Case Report
A 64-year-old woman presented to the emergency department with fever and dark urine of one week’s duration. Two years earlier, PNH was diagnosed, based on flow cytometry findings of peripheral blood. Although thrombocytopenia had been present, no specific therapy was necessary. Her past medical history included hyperlipidemia and Helicobacter pylori -induced gastritis; H. pylori had been eradicated. She had a temperature of 38.2℃ and tenderness in the left costovertebral angle. Laboratory tests revealed severe anemia, thrombocytopenia, and coagulopathy (Table 1). C-reactive protein was 33.2 mg/dL [normal range, 0.00–0.14], lactate dehydrogenase 802 U/L [124–222], and serum creatinine 3.81 mg/dL [0.46–0.79]. Blood and urine cultures were negative, probably because she had taken oral antibiotics before presentation. Bacterial infection-triggered hemolysis, possibly from pyelonephritis, was suspected.
She was admitted, and we started intravenous administration of prednisolone, lansoprazole, and ceftriaxone (Fig. 1). Blood products, including haptoglobin, were also given. Two days later, hematologic and inflammatory markers improved; however, she developed upper abdominal pain and vomiting. Computed tomography (CT) revealed marked thickening of the duodenal wall (Fig. 2). Esophagogastroduodenoscopy (EGD) revealed patchy redness of the gastric mucosa (Fig. 3a) and a large punched-out ulcer in the duodenum (Fig. 3b), with clear demarcation between normal and affected mucosa. Biopsy specimens had no specific findings ― malignant cells, granuloma, nor viral inclusion bodies. Thus, we suspected transient ischemia due to PNH-related thrombosis had caused the gastroduodenal injury.
Because there were no signs suggestive of bowel obstruction, perforation, or uncontrolled bleeding, we continued therapy for PNH, with corticosteroids, proton pump inhibitor, and diet control. Her symptoms improved in a few days. In a follow-up EGD performed 3 weeks after admission, the gastric patchy redness had disappeared, and the duodenal ulcer had improved (Fig. 4). In laboratory tests, fibrin-related markers also normalized (Table 1). After vaccination against meningococcus, eculizumab was added to the therapy to achieve better control of the PNH.