A 28-year-old woman, gravida 1 para 0, at 35 weeks gestation comes to the emergency department due to nausea, vomiting, and right upper quadrant pain. The patient's symptoms began last night but have progressively worsened throughout the day. Temperature is 37.2 C (99 F), blood pressure is 160/94 mm Hg, and pulse is 80/min. Physical examination shows right upper quadrant tenderness without rebound or guarding. Fetal heart rate tracing shows a baseline of 140 with moderate variability, no decelerations, and no accelerations. Cardiopulmonary examination is unremarkable. The abdomen is tender to palpation over the right upper quadrant; the uterus is nontender. There is 2+ edema of the lower extremities. Laboratory results are as follows: Which of the following is the most likely cause of this patient's presentation?

Question

A 28-year-old woman, gravida 1 para 0, at 35 weeks gestation comes to the emergency department due to nausea, vomiting, and right upper quadrant pain.  The patient's symptoms began last night but have progressively worsened throughout the day.  Temperature is 37.2 C (99 F), blood pressure is 160/94 mm Hg, and pulse is 80/min.  Physical examination shows right upper quadrant tenderness without rebound or guarding.  Fetal heart rate tracing shows a baseline of 140 with moderate variability, no decelerations, and no accelerations.  Cardiopulmonary examination is unremarkable.  The abdomen is tender to palpation over the right upper quadrant; the uterus is nontender.  There is 2+ edema of the lower extremities.  Laboratory results are as follows:  Which of the following is the most likely cause of this patient's presentation?

Quizplus · Accepted Answer

11ec25c8_da73_50f3_bc81_8f691f20cd97_MD0003_00 This patient at 35 weeks gestation likely has HELLP (Hemolysis, Elevated Liver enzymes, Low Platelets) syndrome, a life-threatening disorder related to preeclampsia with severe features. Similar to preeclampsia, the etiology is likely abnormal placental development during early pregnancy with the placental release of antiangiogenic factors; these abnormalities cause widespread maternal endothelial dysfunction and dysregulation of vascular tone (eg, new-onset hypertension). abnormal placental development HELLP syndrome is associated with systemic microangiopathy and excessive platelet consumption (likely due to severe endothelial injury) that leads to overactivation of the coagulation cascade and thrombi formation in the microvasculature. As platelets are consumed and platelet-fibrin thrombi form, patients develop thrombocytopenia and shearing of red blood cells by the widespread thrombi, resulting in microangiopathic hemolytic anemia (MAHA) (as evidenced by this patient's indirect hyperbilirubinemia), which is detrimental to the liver. The hepatocellular injury (from vasospasm and/or hepatic portal system thrombi) leads to distension of the hepatic (Glisson) capsule, causing worsening right upper quadrant pain with nausea, vomiting, and elevated transaminases. Other potential complications include liver rupture, eclampsia, and intrauterine fetal demise. Patients with HELLP syndrome require prompt delivery because removal of the placenta is the only definitive cure. (Choice A) Acute fatty liver of pregnancy, the microvesicular fatty infiltration of hepatocytes, can cause elevated liver transaminases and thrombocytopenia. However, patients typically have fulminant hepatic failure (eg, prolonged prothrombin time and partial thromboplastin time, profound hypoglycemia), which is not seen in this patient. (Choice B) Immune thrombocytopenic purpura typically causes isolated thrombocytopenia and is a diagnosis of exclusion. It is not associated with MAHA. (Choice C) Intrahepatic cholestasis of pregnancy causes generalized pruritus, hyperbilirubinemia, and elevated transaminases due to impaired bile acid flow. However, it is not associated with hypertension, MAHA, or thrombocytopenia. (Choice D) Premature placental separation (ie, abruptio placentae) most commonly occurs in patients with preeclampsia or HELLP syndrome. It classically presents with severe uterine tenderness and fetal compromise due to decreased fetoplacental perfusion. In contrast, this patient has right upper quadrant pain, no uterine tenderness, and a reassuring fetal heart rate tracing (eg, normal baseline, moderate variability). Educational objective: HELLP (Hemolysis, Elevated Liver enzymes, Low Platelets) syndrome can present with right upper quadrant pain, microangiopathic hemolytic anemia, elevated transaminases, and thrombocytopenia. The pathogenesis involves widespread endothelial dysfunction leading to microangiopathy, overactivation of the coagulation cascade, and excessive platelet consumption. References: The differential diagnosis of thrombocytopenia in pregnancy. (http://www.ncbi.nlm.nih.gov/pubmed/26634939) Clinical characteristics and pregnancy outcomes of atypical hemolysis, elevated liver enzymes, and low platelets syndrome: a case series. (http://www.ncbi.nlm.nih.gov/pubmed/32358352)

A 28-Year-Old Woman, Gravida 1 Para 0, at 35 Weeks