A severe case of atypical hemolytic uremic syndrome associated with pneumococcal infection and T activation treated successfully with plasma exchange

Abstract

The dramatic response seen in this patient suggests that PE utilizing albumin replacement may benefit patients with severe pHUS.

Management of hemolytic uremic syndrome

“…All six pheresed patients survived with renal and neurological outcomes similar to the remainder of the surveyed cohort. Full recovery was also noted in a young child with DAT and T-antigen positive pnHUS who received 13 PE against 5% albumin [58]. Six of 12 patients reported by Brandt et al [50] (three Coombs test positive) received FFP infusions without discernible effect on platelet counts following treatment.…”

“…Neuraminidase may also be neutralized by donor plasma which may have enzymeneutralizing capacity [53]. However, experience with PE for pnHUS is limited [46,50,51,58]. Six of 43 patients with pnHUS described by Waters et al [46] received PE, two were exchanged against ''low-titre anti-TF'' fresh frozen plasma Management of hemolytic uremic syndrome (FFP) and three against albumin.…”

“…Two of 11 patients reported by Prestidge et al [51] received FFP and one cryoprecipitate, without apparent adverse effects. In summary, although PE against FFP has been described in few patients with pnHUS without reported adverse effects [46], the efficacy of this approach is uncertain and it seems prudent to use albumin replacement [58].…”

Severe transient ADAMTS13 deficiency in pneumococcal-associated hemolytic uremic syndrome

“…However, the necessity of avoiding transfusions of plasma-containing blood products in p-HUS is controversial. Instead, plasma exchange with albumin as replacement fluid has been advocated by others [18]. The rationale is that plasma exchange represents a logical therapy by removing the level of circulating anti-TF antibodies and neuraminidase.…”

Egészségügyi szakmai irányelv – A thromboticus thrombocytopeniás purpura (TTP) és a haemolyticus uraemiás syndroma (HUS) kezeléséről

“…A plazmaterápiával kapcsolatban kontrollált tanulmányok nem állnak rendelkezés-re. A patogenezis alapján a plazmaterápia (FFP-infúzió vagy FFP-szubsztitúcióval végzett plazmacsere) kerülen-dő az akut szakban, helyette albuminszubsztitúcióval végzett plazmacsere lehet hatásos [70][71][72]. Az a ferezistől várható haszon és a lehetséges mellékhatások, szö-vődmények egyéni mérlegelést igényelnek.…”