P0137FIRST RUSSIAN BOSIMILAR OF ECULIZUMAB IN THE TREATMENT OF AN ADULT PATIENT WITH ATYPICAL HEMOLYTIC UREMIC SYNDROME
Abstract Background and Aims The disease diagnostics results and the efficacy of pathogenetic treatment when transferring from the original drug to the biosimilar were evaluated during clinical observation of an adult patient with aHUS. Method A 46-year-old patient has a history of hypertension sinc...
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Veröffentlicht in: | Nephrology, dialysis, transplantation dialysis, transplantation, 2020-06, Vol.35 (Supplement_3) |
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Sprache: | eng |
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Zusammenfassung: | Abstract
Background and Aims
The disease diagnostics results and the efficacy of pathogenetic treatment when transferring from the original drug to the biosimilar were evaluated during clinical observation of an adult patient with aHUS.
Method
A 46-year-old patient has a history of hypertension since 2012 and maximum ABP of 230/130 mmHg. In 2016, newly diagnosed azotemia was identified (blood creatinine — 140 μmol/L, GFR by EPI — 54 mL/min/1.73m2). In 2018, the patient suffered an acute cerebrovascular accident in the vertebrobasilar area. On admission to Almazov National Medical Research Centre, blood test showed: creatinine – 260 μmol/L (GFR by EPI — 28 mL/min/1.73m2), urea – 11.52 mmol/L, LDH – 130 U/L, complement system C3 – 1.32 g/L, С4 – 0.37 g/L, hemoglobin – 144 g/L, platelets – 302*109/L; urinalysis showed: daily protein loss – 2.6 g/day, RBC – 0–1 per field. Taking into account the kidney damage, the renal fine-needle aspiration biopsy was performed, revealing histopathological findings typical of chronic thrombotic microangiopathy of extreme severity, arteriolar arteriosclerosis with total or subtotal luminal occlusion, extensive secondary perihilar segmental glomerulosclerosis (27%); and global glomerulosclerosis (55%). According to the study results, the secondary genesis of TMA was ruled out, as well as STEC-HUS and thrombotic thrombocytopenic purpura (TTP), systemic diseases (systemic lupus erythematosus, antiphospholipid syndrome (APS), scleroderma), malignancies, HIV infection, sepsis, malignant arterial hypertension, adverse drug events, or disseminated intravascular coagulation (DIC). Plasma ADAMTS-13 levels were also assessed. Its activity was 64% (normal range 93–113%) of ADAMTS-13 activity in the control plasma, thus the diagnosis of thrombocytopenic purpura was ruled out. Due to the absence of anti-CFH-antibodies the antibody origin of aHUS was also ruled out. To assess the contribution of additional factors promoting the development of TMA, the polymorphism of hemostasis genes was studied to reveal homozygous genotypes of platelet collagen receptors (ITGA2: 807 С/Е), heterozygous genotypes of fibrinogen genes (FGB: 455 G/А), folate cycle enzymes (methylenetetrahydrofolate reductase (MTHFR: 677 C/T), methionine synthase (MTR: 2756 A/G (D919G)), responsible for a pronounced tendency towards hyperhomocysteinemia. In the clinical case the diagnosis of aHUS was obvious, which was supported by the classical symptom cluster of TMA and hist |
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ISSN: | 0931-0509 1460-2385 |
DOI: | 10.1093/ndt/gfaa142.P0137 |