse was referred to hospital with hypotension and symptoms of infection, including high fever and diarrhea, which had occurred since three days. On admission the patient presented with septic shock and was transferred to the intensive care unit. There she showed signs of hemolytic uraemic syndrome, including severe thrombocytopenia (11 Gpt/l), schistocytosis, elevated lactate-dehydrogenase (LDH; max. 2300 U/l), low haptoglobine (<0,3g/l) and acute anuric renal failure with need of continuous renal replacement therapy. Severe confusion and necrotic lesions of the extremities completed the clinical picture. Plasma exchange (PE) was initiated under suspected thrombocytopenic thrombotic purpura (TTP). Under PE the clinical situation did not ameliorate and signs of severe HUS persisted. A total of four PE were realized, TTP was excluded by Factor VIII-ADAMTS13-activity above the diagnostic limit. In the peripheral blood cultures Streptococcus pneumonia was detected, and antimicrobial was deescalated from piperacillin/tazobactam to penicillin G. Under the clinical picture of a persistent severe pneumococcal associated HUS, without clinical benefit of PE, we decided to start therapy with eculizumab, a monoclonal anti-C5-antibody, approved for the therapy of aHUS and with anecdotical evidence of efficacy in SP-HUS. We started with 1200 mg of ECZ, followed by doses of 900 mg weekly with a total of four doses. Under therapy thrombocyte count normalized, renal function ameliorated and dialysis could be discontinued, the levels of haptoglobin and LDH normalized. The patient could be referred to a rehabilitation clinic, where renal function normalized and no further signs of hemolysis were seen three months after discontinuation of therapy under monitoring. The forefoot remained necrotic with need of surgery in future, the ischemic lesions of the hands completely disappeared. Conclusion Eculizumab could provide a therapeutic option of pneumococcal associated HUS. Therapy could be safely discontinued after 1 month under strict monitoring of signs of HUS. Further studies are required to document the benefit of complement blockade therapy in severe forms of SP-HUS.