Primary Hodgkin lymphoma of the ileum

Abstract

Primary classical Hodgkin lymphomas of the gastrointestinal tract are rare lymphoproliferative neoplasms as against other well established mucosa-associated lymphoid tissue (MALT) lymphomas. The primary nature of these cases is established based on chest radiograph, computed axial tomography, positron emission tomography, peripheral blood and bone marrow biopsy examination. Here, we report a 79-year-old male patient, who presented to the emergency department with complaints of abdominal discomfort on right side and low grade fever. An ultrasonogram was performed which suggested of an intestinal perforation. An emergency laporotomy was done with resection of the ileal segment. The histomorphology of the resected ileal segment revealed features of classical Hodgkin lymphoma which were confirmed by immunohistochemistry. Epstein Barr virus encoded RNA (EBER) via in-situ hybridization was positive and provided substantial evidence of EBV etiology.

Primary Intestinal Hodgkin’s Lymphoma: An Uncommon Presentation

“…[1] In view of its rare occurrence, diagnosis has to be made in accordance with histological as well as other clinical and radiological criteria. [6] These can be outlined as: (a) no superficial lymphadenopathy should be present at the time of diagnosis (b) no involvement of mediastinal lymph node on chest imaging (c) complete blood count and WBC differential should be within normal limits (d) predominance of GIT lesion with or without positive adjacent lymph node (e) liver and spleen should be disease free at the time of diagnosis.…”

“…It has been well-documented that ileum is rich in lymphoid follicles[4] hence, neoplastic transformation due to changes in immune response can occur. [1] Because it is rare for HL to localize to the small intestine, any diagnosis of the small bowel HL should prompt a thorough search for additional disease at the other site. [8]…”

“…[123] Diagnosis of the primary lesion requires the lack of peripheral or mediastinal lymphadenopathy, normal white blood cell (WBC) count and differential on peripheral blood smear. [4] The most common site of extranodal lymphoma is the GIT with the majority of cases in the ileum due to the abundant lymphoid follicles.…”

Primary Intestinal Hodgkin Lymphoma Mimicking Intraabdominal Abscess in a Renal Transplant Recipient: A Case Report

“…In addition, 11 case reports describing patients with small intestine HL have been published in the English-language journals since 1967 [13]. Bearing in mind that all these patients were diagnosed without using immunohistochemistry, the diagnoses have been called into question to the point that some authors doubt the existence of primary intestinal HL [12,14]. …”

“…Primary extranodal HL appears extremely rare in general, comprising 1-3% of HL patients, the most common being primary gastrointestinal involvement [11,12]. 7 patients with HL of the small intestine were identified in the literature review among 778 gastrointestinal lymphomas from 9 case series.…”