Peer Reviewed
Diffuse Large B-Cell Non-Hodgkin Lymphoma Presenting as Acute Pancreatitis
Discussion. Diffuse large B-cell lymphoma (DLBCL) is the most common lymphoid malignancy, comprising 31% of all NHLs in Western countries and accounting for 4% of all reported cancer cases in the United States.1-3 The lymphoma can present as enlarged lymph nodes at the primary site or as enlargement of extranodal tissues. At times, DLBCL can arise in extranodal sites such as the liver, large intestine, and small intestine, among others.1 While the lymphoma can arise in virtually any tissue in the body, the most common site of extranodal involvement is the stomach, comprising 68% to 75% of all cases, with the small bowel following at 9%, the ileocecal region at 7%, the rectum at 2%, and diffuse colonic involvement at 1%.4,5
DLBCL is very heterogeneous in gene expression, and so far more than 150 genetic driver genes of this disease have been identified, but the exact causes are still unknown.6,7 The clinical presentation of DLBCL ranges from generalized B symptoms to specific somatic complaints.8
In very rare cases, DLBCL can present with pancreatic involvement, accounting for 0.2% to 2% of all cases.1 To our knowledge, there has been only one other published case of secondary pancreatic involvement of a diffuse large B-cell NHL presenting as acute pancreatitis.9 Our patient presented with clinical signs of acute pancreatitis, which was further confirmed with diagnostic testing.
The most common causes of acute pancreatitis include gallstones, alcohol, and hypertriglyceridemia, among others. Our patient adamantly denied ingestion of alcohol and had well-controlled lipid levels. While biliary and liver abnormalities were identified on imaging and laboratory test results, the placement of a biliary stent through transparietal transhepatic cholecystostomy failed to improve the pancreatitis. After exhausting all diagnostic modalities only to have the patient’s status worsen, a last-ditch diagnostic effort was made by sending the ascitic fluid for pathological analysis, which finally shed light on the root problem, a diffuse large B-cell NHL.
This case highlights the importance of ruling out lymphoma in cases of gastrointestinal tract symptoms that fail to improve with conventional treatment modalities.
References:
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- Key statistics for non-Hodgkin lymphoma. American Cancer Society website. https://www.cancer.org/cancer/non-hodgkin-lymphoma/about/key-statistics.html. Revised January 8, 2019. Accessed July 31, 2019.
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- Koch P, del Valle F, Berdel WE, et al; German Multicenter Study Group. Primary gastrointestinal non-Hodgkin’s lymphoma: I. Anatomic and histologic distribution, clinical features, and survival data of 371 patients registered in the German multicenter study GIT NHL 01/92. J Clin Oncol. 2001;19(18):3861-3873.
- Papaxoinis G, Papageorgiou S, Rontogianni D, et al. Primary gastrointestinal non-Hodgkin’s lymphoma: a clinicopathologic study of 128 cases in Greece. A Hellenic Cooperative Oncology Group study (HeCOG). Leuk Lymphoma. 2006;47(10):2140-2146.
- Reddy A, Zhang J, Davis NS, et al. Genetic and functional drivers of diffuse large B cell lymphoma. Cell. 2017;171(2):481-494.e15.
- Zhang J, Grubor V, Love CL, et al. Genetic heterogeneity of diffuse large B-cell lymphoma. Proc Natl Acad Sci U S A. 2013;110(4):1398-1403.
- Le Gouill S, Talmant P, Touzeau C, et al. The clinical presentation and prognosis of diffuse large B-cell lymphoma with t(14;18) and 8q24/c-MYC rearrangement. Haematologica. 2007;92(10):1335-1342.
- Saif MW, Khubchandani S, Walczak M. Secondary pancreatic involvement by a diffuse large B-cell lymphoma presenting as acute pancreatitis. World J Gastroenterol. 2007;13(36):4909-4911.