A Rare Case of Hemophagocytic Lymphohistiocytosis in Pregnancy With Clinical Features Resembling Acute Fatty Liver of Pregnancy

Abstract

Hemophagocytic lymphohistiocytosis (HLH) during pregnancy is a rare but critical condition that is difficult to diagnose due to its complex and nonspecific presentation. We report a case of HLH in a 27-year-old woman at 32 weeks of gestation. She presented with persistent fever and liver dysfunction, leading to a suspected diagnosis of HELLP syndrome and an emergency cesarean section. However, her condition deteriorated after delivery, with the development of pancytopenia, coagulopathy, hemophagocytosis, and fatty liver. These findings raised suspicion of HLH or acute fatty liver of pregnancy (AFLP). A comprehensive diagnostic workup, including liver biopsy and genetic testing, confirmed the diagnosis of HLH. HLH during pregnancy often mimics obstetric complications such as AFLP, underscoring the importance of careful differential diagnosis.

Keywords: acute liver failure (alf); aflp; hemophagocytic lymphohistiocytosis (hlh); plasma exchange therapy; coagulopathy.

Figure 1. Non-contrast CT image of the upper abdomen

Figure 1A was obtained at the time of cesarean section, and Figure 1B at the time of transfer to our ICU. In both images, the liver-to-spleen attenuation ratio was below 0.9, indicating fatty liver. The hepatic attenuation in Figure 1B was further reduced compared to Figure 1A. CT: Computed tomography

Figure 2. Contrast-enhanced CT after liver biopsy showing hemorrhagic ascites

The images show high-density fluid consistent with hemorrhagic ascites surrounding the liver, suggestive of intra-abdominal bleeding. No obvious active extravasation was noted. CT: Computed tomography

Figure 3. Contrast-enhanced CT showing active extravasation from the left uterine artery

The images demonstrate active extravasation of contrast medium from the left uterine artery, consistent with ongoing arterial bleeding. CT: Computed tomography

Figure 4. Timeline of the clinical course, laboratory results, and treatments

UAE: Uterine artery embolization, T-Bil: Total bilirubin, FFP: Fresh frozen plasma, RBC: Red blood cell concentrate, PC: Platelet concentrate, PE: Plasma exchange, WBC: White blood cell, Hb: Hemoglobin, Plt: Platelet, AST: Aspartate aminotransferase, ALT: Alanine aminotransferase.