Atypical HUS caused by anti-complement factor H antibody: a hematologist's perspective
نویسندگان
چکیده
Hemoglobin 4.5 g/dL 11.5–15.5 g/dL MCV 98 fL 77–94 fL RBC count 3.2×10/L 4.0–5.2×1012/L Total leukocyte count 10.3×10/L 5.0–13.0×109/L Platelet count 60×10/L 170–450×109/L Reticulocyte production index 7.2 0.5–2.5 Urea 284 mg/dL 20–40 mg/dL Creatinine 6.1 mg/dL 1–2 mg/dL Uric acid 24.1 mg/dL 2–4 mg/dL Total bilirubin 3.3 mg/dL Up to 1.0 mg/dL AST 127 U/L 0–35 U/L ALT 30 U/L 0–35 U/L LDH 3,876 U/L 100–190 U/L Direct Coombs test Negative Negative PNH clone (FLAER) Negative ANA, dsDNA Negative C3 complement levels 35 mg/dL 75–135 mg/dL Stool culture No growth -
منابع مشابه
Anti-Factor H autoantibodies associated with atypical hemolytic uremic syndrome.
Several studies have demonstrated genetic predisposition in non-shigatoxin-associated hemolytic uremic syndrome (HUS), involving regulatory proteins of the complement alternative pathway: Factor H (FH) and membrane co-factor protein (CD46). Regarding the observations of thrombotic thrombocytopenic purpura patients, in whom a von Willebrand factor protease (ADAMST-13) deficiency may be inherited...
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