Case presentation
An 84-year-old woman was admitted to the general ward of our hospital
with the chief complaints of stomachache and diarrhea. She was a known
case of myasthenia gravis; she had undergone thymoma extraction 6 years
ago, after which she had started taking prednisolone 6mg/day and
tacrolimus 3mg/day. On admission, computed tomography (CT) showed
ascending colon cancer. Three days later, she underwent colon stenting
with an endoscope. She complained of feverishness and a severe
stomachache that night. The CT scan was re-examined and free air was
found in the abdominal cavity. She was immediately taken in for
emergency surgery. She underwent a right hemicolectomy and ileostomy
through open surgery.
After surgery, she was admitted to the intensive care unit (ICU), where
she was orally intubated and ventilated. Extracellular fluid was
administered at 200 mL/h and noradrenaline was administered at 0.3 γ.
Her vital signs were: body temperature 35.8° C, blood pressure 108/65
mmHg, pulse 122/min, respiratory rate 15/min under sedation. Meropenem
3g/day and daptomycin 350mg/day were used as antibiotics. Table 1 shows
the blood test parameters on admission to the ICU. The blood pressure
started dropping 5 hours after the operation; the fluid volume was
increased to 500 mL/h, but the blood pressure did not rise. Therefore, 7
hours after the operation, continuous administration of hydrocortisone
(200 mg/day) and continuous hemodiafiltration (CHDF) were introduced. We
were using the EV1000🄬 (Edwards Life Science, Ltd) for hemodynamic
monitoring. Based on the data provided by the EV1000🄬, especially the
global end-diastolic volume index (GEDI), up to 17 hours after the
operation, we gradually reduced the infusion volume. The day after the
operation, the infusion was 200 mL/h. By the 2nd postoperative day, the
infusion could be reduced to 100 mL/h; however, after a while, the blood
pressure began to decrease. The infusion volume was therefore again
increased to 500 mL/h. On the 3rd postoperative day, it was not possible
to reduce the amount of the fluid, and we had to increase the infusion
to 1000 mL/h. Although her condition was initially attributed to
uncontrolled sepsis, her C-reactive protein level, white blood cell
count, and procalcitonin level had improved on the third postoperative
day; moreover, no bacteria were detected in the postoperative culture
specimens. In addition, the serum albumin level was extremely low (0.7
mg/dL), due to which we suspected SCLS for the first time. We conducted
blood tests, which revealed increased level of kappa light chain M
protein (9.6 mg/dL). Urinary Bence Jones protein was negative and C-1
esterase inhibitor level was low (Table 1). Based on these results, we
diagnosed SCLS. At the time of diagnosis, her condition was quite
critical, with the blood pressure dropping to 48/30 mmHg despite an
infusion speed of 1000 mL/h. Therefore, PDF was introduced in
preparation for administration of a high dose of IVIG (0.4 g/kg/day).
After PDF was started, her blood pressure gradually increased. About 2
hours later, her blood pressure was 132/70 mmHg. Soon after that, IVIG
was started. The infusion speed was gradually decreased, and 9 hours
after PDF started, the infusion speed was 60 mL/h. The PDF was continued
for 39 hours and we continued CHDF. The patient’s condition was stable
for approximately 37 hours after PDF was stopped. Subsequently, her
blood pressure gradually decreased. We reintroduced PDF as soon as we
expected the SCLS to relapse. In addition, steroid pulse therapy
(methylprednisolone 1 g/day) was also initiated, as PDF alone raised
concerns about the patient relapsing. Her condition began to improve and
stabilized again.
However, after the PDF was stopped, the patient reverted to her initial
state in approximately 2 days. We therefore used PDF for the third time.
PDF was effective, but the patient relapsed a few days later. PDF was
effective, but the patient relapsed a few days later and died. The ICU
course is shown in Fig. 1.