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CC BY-NC-ND 4.0 · AJP Rep 2024; 14(01): e96-e100
DOI: 10.1055/a-2164-8438
Case Report

Pregnancy-Associated Atypical Hemolytic Uremic Syndrome: A Case Report with MCP Gene Mutation and Successful Eculizumab Treatment

Alex Domínguez-Vargas 
1   División Ciencias de la Salud, Universidad del Norte, Barranquilla, Colombia
2   Facultad de Ciencias de la Salud, Universidad Simón Bolívar, Barranquilla, Colombia
,
Fanny Ariño
2   Facultad de Ciencias de la Salud, Universidad Simón Bolívar, Barranquilla, Colombia
,
Diana Silva
2   Facultad de Ciencias de la Salud, Universidad Simón Bolívar, Barranquilla, Colombia
,
Henry J. González-Tórres
2   Facultad de Ciencias de la Salud, Universidad Simón Bolívar, Barranquilla, Colombia
,
Gustavo Aroca-Martinez
1   División Ciencias de la Salud, Universidad del Norte, Barranquilla, Colombia
2   Facultad de Ciencias de la Salud, Universidad Simón Bolívar, Barranquilla, Colombia
3   Departamento de Nefrología, Clínica de la Costa, Barranquilla, Colombia
,
Eduardo Egea
1   División Ciencias de la Salud, Universidad del Norte, Barranquilla, Colombia
,
Carlos G. Musso
2   Facultad de Ciencias de la Salud, Universidad Simón Bolívar, Barranquilla, Colombia
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Abstract

Pregnancy-associated atypical hemolytic uremic syndrome (P-aHUS) is a rare condition characterized by microangiopathic hemolytic anemia and kidney injury from thrombotic microangiopathy. P-aHUS occurs in approximately 1 in 25,000 pregnancies and is strongly related to complement dysregulation and pregnancy-related disorders, such as preeclampsia, eclampsia, and hemolysis, elevated liver enzymes, low platelet (HELLP) syndrome, resulting in adverse perinatal and fetal outcomes. Complement dysregulation in P-aHUS is commonly attributed to genetic mutations or autoantibodies affecting complement factors, including CFH, CFI, and MCP. We present a case of a 25-year-old primigravida who experienced severe preeclampsia and HELLP syndrome followed by the development of complicated P-aHUS during the early postpartum period. The patient exhibited severe clinical manifestations, including hypertensive emergency, central nervous system involvement, renal impairment, and microangiopathic hemolytic anemia. Timely initiation of eculizumab therapy resulted in successful disease remission. Further genetic analysis revealed a likely rare pathogenic MCP gene variant.

Keywords

aHUS - pregnancy - thrombotic microangiopathy - eculizumab - HELLP syndrome

Ethical Approval

This study was approved by the Ethics Committee of the Clinica de la Costa, Barranquilla, Colombia. All procedures were performed under the relevant guidelines and regulations.


Informed Consent

Written informed consent was obtained from the patient for publication. There are no identifying images or other personal or clinical details of the patient that compromise her anonymity in this manuscript.


Availability of Data and Materials

The datasets used and/or analyzed during the current study are available from the corresponding author on reasonable request.


Author's Contributions

A.D.V. contributed to the conceptualization and writing of the original draft. H.J.G.T. and F.A. contributed to data curation. D.S. contributed to visualization; E.E., G.A.M., and C.G.M. contributed to writing, review, and editing. All authors read and approved the final manuscript.




Publication History

Received: 27 July 2023

Accepted: 08 August 2023

Accepted Manuscript online:
01 September 2023

Article published online:
21 February 2024

© 2024. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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