a life-threatening disorder related to preeclampsia with severe features (same pathophysiology).
Hemolysis, Elevated Liver enzymes, Low Platelet count
| Clinical findings | • Nausea/vomiting • Right upper quadrant pain without peritoneal signs classically • Headache • Visual changes • Hypertension | | --- | --- | | Laboratory abnormalities | • Microangiopathic hemolytic anemia • Elevated liver enzymes • Thrombocytopenia • ± Proteinuria | | Treatment | • Prompt delivery (removal of the placenta is the only definitive cure) • Magnesium sulfate for seizure prophylaxis • Antihypertensives (eg, hydralazine) | | Complications | • Abruptio placentae • Subcapsular liver hematoma • Liver rupture • Eclampsia • Acute renal failure • Pulmonary edema • Disseminated intravascular coagulation • IUFD |
HELLP syndrome is associated with systemic microangiopathy and excessive platelet consumption (likely due to severe endothelial injury) that leads to over activation 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 gradual distension of the hepatic (Glisson) capsule, causing worsening right upper quadrant pain with nausea, vomiting, and elevated transaminases.
Severe cases may progress to liver necrosis and hemorrhage, resulting in a subcapsular hematoma (ie, blood pooling beneath the hepatic capsule).
(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.