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Year : 2019  |  Volume : 62  |  Issue : 3  |  Page : 488-490
Composite lymphoma comprising mantle cell lymphoma and Epstein-Barr virus positive classic Hodgkin lymphoma: A rare case


1 Department of Histopathology, SRL Diagnostics, Clinical Reference Laboratory, Gurgaon, Haryana, India
2 Department of ENT, Shri Lal Bahadur Shastri Government Medical College, Mandi, Himachal Pradesh, India

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Date of Web Publication26-Jul-2019
 

How to cite this article:
Sharma S, Singh V, Bisaria D, Tangri R. Composite lymphoma comprising mantle cell lymphoma and Epstein-Barr virus positive classic Hodgkin lymphoma: A rare case. Indian J Pathol Microbiol 2019;62:488-90

How to cite this URL:
Sharma S, Singh V, Bisaria D, Tangri R. Composite lymphoma comprising mantle cell lymphoma and Epstein-Barr virus positive classic Hodgkin lymphoma: A rare case. Indian J Pathol Microbiol [serial online] 2019 [cited 2019 Aug 23];62:488-90. Available from: http://www.ijpmonline.org/text.asp?2019/62/3/488/263478




Composite lymphoma is a rare lymphoid neoplasm defined as the presence of two or more morphologically and immunophenotypically distinct types of lymphoma involving the same anatomic site or tissue that can occur concurrently or sequentially. Composite lymphoma can be a combination of two non-Hodgkin lymphoma or combination of a non-Hodgkin and a Hodgkin lymphoma.[1]

We wish to report a case of composite mantle cell lymphoma and Epstein-Barr virus (EBV) positive classic Hodgkin lymphoma involving tonsil as the primary site. The diagnosis was based on histomorphological examination and immunohistochemistry (IHC) results.

A 62-year-old male presented to the outpatient department with chief complaints of difficulty in swallowing and weight loss since 1 month. On examination, he was found to have enlarged right tonsil and single palpable right cervical lymph node. Computerized tomography scan revealed the size of enlarged tonsil as 6.4 × 5.2 × 4.3 cm, while lymph node measured 1.5 × 1.3 × 1.2 cm. There was no evidence of hepatosplenomegaly or other significant lymphadenopathy. His complete blood counts and routine biochemical tests were within normal limits. Patient was seronegative for HIV, HbsAg, and hepatitis C virus. Right-side tonsillectomy was performed. Histopathological examination of the tonsil revealed stratified squamous epithelium-lined lymphoid tissue with effaced architecture by vague nodules of small- to medium-sized lymphoid cells [Figure 1]a. The lymphoid cells showed small round nuclei with slightly irregular contour, inconspicuous nucleoli, and scant cytoplasm. The internodular areas showed few scattered large mononuclear and binucleate Reed–Sternberg-like cells [Figure 1]b in a background composed of small lymphocytes, plasma cells, and prominent eosinophils [Figure 1]c. These large cells had moderate to abundant cytoplasm with enlarged nuclei and prominent eosinophilic nucleoli. Immunohistochemical study showed that the nodules were positive for leukocyte common antigen (LCA), CD20 [Figure 2]a, CD5 [Figure 2]b, B-cell lymphoma 2 (BCL2) [Figure 3]a, and cyclin D1 [Figure 1]d, whereas negative for CD23, CD10 [Figure 2]c, and BCL6. The Ki67 [Figure 3]b proliferation index was 8–10% in the nodules. The internodular areas chiefly comprised CD3 positive T-lymphocytes and scattered large cells that showed membranous positivity for CD30 [Figure 1]e and [Figure 2]d and CD15 [Figure 1]f, whereas dim nuclear positivity for PAX-5 [Figure 2]e. These large cells showed cytoplasmic positivity for EBV-latent membrane protein 1 (LMP1) [Figure 2]f and were negative for LCA, CD20, and BCL6. Based on histomorphology and IHC findings, diagnosis of composite lymphoma is rendered, which comprised mantle cell lymphoma and EBV positive classic Hodgkin lymphoma. The patient was then referred to a tertiary care center for treatment and further management.
Figure 1: (a) Vague nodules (H and E, ×50). (b) Large cells in internodular area (H and E, ×100). (c) Prominent eosinophils in internodular area (H and E, ×100). (d) Cyclin D1 (IHC, ×50). (e) CD30 (IHC, ×50). (f) CD15 (IHC, ×450)

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Figure 2: (a) CD20 (IHC, ×50). (b) CD5 (IHC, ×200). (c) CD10 (IHC, ×100). (d) CD30 (IHC, ×450). (e) PAX-5 (IHC, ×450). (f) EBV-LMP1 (IHC, ×450)

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Figure 3: (a) BCL2 (IHC, ×50). (b) Ki67 (IHC, ×100)

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The term composite lymphoma was given by Custer in 1954 and was later modified by Kim et al. to the current accepted definition. The etiology and pathogenesis of composite lymphoma are complex and variable. With the help of molecular pathology, it was observed that in some cases of composite lymphoma, the components are clonally related, whereas in others they are clonally unrelated. Composite B-cell non-Hodgkin lymphoma are often clonally unrelated. However, in many composite Hodgkin and non-Hodgkin lymphoma, the components are clonally related. In most of such cases, the malignant clones developed separately from a common precursor, usually a germinal center B-cell.

Further studies and investigations need to be carried out to understand the complex etiopathogenesis of composite lymphoma, a rare lymphoid neoplasm with no agreed standards of treatment at present.

The association of classic Hodgkin lymphoma and mantle cell lymphoma is very rare with only few cases described in the literature. Caleo et al.[2] and Tinguely et al.[3] were the first to describe composite Hodgkin lymphoma and mantle cell lymphoma. Caleo et al. described one case affecting the spleen and the eyelid and cervical lymph nodes in another case. Tinguely et al. described an additional case involving a lymph node. Hayes et al.[4] reported a case of composite mantle cell lymphoma and EBV negative classic Hodgkin lymphoma involving cervical and axillary lymph nodes.

To the best of author's knowledge, this is the first reported case of composite mantle cell lymphoma and EBV positive classic Hodgkin lymphoma involving tonsil as the primary site.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Kuppers R, Duhrsen U, Hansmann ML. Pathogenesis, diagnosis and treatment of composite lymphomas. Lancet Oncol 2014;15:e435-46.  Back to cited text no. 1
    
2.
Caleo A, Sánchez-Aguilera A, Rodríguez S, Dotor AM, Beltrán L, de Larrinoa AF, et al. Composite Hodgkin lymphoma and mantle cell lymphoma: Two clonally unrelated tumors. Am J Surg Pathol 2003;27:1577-80.  Back to cited text no. 2
    
3.
Tinguely M, Rosenquist R, Sundström C, Amini RM, Küppers R, Hansmann ML, et al. Analysis of a clonally related mantle cell and Hodgkin lymphoma indicates Epstein-Barr virus infection of a Hodgkin/Reed-Sternberg cell precursor in a germinal center. Am J Surg Pathol 2003;27:1483-8.  Back to cited text no. 3
    
4.
Hayes SJ, Banerjee SS, Cook Y, Houghton JB, Menasce LP. Composite mantle-cell lymphoma and classical Hodgkin lymphoma. Histopathology 2006;48:621-3.  Back to cited text no. 4
    

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Correspondence Address:
Shobhna Sharma
Department of Histopathology, SRL Diagnostics, Clinical Reference Laboratory, Gurgaon, Haryana
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/IJPM.IJPM_34_18

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