Zespół HELLP – patogeneza i postępowanie kliniczne

Abstract

The HELLP syndrome is a serious complication of pregnancy characterized by haemolysis, elevated liverenzymes and low platelet count. In the past it was thought to be related to severe preeclampsia, nowadays itis believed to be a separate disease. Its pathogenesis remains to be elucidated – the importance of genetic andimmunological origins is highlighted. This review presents pathogenesis, diagnosis and management in patientswith the HELLP syndrome. About 70% of the cases develop before delivery, the majority between the 24th and37th gestational weeks; the remainder (rest) within 48 hours after delivery. The HELLP syndrome may be completeor incomplete. In the Tennessee Classification System, diagnostic criteria for HELLP are haemolysis with increasedLDH (> 600 U/L), AST (≥ 70 U/L), and platelets < 100 000/mm3. The Mississippi Triple-class HELLP Systemfurther classifies the disorder by the nadir platelet counts. The syndrome is a progressive condition and seriouscomplications are frequent. Delivery is indicated if the HELLP syndrome occurs after the 34th gestational week orthe foetal and/or maternal conditions (status) deteriorate. Conservative treatment (≥ 48 hours) is controversialbut may be considered in selected cases before 34 weeks' gestation. Most authors prefer a single course ofcorticosteroid therapy for RDS prophylaxis between 24th and 34th week of pregnancy. Standard corticosteroidtreatment is still of uncertain clinical value in the management of the maternal HELLP syndrome, but justifiedin some cases, mainly with severe thrombocytopenia. Blood pressure should be kept below 155/105 mm Hgas well as the therapy with MgSO4 should be offered to all patients with the HELLP syndrome.

Keywords

• HELLP syndrome
• pathophysiology
• diagnosis
• corticosteroid therapy
• delivery