A patient with paroxysmal nocturnal hemoglobinuria being treated with eculizumab who underwent laparoscopic cholecystectomy: report of a case
Patient
A 48-year-old female.
Chief complaint
No symptoms.
Past history
She had developed PNH when she was 39 years old. At the age of 46 years, eculizumab
administration once every 2 weeks was initiated, and neither progression of anemia
nor hemoglobinuria was observed during the administration period.
Present illness
In March 2013, she visited a local hospital due to jaundice and abdominal pain, and
was diagnosed with cholecystolithiasis and choledocholithiasis. She continued administration
of eculizumab once every 2 weeks then for two years. She was referred to the Department
of Gastroenterology. Common bile duct stones were removed using endoscopic sphinterotomy
(EST), and she was then referred to our department for surgery for cholecystolithiasis.
Physical findings
She was 165.3 cm tall and weighed 74.7 kg, and showed a BMI of 27.94, blood pressure
of 127/94 mmHg, pulse of 74 bpm, body temperature of 36.8°C, and no anemia or jaundice
in the conjunctiva.
Blood examination findings
The leukocyte count was 2,930, and the Hb value was 8.2, both showing decreases.
ERCP findings
Radiolucent images in the common bile duct showing aggregation and extension to the
porta hepatis were observed, suggesting many stones (Fig. 1).
Fig. 1. There were many stones accumulating in the common bile duct
CT findings
There were many high-density shadows suggesting gallstones (Fig. 2).
Fig. 2. High-density shadows were observed, suggesting stones in the gallbladder
MRCP findings
After EST, there were no findings suggesting stones in the common bile duct. In the
gallbladder, many stones were observed (Fig. 3).
Fig. 3. After EST, no stones were observed in the common bile duct. There were numerous stones
in the gallbladder
Surgical findings
Eculizumab was administered 10 days before surgery, and laparoscopic cholecystectomy
was performed. The operation time was 1 hour and 59 minutes, and the bleeding volume
was small. No complications occurred during the operation.
Postoperative course
For the prevention of thrombosis, elastic stockings and a foot pump were used without
anticoagulant administration. After the operation, neither the progression of anemia
or hemoglobinuria was observed. Slight increases in D-Bil and LDH with peaks on postoperative
day 3 and a slight decrease in Hb were observed, but rapidly improved (Fig. 4). On postoperative day 5, after eculizumab had been administered as planned, she
showed a favorable general condition, and was discharged.
Fig. 4. After the operation, bilirubin and LDH slightly increased but showed rapid improvement.
No progression of anemia requiring blood transfusion was observed