HELLP Syndrome at 17 Weeks Gestation: A Rare and Catastrophic Phenomenon

Abstract

HELLP syndrome is a collection of symptoms described as hemolysis, elevated liver enzymes and low platelets. HELLP syndrome complicates 0.01-0.6% of pregnancies and can be considered a severe variant of preeclampsia. The occurrence of HELLP syndrome diagnosed before the 20th week of gestation has been most commonly reported in association with antiphospholipid antibody syndrome (APS) or triploid chromosomal anomalies. A 41-year-old primigravida was admitted at 17 weeks and 6 days gestation with hypertension, proteinuria, hemolytic anemia and acute renal injury. She was diagnosed with HELLP syndrome, and subsequently suffered from an intrauterine fetal demise. After delivery, the clinical manifestations of HELLP syndrome resolved within 7 days with the exception of her acute renal failure. Interdisciplinary teams of physicians were able to exclude other imitators of preeclampsia, such as hemolytic uremic syndrome (HUS), thrombotic thrombocytopenic purpura (TTP), APS, lupus and acute fatty liver of pregnancy. This case is difficult to diagnose, given the similar presentation of several microangiopathic hemolytic anemias. The clinical manifestations and laboratory findings of HELLP and its mimicking conditions seem as if they are mirror images of each other. However, the discrete differences in our patient presentation, clinical findings, laboratory results and overall postpartum course leave HELLP syndrome as the most consistent diagnosis. It is imperative to investigate for all possible etiologies as HELLP syndrome at 17 weeks gestation is extremely rare and mimicking conditions may require alternative management strategies.

Recurrent HELLP Syndrome at 22 Weeks of Gestation

“…She was anticardiolipin antibody-positive. The higher age of this patient is consistent with our case and another case of a 41-year-old primigravida with severe HELLP syndrome at GA of 17 weeks [ 11 ].…”

Pancreatic carcinoma underlying a complex presentation in late pregnancy: a case report

“…The triad of HUS is classically described as thrombocytopenia, microangiopathic hemolytic anemia, and renal dysfunction. The addition of fever and neurologic symptoms makes the classic pentad of TTP [12]. In this case, a physical examination of our patient revealed hypertension but no evidence of purpura or petechia, neurologic abnormalities, or edema.…”

Preeclampsia: The Need for a Biological Definition and Diagnosis

“…27 Without a doubt, preeclampsia manifesting before 20 weeks' gestation is a rare occurrence but not impossible. In an ad hoc literature search limited to the period 2003 to 2017, we identified 11 published case reports of preeclampsia developing before 20 weeks' gestation, [28][29][30][31][32][33][34][35][36][37][38] many confirmed by renal biopsy and five of them presenting as HELLP syndrome, an entity with reported median gestational age of 36 weeks at presentation. 39 It has also been suggested that antiphospholipid syndrome may predispose to preeclampsia before 20 weeks' gestation.…”