Atypical HELLP syndrome or atypical hemolytic uremic syndrome?
Background. Despite the fact that maternal mortality associated with HELLP syndrome has declined considerably during the last decades, now it remains rather high and ranges from 0 to 24%; perinatal mortality is very high, amounting to 8 to 60%. The HELLP syndrome is very often subtle, without manifestations of a classic symptom triad. In this case, clinicians always raise a question as to whether this diagnosis is justifiable. There is the greatest difficulty in the differential diagnosis of HELLP syndrome and atypical hemolytic uremic syndrome (aHUS) due to their very similar clinical and laboratory manifestations and to the lack of clear clinical and laboratory markers used to confirm or exclude these syndromes. The paper describes a case of HELLP syndrome or aHUS, which has caused substantial difficulties in their differential diagnosis.Kalachin K.A., Pyregov A.V., Fedorova T.A., Grishchuk K.I., Shmakov R.G.
Case report. Patient O. aged 33 years was admitted to the V.I. Kulakov Research Center of Obstetrics, Gynecology, and Perinatology on January 19, 2016. Two previous pregnancies were uneventful and ended favorably; this was her third pregnancy. The patient had episodes of high blood pressure (BP) in the second trimester and received therapy. Edema in the hands appeared at 36 weeks’ gestation. Her total weight gain was 24 kg. On January 20, the patient gave birth to a full-term live girl with an Apgar score of 7/8. The patient had a total blood loss of 650 ml. In the early postpartum period, she developed hypotension and tachypnea; brown-red urine was taken using a urinary catheter; the results of her analyses were as follows: leukocytes, 18.28 × 109/l; hemoglobin, 70 g/l; red blood cells, 2.24 × 1012/l; and platelets, 62 × 109/l. She was transferred to an intensive care unit and started intensive therapy. On the first day of her stay there, the patient had oligoanuria, she underwent a hemodiafiltration session; the subsequent urine output rate remained normal. She also had three plasma exchange sessions and one plasmapheresis session. Despite positive changes in the levels of platelets, lactate dehydrogenase, and aspartate aminotransferase, there was a continuing rise in creatinine and urea with the normal urine output rate. This fact could put in doubt the diagnosis of HELLP syndrome and think about the diagnosis of aHUS; however, the values of creatinine and urea began to normalize after the fourth plasmapheresis session and a week later the levels of these indicators were 1583 µmol/l and 6.9 mmol/l, respectively.
Conclusion. The paper reviews the literature on the simulators for HELLP syndrome and a variant of differential diagnosis if a pregnant woman is found to have thrombocytopenia.
Keywords
HELLP syndrome
thrombotic microangiopathy
atypical hemolytic uremic syndrome
thrombotic thrombocytopenic purpura
microangiopathic hemolytic anemia
thrombocytopenia
plasmapheresis
plasma exchange
hemodiafiltration
Supplementary Materials
- Fig. 1. Dynamics of indicators of biochemical blood test: lactate dehydrogenase (U/l) aspartate aminotransferase (U/l)
- Fig. 2. Dynamics of average daily rate diuresis (ml/kg/HR), and biochemical analysis of blood: urea (mmol/l), creatinine (µmol/DL)
- Fig. 3. Dynamics of indexes of general blood analysis: platelets and thrombocytes by Fonio (x 10^9/L), hemoglobin (g/l), schistocytosis (PCs per 10000 erythrocytes)
- Table 1. Differential diagnosis between HELLP syndrome, acute fatty liver of pregnant women, thrombotic thrombocytopenic purpura and the atypical hemolytic uremic syndrome.
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Received 26.08.2016
Accepted 02.09.2016
About the Authors
Kalachin Konstantin A., anesthesiologist-resuscitator, Research Center of Obstetrics, Gynecology, and Perinatology, Ministry of Health of Russia.117997, Russia, Moscow Ac. Oparina str. 4. Tel.: +79169186789. E-mail: Konstantin.Kalachin@yandex.ru
Pyregov Alexey Viktorovich, MD, Head of the Department of Anesthesiology and Resuscitation, Research Center of Obstetrics, Gynecology, and Perinatology,
Ministry of Health of Russia. 117997, Russia, Moscow Ac. Oparina str. 4. Tel.: +79161406549. E-mail: pyregov@mail.ru
Fedorova Tatiana A., MD, Professor, Head of the Department of Transfusion and extracorporeal blood correction, Research Center of Obstetrics, Gynecology, and Perinatology, Ministry of Health of Russia. 117997, Russia, Moscow Ac. Oparina str. 4. Tel.: +79859243585. E-mail: t_fedorova@oparina4.ru
Grischuk Konstantin I., anesthesiologist-resuscitator, Research Center of Obstetrics, Gynecology, and Perinatology, Ministry of Health of Russia.
117997, Russia, Moscow Ac. Oparina str. 4. Tel.: +79295508374. E-mail: K_grischuk@oparina4.ru
Shmakov Roman Georgievich, chief physician, Research Center of Obstetrics, Gynecology, and Perinatology, Ministry of Health of Russia.
117997, Russia, Moscow Ac. Oparina str. 4. Tel.: +79255859171. E-mail: r_shmakov@oparina4.ru
For citations: Kalachin K.A., Pyregov A.V., Fedorova T.A., Grishchuk K.I., Shmakov R.G. Atypical HELLP syndrome or atypical hemolytic uremic syndrome? Akusherstvo i Ginekologiya/Obstetrics and Gynecology. 2017; (1): 94-102. (in Russian)
http://dx.doi.org/10.18565/aig.2017.1.94-102
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