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Atypical Hemolytic Uremic Syndrome

Atypical Hemolytic Uremic Syndrome
#00060948
Author: Anna Elias; Richard Elias; Lakshmi Nath; Shriram Nath
Category: Red Cell: Hemolytic Anemia (HA) > Acquired non-immune HAs > Fragmentation Hemolysis > Microangiopathic Hemolytic Anemias > Hemolytic Uremic Syndrome
Published Date: 01/23/2017

A 28-year-old G1P0 woman at 10 weeks gestation presented to hospital with fever, vomiting and right lower quadrant tenderness.  Ultrasound demonstrated fetal demise.  The patient underwent a laparoscopic appendicectomy and dilatation and curettage.  There was significant blood loss associated with the latter procedure.  Shortly thereafter she developed acute renal failure, severe anemia, thrombocytopenia and hemolysis creatinine 5.09 mg/dL, Hb 75 g/L, Plt 18 x 10^9/L, WBC 25 x 10^9/L.  PT, APTT, S. Fibrinogen was normal. LDH 2784 U/L, S. Haptoglobin was <0.01 g/L and DAT negative. Stool for Shigella toxin was negative.  ADAMTS13 level was 78%.  Blood film demonstrated fragmented red cells, helmet cells, microspherocytes, polychromasia.   A diagnosis of atypical hemolytic uremic syndrome (HUS) secondary to missed miscarriage was made. The patient received initial plasmapheresis, red cell and platelet transfusions and treatment with eculizumab.  She required a period of continuous renal replacement therapy.