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CASE REPORT
Year : 2010  |  Volume : 6  |  Issue : 3  |  Page : 342-343

Primary Hodgkin lymphoma of the ileum


Department of Histopathology, Rajiv Gandhi Cancer Institute & Research Centre, Rohini, Delhi- 110085, India

Date of Web Publication29-Nov-2010

Correspondence Address:
Jatin S Gandhi
C-2-C/12/58, Janak Puri, New Delhi - 110058
India
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DOI: 10.4103/0973-1482.73354

PMID: 21119271

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 > 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.

Keywords: EBER-ISH, hodgkin lymphoma, ileum, reed sternberg cells


How to cite this article:
Gandhi JS, Mehta A, Sharma A, Kamboj M. Primary Hodgkin lymphoma of the ileum. J Can Res Ther 2010;6:342-3

How to cite this URL:
Gandhi JS, Mehta A, Sharma A, Kamboj M. Primary Hodgkin lymphoma of the ileum. J Can Res Ther [serial online] 2010 [cited 2014 Aug 23];6:342-3. Available from: http://www.cancerjournal.net/text.asp?2010/6/3/342/73354


 > Introduction Top


Primary classical Hodgkin lymphomas are well established lymphoproliferative neoplasms of the lymph node comprising mononuclear Hodgkin cells and multinucleated Reed Sternberg cells in variable amount of non neoplastic lymphocytes, eosinophils, neutrophils, histiocytes, plasma cells, fibroblasts and collagen fibers. Hodgkin lymphoma most commonly occurs in the cervical lymph nodes with bimodal age curve. The extranodal forms are rare and accounts for less than 1%. The most common site of involvement is the gastrointestinal tract, followed by (in order of descending frequency) the pulmonary system, thyroid, skin, genitourinary system, and central nervous system. The clinical manifestations are limited to small intestine in the primary Hodgkin lymphomas of the ileum. Ileum is the most common site in small intestine due to the presence of abundant lymphoid follicles. [1] Patients with inflammatory bowel disease are at increased risk for gastrointestinal malignancies, such as adenocarcinoma and non Hodgkin lymphoma and possibly Hodgkin lymphoma. [1] However, primary gastrointestinal Hodgkin lymphoma without any lymph node association is controversial, because only a few such cases with limited immunohistochemical and/or molecular confirmation have been reported in the literature. Here we describe an additional case of primary extranodal classical Hodgkin lymphoma arising in the terminal ileum in a 79-year-old man.


 > Case Report Top


A 79-year-old gentleman presented to the medical emergency department with complaints of sudden right sided abdominal discomfort, generalized weakness and constipation since two days and low grade fever associated with chills since one month. A differential diagnosis of intestinal tuberculosis with perforation and superior mesenteric artery occlusion was suspected. The chest radiograph was within normal limits. An ultrasonogram and radiography of the abdomen was done which revealed air fluid levels in the peritoneum. The ultrasonogram also revealed thickened bowel loops in the right pelvic region. An emergency exploratory laporotomy with ileostomy and resection was done under general anesthesia. Patient was kept in intensive care unit under observation.

Pathologic findings

The resected segment of the ileum measuring 9.5 cm in length and 3 cm in diameter was sent to the histopathology department. On gross, external aspect was covered with exudates and showed an area of perforation 5 cm from the proximal cut end. An indurated lesion was seen adjacent to the site of perforation which was located 2 cm from the proximal cut end of the ileum. On opening the bowel, a greyish-white tumor with nodular appearance measuring 3 × 1.5 cm was noted. On serial slicing, the tumor thickness was 0.8 cm involving the mucosa and submucosa. The adjacent mucosa was pale and edematous in appearance. Extensive sampling of the regional lymph nodes was done to rule out any primary in the lymph nodes. Multiple sections were studied from the small intestine, which on microscopy showed intestinal mucosa with altered glandular architecture displaying regenerative changes. The lamina propria and submucosa had abundant lymphoid tissue both in diffuse and follicular pattern. The lymphocytic infiltrate was seen in conjunction with plasma cells, histiocytes and eosinophils and was seen extending into the muscularis propria and serosa. Interspersed in between these mixed population of cells, were numerous mononuclear Hodgkin cells, multinucleated and multilobated Reed Sternberg cells having vesicular chromatin and prominent eosinophillic nucleoli [Figure 1]a. Mummified cells were also seen in the background.
Figure 1: (a) Ileum showing binucleate Reed Sternberg cell in a polymorphic background (arrow) (H and E, ×200). (b) Reed Sternberg cells showing negativity for leukocyte common antigen (arrow) (DAB, ×400). (c) Reed Sternberg cells showing positivity for CD 30 (DAB, 100); higher power view in inset (DAB, 400). (d) Reed Sternberg cells showing positivity for CD 15 (arrow) (DAB, 200). (e) and (f) showing
positivity for Epstein Barr Virus (EBER-ISH, ×400)


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An immunohistochemical (IHC) examination revealed the Reed Sternberg cells to be immunonegative for leukocyte common antigen (Dako) [Figure 1]b and immunopositive for cluster of differentiation (CD), CD30 (Dako) [Figure 1]c and CD15 (Dako) [Figure 1]d. They were also immunonegative for CD3 (Dako), CD20 (Dako) and ALK1 protein (Novocastro). Epstein Barr virus encoded RNA (EBER) was done via in situ hybridization according to the kit guidelines and showed positive staining for Epstein Barr virus (EBV) infected Hodgkin cells [[Figure 1]e and f]. The background population was rich in reactive B and T lymphocytes. The IHC kits were standardized according to the kit guidelines and then studied along with the prescribed controls.


 > Discussion Top


Primary extranodal Hodgkin lymphomas of the ileum are very rare lympho-proliferative neoplasms consisting of 1% of the cases. Usually, these have bimodal age peaks. A review of the literature revealed nine case series, in which a total of 778 gastrointestinal lymphomas were evaluated, and only seven patients with Hodgkin lymphoma of the small intestine were identified. Eleven individual patients with small bowel Hodgkin lymphoma have also been reported in English-language journals since 1967. [2] All the diagnosis were made in the absence of immunohistochemistry, hence there diagnosis has been questioned to the point of their existence by some authors. [3] Because cases of primary gastrointestinal Hodgkin lymphoma are so rare, the diagnosis should only be made following strict histologic and other criteria proposed by Dawson et al, [4] that is (a) no superficial lymphadenopathy should be present at the time of diagnosis; (b) chest radiological studies should indicate no involvement of mediastinal lymph nodes; (c) the complete blood count and white cell differential should be within normal limits; (d) gastrointestinal lesion should predominate with or without positive adjacent lymph nodes; and (e) liver and spleen should be free of disease at the time of diagnosis.

Our case fulfils the criteria laid down by Dawson et al, and confirmation for the same was done by immunohistochemistry. The Reed-Sternberg cells and their variants demonstrated a typical immunohistochemical profile (membrane and cytoplasmic staining with dot-like Golgi enhancement of CD30, moderate cytoplasmic staining of CD15 in the Golgi area, and CD45 and epithelial membrane antigen negativity. Our patient had undergone a whole body positron emission tomography, chest radiography and bone marrow examination to look for any disease activity anywhere else in the body. These investigations were all within normal limits, thus proving the case to be of primary ileal origin.

The association of Epstein Barr Virus with Hodgkin lymphoma is well known. [1] We demonstrated Epstein Barr Virus infectivity via EBER-ISH in the Reed Sternberg cells which generally occurs after immunosuppression, as in our case who was 79-years-old and had developed senescence- related immunosuppression.

In conclusion, this case is presented for its rarity for site. It demonstrates that ileum being rich in lymphoid follicles (peyers patches) can undergo neoplastic transformation due to senescence-related changes in the immune response.

 
 > References Top

1.Shiyong Li, Borowitz MJ. Primary Epstein-Barr Virus-Associated Hodgkin Disease of the Ileum Complicating Crohn Disease. Arch Pathol Lab Med 2001;125:424-7.  Back to cited text no. 1
    
2.Morgan PB, Kessel IL, Xiao SY, Colman M. Uncommon presentations of hodgkins disease. J Clin Oncol 2004;24:193-8.  Back to cited text no. 2
    
3.Pai SA, Naresh KN, Borges AM. Primary Hodgkin's disease of the small intestine: Does it really exist? Indian J Gastroenterol 1994;13:150.  Back to cited text no. 3
[PUBMED]    
4.Dawson IM, Cornes JS, Morson BC. Primary malignant lymphoid tumors of the intestinal tract: Report of 37 cases with a study of factors influencing prognosis. Br J Surg 1961;48:80-94.  Back to cited text no. 4
    


    Figures

  [Figure 1]


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