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 conf irm or exclude these syndromes. The paper describes a case of HELLP syndrome or aHUS, which has caused substantial difficulties in their differential diagnosis. Case report. Patient O. aged 33years 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 of650 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 x 109/l; hemoglobin, 70 g/l; red blood cells, 2.24 x 1012/l; and platelets, 62 x 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.

HELLP syndrome, thrombotic microangiopathy, atypical hemolytic uremic syndrome, thrombotic thrombocytopenic purpura, microangiopathic hemolytic anemia, thrombocytopenia, plasmapheresis, plasma exchange, hemodiafiltration