Post-Liver Transplant Lymphoproliferative Syndrome as a Cause of Intestinal Ischemia and Perforation: A Case Report
Moreira Ei1; Viana Si1; Amorín, Ei2; Rey Ri3, Rodrigo Vierai1, Jorge Curi1
¹General Surgeon, Emergency Department, Hospital Policial. Montevideo, Uruguay.
²Resident, Intensive Care Unit, Hospital Policial. Montevideo, Uruguay.
³Internist, Liver Transplant Program, Hospital Policial. Montevideo, Uruguay
*Corresponding author
Moreira E, General Surgeon, Emergency Department, Hospital Policial. Montevideo, Uruguay.
E-Mail: moreyyy@hotmail.com
DOI: 10.55920/JCRMHS.2025.12.001525
Figure 1 : Thoracic CT imaging and fluid (figure 1), pneumatosis in distal demonstrating persistent right lower ileum and cecum (without lobe consolidation with associated pneumoperitoneum), moderate free fluid pleural effusion (+) and pericardial predominantly in the lower and right effusion (*)
ECG demonstrated narrow-complex tachycardia up to 150 bpm. Laboratory tests showed progressive pancytopenia, liver failure, and worsening renal failure.
The patient was transferred to the Intensive Care Unit with presumed enteric septic shock. Management included packed red blood cell transfusion, fluid resuscitation, and broad-spectrum antibiotics (vancomycin, amikacin, metronidazole) with fluconazole.
Repeat abdominal CT showed new moderate bilateral pleural effusions, 9 mm pericardial effusion, persistent right lung consolidation abdomen, and abnormal mesenteric fat density (figure 2).
Figure 2 : Abdominal CT imaging demonstrating abdnormal mesenteric fat strading (*) free fluid predominantly in the right lower quadrant (+) and pneumatosis in the right colon (arrow)
Figure 3 : Abdominal CT imaging demonstrating abdnormal mesenteric fat strading (*) free fluid predominantly in the right lower quadrant (+) and pneumatosis in the right colon (arrow)
During exploratory laparotomy, turbid fluid and pseudomembranes were found. The distal ileum (last 20 cm) showed ischemic injury and aperistalsis, with associated ischemia of the right colon. An extended right colectomy was performed, resecting both segments with distal closure and proximal ileostomy. A fibrous band causing partial obstruction 100 cm from the ileocecal valve was lysed, and the underlying serosal injury was repaired. At 140 cm from previous injury site, a segment of mid-jejunum showed necrosis with perforation, requiring resection and hand-sewn anastomosis. The abdomen was temporarily closed as a laparostomy (figure 3).
Figure 3 : On the left side, at 140 cm of ileocecal valve a segment of mid-jejunum with necrotic perforation, simple suture was performed. On central image, ischemic changes in the right colon. On the right side ischemic changes on the las 20cm of the ileum, right colectomy with ileostomy was performed.
Postoperatively, the patient showed transient improvement with decreasing lactate and partial recovery of organ function. However, she developed anuria requiring hemodialysis, followed by clinical deterioration with increasing organ dysfunction and hypothermia. Antibiotic therapy was adjusted by the transplant infectious disease specialist.
Forty-eight hours past the first surgery, bilious drainage from the laparostomy system was noted and re-exploration was performed. It revealed bile-stained dressings with edematous but viable-appearing bowel. A new ischemic perforation was identified along the mesenteric border of the proximal jejunum (figure 4), which was managed with limited resection and primary anastomosis. Previous anastomotic sites appeared intact. The abdomen was again temporarily closed.
Figure 4 :Diffuse bile staining of peritoneal surfaces. A new ischemic perforation was identified along the mesenteric border of the proximal jejunum. Resection and primary anastomosis were performed
The patient progressed to refractory shock accompanied by escalating serum lactate levels, acidosis, and hemodynamic instability despite aggressive fluid resuscitation and high dose vasopressors. She died several hours later.
Pathological Findings: Histopathological examination of the specimens from both the first and second surgeries revealed necrotic
and ulcerated areas with diffuse atypical lymphoid proliferation in the submucosa, composed of medium-sized cells exhibiting irregular nuclei, prominent nucleoli, and scant cytoplasm. A transmural mixed lymphomononuclear and polymorphonuclear inflammatory infiltrate was present, along with vascular thrombosis.
Immunohistochemical studies performed using available antibodies demonstrated that the atypical lymphoid cells were strongly and diffusely positive for CD45 (leukocyte common antigen), BCL2, and CD20, while negative for CD3, CD5, cytokeratin (CK), and CD10. The proliferation index, as assessed by Ki67 staining, was markedly elevated at 90%. Based on the morphological features and immunophenotypic profile including B-cell lineage differentiation, diffuse growth pattern, and high proliferative activity. The findings are consistent with a large B-cell lymphoproliferative disorder4 (figure 5).
Figure 5: On the left side HE 40x showing intestinal sections with extensive replacement by an atypical lymphoid cell proliferation showing a diffuse pattern. On the right side HE 400x showing atypical cellularity with lobulated nuclei, prominent nucleoli, and diffuse pattern.
