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Double-hit primary high-grade gastric B-cell lymphoma presenting with pancytopaenia and atraumatic back pain: A case
Michele Yi Fu1, Kevin Jia1, Christina Lee1
1Department of Gastroenterology and Hepatology, Campbelltown Hospital, Campbelltown 2560, New South Wales, Australia.
Background:
Primary gastric lymphoma is relatively rare, accounting for 5% of primary gastric neoplasms, with the two most common lymphoma subtypes being diffuse large B-cell lymphoma and mucosa-associated lymphoid tissue lymphoma. Many patients with gastrointestinal lymphoma have a delayed presentation involving vague symptomatology. On endoscopy, there are three main injury patterns of gastric lymphoma: Ulceration, diffuse infiltration with enlarged mucosal folds, and the development of a polypoid mass, usually affecting the fundus or antrum. "Double-" and "triple-hit" high-grade B-cell lymphoma are an emerging subtype of B-cell lymphoma characterised by cellular melocytomatosis oncogene and B-cell lymphoma (BCL) 2 and/or BCL6 rearrangement identified by cytogenetic testing or fluorescence in situ hybridisation testing. This is an uncommon variant of B-cell lymphoma, which is associated with an adverse prognosis compared to its standard diffuse large B-cell lymphoma counterparts; however, there is limited literature pertaining to its clinical significance in the context of primary gastric high-grade lymphoma.
Case Summary:
We present a case of a 71-year-old male with disseminated primary gastric high-grade B-cell lymphoma with bony metastases. This was identified after the patient presented to the emergency department on three separate occasions with vague symptomatology, including chest, abdominal and back pain, and loose stools. Chest X-ray identified an atraumatic left rib fracture, and this was further explored with an outpatient computed tomography chest, which showed left fifth, seventh and eighth rib fractures, with ill-defined lucencies suspicious for underlying lesions. Computed tomography lumbar spine showed possible metastatic disease in the lumbar vertebrae. Positron emission tomography scan revealed non-specific mild to moderate heterogeneous uptake in the stomach, with disseminated, mixed, predominantly lytic bone metastases throughout the axial and appendicular skeletons. Gastroscopy subsequently identified multiple 10 mm to 20 mm semi-sessile polyps in the stomach, and biopsy confirmed high-grade "double-hit" B-cell lymphoma with melocytomatosis oncogene and BCL6 rearrangements. The patient was treated with rituximab, etoposide, prednisolone, vincristine, cyclophosphamide, doxorubicin and intrathecal methotrexate.
Conclusion:
This case report highlights an uncommon presentation of high-grade primary gastric lymphoma and re-emphasises the aggressive nature of the disease.
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