A Rare Cause of Elevated Liver Tests in a Pregnant Patient

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Case: 26-year-old female at 22 weeks gestation with history of anemia presented with nausea, vomiting and sharp, epigastric, non-radiating abdominal pain for 4 days. She denied any bleeding. Surgical and medication history were notable for a previous dilation and curettage and a daily vitamin. Vital signs were normal, physical exam revealed pallor, gravid abdomen with epigastric tenderness to palpation. Hemoglobin was 6.8 g/dL and platelets 84 K/mcL. Liver panel showed AST 87 U/L, ALT 42 U/L and total bilirubin 3.2 mg/dL (direct 0.50 mg/dL). Reticulocytes were 8%, LDH 1142 U/L, haptoglobin undetectable with negative direct antiglobulin test. Peripheral smear showed macrocytosis, thrombocytopenia, anisopoikilocytosis, and no schistocytes. Acute viral hepatitis panel and parvovirus serologies were negative. Doppler ultrasound showed splenomegaly, normal fetus and liver with near occlusive thrombus of the middle hepatic vein with reversal of flow in the right portal vein. CT abdomen/pelvis revealed heterogeneous enhancement of liver with filling defects in multiple hepatic veins suggestive of thrombi. ADAMST13 level, protein C and S, antithrombin, glucose-6-phosphate dehydrogenase levels, factor V Leiden, Beta-2 glycoprotein, ANA, ASMA, SPEP and AMA were all negative. Endoscopy was negative for portal hypertensive gastropathy or varices. Duodenal biopsies were normal. Flow cytometry showed PNH clone percent of 84% in granulocytes, 68% in monocytes, and type III RBC (complete CD59 deficiency) of 8% confirming diagnosis of PNH. The patient was transfused, started on anticoagulation and eculizumab. Her anemia improved and she delivered a healthy, full-term baby. Discussion: PNH is characterized by a triad of intravascular hemolysis, thrombosis and bone marrow failure. Thrombosis occurring in atypical locations should prompt consideration of PNH. The hepatic vein is the most frequently involved vessel. PNH is associated with increased fetomaternal morbidity and mortality and thrombosis is the leading cause of death. Unexplained hemolytic anemia in a pregnant patient with concomitant abnormal liver tests may initially evoke diagnosis of HELLP (hemolysis, elevated liver enzymes, and low platelets), however, management of the two condition differs vastly. Anticoagulation is recommended for pregnant women with PNH thrombosis unless contradicted.Eculizumab reduces the risk of thrombosis. BMT is the only curative therapy for PNH with success rates of up to 100%.