WBR0171

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Author [[PageAuthor::Yazan Daaboul, M.D. (Reviewed by Serge Korjian)]]
Exam Type ExamType::USMLE Step 1
Main Category MainCategory::Pathology
Sub Category SubCategory::Reproductive
Prompt [[Prompt::A 38-year-old woman, G3P2A0, presents to the emergency department at 34 weeks gestation with complaints of severe nausea and dull right upper abdominal pain. Her past medical history is significant for preeclampsia in her previous pregnancy warranting premature delivery. On arrival, her heart rate is 94/min, blood pressure is 182/98, and temperature is 36.9 °C (98.4 °F). Initial work-up reveals thrombocytopenia, elevated ALT and AST, elevated LDH, low haptoglobin, and 4+ protein on urine dipstick. The physician informs the patient that immediate delivery is recommended. What is the most likely cause of mortality in this patient's condition?]]
Answer A AnswerA::GI bleeding
Answer A Explanation AnswerAExp::GI bleeding is not a very common cause of mortality in patients with HELLP syndrome.
Answer B AnswerB::Myocardial infarction
Answer B Explanation AnswerBExp::Myocardial infarction is not a very common cause of mortality in patients with HELLP syndrome.
Answer C AnswerC::Uterine bleeding
Answer C Explanation AnswerCExp::Uterine bleeding is not a very common cause of mortality in patients with HELLP syndrome. Uterine atony is a common cause of uterine bleeding post-delivery that may be associated with mortality.
Answer D AnswerD::Hematologic malignancy
Answer D Explanation AnswerDExp::Hematologic malignancies, such as lymphomas and leukemias, are not associated with HELLP syndrome and are not a major cause of mortality in these patients.
Answer E AnswerE::Intracranial hemorrhage
Answer E Explanation AnswerEExp::Intracranial hemorrhage is a common cause of death in patients with HELLP syndrome. It is thought to be due to the combination of thrombocytopenia and severe hypertension.
Right Answer RightAnswer::E
Explanation [[Explanation::The patient in this scenario is most likely presenting with HELLP (hemolysis, elevated liver enzymes, and low platelets) syndrome. The disease is classically described as a complication of preeclampsia, despite a recent trend considering HELLP as one extreme on the pregnancy-related hypertensive disease spectrum. HELLP is a life-threatening condition that most commonly manifests in pregnant women between 32-34 weeks of gestation, classically those with diagnosed preeclampsia. Similar to this scenario, patients with HELLP syndrome present with right upper abdominal pain with non-specific symptoms, such as fatigue and nausea. The hepatic lesions (microthrombi and fibrin clots) associated with HELLP syndrome lead to obstruction of the circulation and liver swelling with tension of the Glisson capsule, causing abdominal pain. Serum haptoglobin is important for the detection of early hemolysis. Usually an elevation in liver enzymes precedes the decrease in platelets. Patients who are at 32 weeks gestation or more are usually recommended to deliver as soon as the diagnosis is made. The most common causes of mortality in patients with HELLP syndrome are intracranial hemorrhage and ARDS.

Educational Objective: Intracranial hemorrhage and ARDS are the most common causes of mortality in patients with HELLP syndrome.
References: Rath W, Faridi A, Dudenhausen JW. HELLP syndrome. J Perinat Med. 2000;28(4):249-60.
Suarez B, Alves K, Senat MV, et al. Abdominal pain and preeclampsia: sonographic findings in the maternal liver. J Ultrasound Med. 2002;21(10):1077-83.]]

Approved Approved::Yes
Keyword WBRKeyword::HELLP, WBRKeyword::HELLP Syndrome, WBRKeyword::Pregnancy, WBRKeyword::Complications, WBRKeyword::Preeclampsia, WBRKeyword::Hemolysis, WBRKeyword::Thrombocytopenia, WBRKeyword::Platelets, WBRKeyword::LFTs, WBRKeyword::Liver
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