Hemolysis, elevated liver enzymes, and low platelet count (HELLP) syndrome is a severe complication in the third trimester of pregnancy. First described by Weinstein in 1982(1)
- HELLP syndrome occurs in up to 0.9% of all pregnancies in the third trimester or in the immediate postpartum period (2)
Associated with adverse pregnancy outcomes, the maternal and perinatal mortality rates of HELLP syndrome may reach up to 23.1% and 56.9% respectively, which is life threatening to both the gravida and the fetus(3)
- Usually, patients develop HELLP syndrome before 36 weeks of gestation with vague symptoms such as malaise (90%), right-upper-quadrant pain (90%), nausea, or vomiting(4)
Preeclampsia (PE) constitutes one of the principal reasons for maternal and perinatal morbidity and mortality worldwide. The circumstance typically implicates formerly healthful normotensive women, after 20 weeks of gestation, typically withinside the third trimester, without regarded threat elements or past deliveries. PE can be further complicated with hemolysis and thrombocytopenia, leading to the emergence of HELLP syndrome. Both conditions are classified as hypertensive diseases of pregnancy (HDP), and their pathogenesis has been linked to an excessive maternal inflammatory response, accompanied by enhanced endothelial activation(5)
. The pathogenesis of HELLP is not fully understood. The findings of this multisystem disease are attributed to abnormal vascular tone, vasospasm and coagulation defects. So far, no common precipitating factor has been found. The syndrome seems to be the final manifestation of some insult that leads to microvascular endothelial damage and intravascular platelet activation (6)
- With platelet activation, thromboxane A and serotonin are released, causing vasospasm, platelet agglutination and aggregations, and further endothelial damage. Thus begins a cascade that is only terminated with delivery(7)
Early detection and accurate diagnosis are essential for correct management. The maternal symptoms may be vague and easily mistaken for a variety of medical or obstetric complications which should be excluded. There are two main diagnostic definitions of the HELLP syndrome. The widely used Tennessee classification requires the presence of (1) microangiopathic hemolytic anemia with abnormal blood smear, low serum haptoglobin and elevated LDH levels, (2) elevation of ASAT above 70 IU/L and LD above 600 IU/L (both enzyme levels more than twice the upper limit of normal values) or bilirubin more than 1.2 mg/dL, and (3) a platelet count below 100 109 L1(8) . HELLP syndrome implies impaired placentation during the early stages of pregnancy, associated with hepatic and coagulation cascade involvement. Coagulation system is activated by the contact of platelets with the injured endothelium leading to increase in consumption as well as bone marrow production of platelets. Raised mean platelet volume (MPV) and platelet distribution width (PDW) have been shown to be correlated with severity of the disease, whenever there is decreased PLT count (9)
. The inflammatory reaction in HELLP syndrome is more severe, but studies on the levels of inflammatory indices [e.g., neutrophil-to-lymphocyte ratio (NLR), platelet-to-lymphocyte ratio (PLR), MPV, PDW, monocyte-to-lymphocyte ratio (MLR)] in HELLP patients have rarely been reported. As NLR, calculated by dividing the absolute neutrophil count by the lymphocyte count, reflects the balance between innate (neutrophil-mediated) and adaptive (lymphocyte-mediated) immunity, with elevated values indicating heightened inflammatory activity. PLR, obtained by dividing platelet count by lymphocyte count, integrates the pro-thrombotic and inflammatory roles of platelets with immune regulation. MLR, the ratio of monocytes to lymphocytes, is considered a marker of chronic inflammation and monocyte activation. MPV represents the average size of circulating platelets and serves as an indicator of platelet activation, while PDW measures the variability in platelet size reflecting heterogeneity in platelet production and turnover (10)
. Sisti et al (11, 12)found that the NLR was higher and the PLR was lower in patients with HELLP syndrome in the third trimester, but these indices in the first trimester did not predict the occurrence of HELLP in the third trimester. Only a relatively small number of cases were included in previous studies on this topic and no simultaneous comparison of normal pregnancies and HELLP was found (13, 14)
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