Clinical observation of a patient with atypical hemolytic-uremic syndrome, complicated by pre-eclampsia and HELLP syndrome
AbstractPatient L., 28 years old, was admitted to the intensive care unit (ICU) of Clinical Hospital “Lapino” on September 23, 2021 at 14:53 with complaints of an increase in blood pressure (BP) to 235 and 140 mm Hg, loss of consciousness in the car for 2–3 minutes, swelling of the lower extremities, weakness, seeing dark spots in one’s vision, blurred vision at 24 weeks and 4 days of pregnancy.
From the anamnesis it is known: in 2015, a cyst and hypoplasia of the left kidney, nephroptosis on the right according to ultrasound data were diagnosed. Since 2018, she has repeatedly undergone inpatient treatment in the nephrology department with a diagnosis of chronic kidney disease, chronic glomerulonephritis (grade 3) hypertension, stage 1, risk 4.
According to MRI of the brain from 2015: aneurysm of the internal carotid artery, without surgical treatment. The previous 2 pregnancies were spontaneous, occurred against the background of increased blood pressure and ended with operative childbirth at 38 weeks and the birth of healthy children.
This pregnancy is the third, spontaneous one.
This is the third pregnancy, spontaneous. In the second trimester, episodes of increased blood pressure were noted, therapy was not taken in full. From the 24th week swelling of the hands appeared. Complex intensive therapy was performed in ICU conditions. Due to the lack of effect from the therapy, she was urgently submitted to the operating room.
At 18:30 on September 24, 2021, a dead premature male fetus weighing 480 g, 31 cm long was born in the fetal bladder. Upon opening the fetal bladder, 50 ml of meconial amniotic fluid was poured out. Blood loss was 1000 ml (1.6% of body weight). In the early postoperative period improvement was noted.
On September 28, 2021, on the fourth day after delivery, an emergency median relaparotomy, revision, and drainage of the abdominal cavity were performed. 09/29/2021, 10/05/2021, 10/12/2021, 10/19/2021, pathogenetic complement-blocking therapy with eculizumab (elizaria) was performed in an induction regimen of 900 mg once a week for 4 weeks, and on 10/26/2021 and 11/09/2021 – in a maintenance regimen (1200 mg per week, then 1200 mg every 2 weeks). Due to the lack of positive dynamics from conservative treatment, persistent fever up to 39 °C, on October 15, 2021, diagnostic laparoscopy, sanitation and drainage of the abdominal cavity were performed. Antihypertensive therapy, antibacterial, infusion, diuretic, and anticoagulant therapy were carried out.
A total of 30 doses of freshly frozen plasma (FFP), 22 doses of erythrocyte mass, 60 doses of albumin 20% were transfused. From 10/09/2021 to 10/28/2021, 15 hemodiafiltration procedures were carried out, and daily plasma exchange procedures were carried out from 09/24/2021. The patient was discharged on 11/10/2021 in satisfactory condition to go home with clinical recommendations.
The presented clinical case will make it possible to identify risk groups among pregnant women, develop differential diagnostics, and develop treatment algorithms for the group of patients with complications under consideration. A review of the clinical example shows that timely diagnosis and adequate treatment determine a favorable prognosis.
Keywords: HELLP syndrome; thrombotic microangiopathy; atypical hemolytic-uremic syndrome; thrombocytopenia; hemodiafiltration; plasma exchange
Funding. The study had no sponsor support.
Conflict of interest. The authors declare no conflict of interest.
For citation: Kurtzer M.A., Spiridonova E.I., Khvatova A.V., Konoplev B.A., Bagdasaryan P.M., Kotenko O.N., Kutakova Yu.Yu., Semenova T.Yu., Kurbatskaya O.N., Petrulevich R.A., Medvedskaya A.K. Clinical observation of a patient with atypical hemolytic-uremic syndrome, complicated by pre-eclampsia and HELLP syndrome. Akusherstvo i ginekologiya: novosti, mneniya, obuchenie [Obstetrics and Gynecology: News, Opinions, Training]. 2024; 12 (2): 70–9. DOI: https://doi.org/10.33029/2303-9698-2024-12-2-70-79 (in Russian)
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