Plasma Volume to Achieve Remission in Atypical Hemolytic Uremic Syndrome

Atypical hemolytic uremic syndrome (HUS) is defined as a heterogeneous group of disorders. Plasma infusion or plasma exchange is the rescue therapy for this life-threatening syndrome. There is no evidence for the volume of plasma required to induce remission. Between 2007 and 2018, Forty – two patients (M=20, F=22) with a diagnosis of recurrent or familial atypical hemolytic uremic syndrome (aHUS) who were admitted to Ali-Asgar Children’s Hospital were enrolled in this observational retrospective study. The total volume of plasma required for normalizing platelet (>150000) and LDH (<500 IU), eliminating hemolysis, and decreasing serum creatinine at first presentation of disease was calculated. Patients with TTP, vasculitis, and post infectious HUS were excluded. The mean age of the patients was 53 months (3-144 m). The majority of patients achieved remission at first presentation by plasma infusion (5 under peritoneal dialysis and 4 under hemodialysis) but ten patients required plasmapheresis. A total of 980 units of FFP perfused with a total volume of 195.975 L. The median (range) total plasma volume required for remission was 166 ml/kg (43-2850 ml/kg). This study showed that the required plasma volume for the acute phase of atypical HUS for controlling the first attack of disease.Keywords: Atypical Hemolytic Uremic Syndrome; Plasma Volume; Remission Induction; Child.