Laboratory blood test results were notable for anemia (hemoglobin 10.4 g/dL), thrombocytosis (566 K/mm3), and a normal white blood cell level (10.7 K/mm3). Further laboratory testing revealed normal levels of carcinoembryonic antigen, cancer antigen, alpha fetoprotein, and Beta 2 Microglobulin. HIV, hepatitis B, and hepatitis C were all negative.
A large mass measuring 13×10×12 cm in the right upper quadrant with resultant mass effect in the area, multiple mesenteric lesions, and a 1.5-cm left ureteral nephrolithiasis were seen on abdominal CT scan.
Based on the results of colonoscopy, biopsy, and histology, mantle cell lymphoma was diagnosed. In addition, an 11×43-mm thrombi was seen in the ascending thoracic aorta on CT scan of the chest with IV contrast, for which enoxaparin 1 mg/kg twice daily was started. Eastern Cooperative Oncology Group score quickly increased from 1 (limited ability to perform activities of daily living) to 3 (bedridden more than 50% of the day). The patient was advised of the risks of chemotherapy; he agreed to proceed with R-CHOP; and treatment with rituximab at 50% dose reduction, CHOP at 25% dose reduction, and prophylactic allopurinol was initiated. Filgrastim was used to mitigate risks of neutropenia.
After 9 days of treatment, the patient developed feculent emesis secondary to symptomatic bowel obstruction. CT imaging revealed free intraperitoneal air consistent with intestinal perforation. Enoxaparin was discontinued, and piperacillin-tazobactam was started for presumed intra-abdominal bacterial seeding. He tested positive for multiple blood cultures of Escherichia coli without antibiotic resistance. The patient was placed on total bowel rest with IV nutrition.
Free abdominal air resolved within days; however, a loculated fluid collection developed in the right hemipelvis requiring placement of a percutaneous drain. The patient did well with continued medical management, with clinical improvement 4 months after the abdominal perforation. His weight increased from 50 kg on admission to 71.2 kg. PET scan after the first R-CHOP cycle demonstrated a hypermetabolic solid intraluminal 9.2×8.4×10.8-cm mass, along with multiple metabolically active retroperitoneal and mesenteric nodes (all less than 1 cm).
Patients with MCL typically present with late-stage disease, extensive lymphadenopathy, bone marrow involvement, splenomegaly (80% of cases), and involvement of extranodal sites such as the GI tract, breast, pleura, or orbits (10% to 20% of patients). Tissue pathology is the standard diagnostic, with histopathology critical to diagnosis. Presence of night sweats, chills, and weight loss prior to diagnosis; high-risk MIPI [mantle cell international prognostic index] scoring; elevated LDH; Ki67 greater than 30%; and histology notable for complex karyotype, blastoid variance, and 17p/TP53 or SOX 11 mutations are predictive of poor outcomes.
Mantle cell lymphoma is typically treated with intensive chemotherapeutic regimens, but treatment often depends on patient tolerance. Side effects and rate of secondary malignancies are often increased. Given the median age at diagnosis, harm vs benefit ratio is strongly considered when determining treatment course.
This patient experienced feculent emesis and symptoms of bowel obstruction concurrently with the GI perforation. CT scan confirmed perforation and intussusception, a rare occurrence in patients with MCL (1%).
The MIPI index may be used to indicate time to treatment failure and OS, but an association between MIPI score and complications such as GI perforation have not been established. Determining patient prognosis and potential for treatment complications remains a challenge. In addition, mortality rates for GI perforation can be high. Therefore, the authors of this case report recommend maintaining a high suspicion for GI perforation in patients with a history of gastrointestinal lymphoma who demonstrate abdominal rebound tenderness with or without feculent emesis, and to obtain CT imaging of the abdomen to evaluate for viscus perforation.
Adashek M, Chan A, Medina A. Gastrointestinal perforation after rituximab therapy in mantle cell lymphoma: a case report. Case Rep Oncol. 2018;11(3):784-790.