Kidney Week

Abstract: SA-PO377

Successful Pregnancies During Ongoing Complement Blockade in Two Patients with Complement Mediated Thrombotic Microangiopathy

Session Information

• Genetic and Diagnostic Trainee Case Reports
  November 09, 2019 | Location: Exhibit Hall, Walter E. Washington Convention Center
  Abstract Time: 10:00 AM - 12:00 PM

Category: Trainee Case Report

• 2000 Women's Health and Kidney Diseases

Authors

• Haninger-Vacariu, Natalja, Medical University of Vienna, Vienna, Austria

Natalja Haninger-Vacariu,

• Gaggl, Martina M., Medical University of Vienna, Vienna, Austria

Martina M. Gaggl,

• Prohaszka, Zoltan, Semmelweis University, Budapest, Hungary

Zoltan Prohaszka,

• Aigner, Christof, Medical University of Vienna, Vienna, Austria

Christof Aigner,

• Kain, Renate, Medical University of Vienna, Vienna, Austria

Renate Kain,

• Bohmig, Georg, Medical University of Vienna, Vienna, Austria

Georg Bohmig,

• Piggott, Leah C., Medical University of Vienna, Vienna, Austria

Leah C. Piggott,

• Sunder-Plassmann, Raute, Medical University of Vienna, Vienna, Austria

Raute Sunder-Plassmann,

• Sunder-Plassmann, Gere, Medical University of Vienna, Vienna, Austria

Gere Sunder-Plassmann,

• Schmidt, Alice, Medical University of Vienna, Vienna, Austria

Alice Schmidt,

Introduction

In patients with pregnancy-associated complement gene variant mediated thrombotic microangiopathy (cTMA) terminal complement blockade is used for treatment of cTMA flares during pregnancy or following delivery. Data on pregnancies of cTMA patients during ongoing eculizumab (ECU) therapy, however, are scarce.

Case Description

We report pregnancy and delivery outcomes of two cTMA patients enrolled in the Vienna TMA cohort and measured complement related proteins and ECU concentrations at regular intervals during pregnancy, thereafter, and in cord blood.
The first manifestation of cTMA occurred in both patients during childhood or young adulthood and was not related to pregnancy. One patient (genetic variants in CFI, CD46, CFH, C3) had a total history of 26 cTMA flares and of two uneventful pregnancies with prophylactic plasma infusions. She started ECU at her last cTMA flare, which was continued during her third pregnancy at the age of 27 yrs. The other patient (genetic variants in CFH, CD46), 29 yrs of age at her second pregnancy, had a history of recent early abortion during long-term ECU therapy following kidney transplantation, which was performed four years after her first manifestation of cTMA. ECU plasma concentrations were maintained in the therapeutic range during both successful pregnancies and were also detectable in the cord blood. Complement related tests did not indicate alternative pathway activation during pregnancies. Kidney function and blood pressure did not change substantially in both cases. However, proteinuria increased at the end of the third trimester in both patients. Both neonates were adequate for gestational age (weight: 3720 and 3082g; head circumference: 35 and 34 cm; length: 52 and 50 cm) with vaginal delivery in week 40+3 and 37+0 of gestation, respectively. Results of complement related tests in cord blood showed deficient complement activity, with low factor and regulator levels without overactivation, which most likely reflects the situation related to age and the presence of ECU in cord blood.

Discussion

Pregnancy and delivery outcomes with ongoing ECU therapy in two genetically high-risk cTMA patients with preserved native kidney and kidney transplant function were excellent.