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.