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Comment
. 2014 Sep 11;124(11):1699-700.
doi: 10.1182/blood-2014-07-590356.

COMPLEMENTing the diagnosis of aHUS

Vahid Afshar-Kharghan  1
Affiliations
Comment

COMPLEMENTing the diagnosis of aHUS

Vahid Afshar-Kharghan. Blood. .

Abstract

In this issue of Blood, Noris et al describe a functional complement assay that can be used to diagnose atypical hemolytic-uremic syndrome (aHUS). This assay might also be useful in identifying aHUS patients in remission and in detecting asymptomatic carriers of complement gene mutations. Besides being used for diagnostic purposes, this assay potentially can be used to monitor anticomplement therapy.

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Conflict of interest statement

Conflict-of-interest disclosure: The author participated on an advisory board and served as a consultant for Alexion Pharmaceuticals.

Figures

None
(A) Resting endothelium is protected against complement activation by complement regulators. (B) Normal serum deposited small amounts of C3b (and C3b degradation products) and C5b-9 on ADP-activated endothelial cells, but complement regulatory proteins presented in normal serum inhibited propagation of complement activation on the surface of endothelium. (C) In the presence of serum from a patient with acute aHUS, complement regulation on endothelial cells is ineffective, resulting in a larger number of C5b-9 complexes deposited on the surface of resting endothelium. (D) Serum samples from patients with acute aHUS, aHUS in remission, and healthy carriers of complement mutations deposited C5b-9 on the surface of ADP-activated endothelial cells. In this assay, HMEC-1 were used as the source of endothelial cells.
See this image and copyright information in PMC

Comment on

  • Dynamics of complement activation in aHUS and how to monitor eculizumab therapy.
    Noris M, Galbusera M, Gastoldi S, Macor P, Banterla F, Bresin E, Tripodo C, Bettoni S, Donadelli R, Valoti E, Tedesco F, Amore A, Coppo R, Ruggenenti P, Gotti E, Remuzzi G. Noris M, et al. Blood. 2014 Sep 11;124(11):1715-26. doi: 10.1182/blood-2014-02-558296. Epub 2014 Jul 18. Blood. 2014. PMID: 25037630 Free PMC article. Clinical Trial.

References

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