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Case Report HIV-related Burkitt lymphoma with florid granulomatous reaction: an unusual case with good outcome

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Int J Clin Exp Pathol 2014;7(10):7049-7053 www.ijcep.com /ISSN:1936-2625/IJCEP0001641

Introduction

Burkitt lymphoma (BL) is a highly aggressive subtype of non-Hodgkin lymphomas (NHL). The World Health Organization (WHO) classification recognizes three clinical variants of BL: sporad-ic, endemsporad-ic, and immunodeficiency related [1]. The immunodeficiency related BL is more com -mon in patient with human immunodeficiency virus (HIV), in whom the lifetime incidence of BL has been estimated at 10-20% [2]. Granu-lomatous reaction usually indicates a chronic inflammation caused by microorganisms and inert foreign bodies. Lymphoma related granu-lomatous reaction rarely occurs in sporadic BL, suggesting limited stage disease and good prognosis [3]. From our review of relevant litera -ture, a total of 15 cases of sporadic BL with granulomatous reaction have been reported in English literature [3-7]. Herein, we report the first case of HIV related Burkitt lymphoma with florid granulomatous reaction.

Case report

A 41-year-old Chinese male presented lymph-adenopathy in the right cervical lymph node for

3 months. Initial clinical examination revealed the enlargement of right side cervical lymph node 1.5 cm in diameter, hard and painful. He underwent a complete excision of the lymph node and was diagnosed as tuberculous lymph-adenitis in other hospital. Subsequently, the case was sent to us for consultation. Blood tests disclosed the following values: haemoglo-bin 114 g/L; leucocytes 4.11 × 109/L with 27.0% lymphocytes and 68.0% neutrophils; platelets 75 × 109/L. Standard ELISA serology test and quantitative PCR (HIV viral load 31600 copies/ml) confirmed the diagnosis of HIV infec -tion. The CD4 cell count was 12.00% (normal reference ranging 33.19% from 47.85%). Blood tests for Hepatitis B virus, Hepatitis C virus and Treponema pallidum antibody were negative. Morphological, immunohistochemical and special staining findings

Microscopically, the normal architecture of lymph node was replaced by prominent granu-lomas of varying size with Langhans giant cells (Figure 1A, 1B). In addition, monotonous infil -trate of medium-sized atypical lymphocytes around the granulomas presented “starry sky”

Case Report

HIV-related Burkitt lymphoma with florid granulomatous

reaction: an unusual case with good outcome

Jin-Nan Li, Li-Min Gao, Wei-Ya Wang, Min Chen, Gan-Di Li, Wei-Ping Liu, Wen-Yan Zhang

Department of Pathology, West China Hospital, Sichuan University, Chengdu 610041, China

Received July 31, 2014; Accepted August 23, 2014; Epub September 15, 2014; Published October 1, 2014

Abstract: Burkitt lymphoma (BL) is a highly aggressive subtype of non-Hodgkin lymphomas (NHL). Lymphoma

re-lated granulomatous reaction rarely occurs in sporadic BL. Herein, we describe the first case of HIV rere-lated Burkitt lymphoma with florid granulomatous reaction. A 41-year-old HIV-positive Chinese male presented lymphadenopathy

in the right cervical region for 3 months. The enlarged lymph node biopsies revealed the presence of prominent granulomas of varying size with Langhans giant cells, leading to the misdiagnosis of tuberculous lymphadenitis in other hospital. Subsequently, the case was sent to us for consultation. The morphology, immunophenotype, special

staining, interphase FISH analysis and blood tests confirmed a diagnosis of HIV related Burkitt lymphoma with

granulomatous reaction. Without radiotherapy and chemotherapy, the patient was alive and well with no evidence

of lymphoma during the observation period of 24 months. The case suggested that lymphoma with florid granulo -matous reaction can easily be misdiagnosed as benign lesions since the large number of epithelioid granulomas could obscure the primary lesion. Moreover, the granulomatous reaction may be an indicator for favorable prognosis in HIV related Burkitt lymphoma.

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growth pattern. The atypical lymphocytes showed round or oval nuclei with finely clumped and dispersed chromatin and 2 ~ 4 basophilic medium sized, centrally situated nucleoli. The cytoplasm was scarce to moderate and baso-philic (Figure 1C, 1D).

By immunohistochemical staining, the atypical lymphocytes were positive for CD20, CD10 and BCL6 and negative for CD3έ, BCL2 and MUM1. Nearly 100% of atypical lymphocytes expressed Ki-67 (Figure 2A-C). In addition, Ziehl-Neelsen, periodic acid-Schiff, and Grocott stainings did not found the evidence of microorganism infection.

In situ hybridization for EBV

In situ hybridization indicated positivity for EBV presence in the nuclei of atypical lymphocytes by using the in situ hybridization detection kit (A300K.9901; Panpath, Holland) (Figure 2D).

However, histiocytes around the atypical lym-phocytes were negative.

Interphase fluorescence in situ hybridization (FISH) analysis

FISH analysis was performed on 4 μm thick paraffin-embedded tissue sections. All of the FISH probes were obtained from Vysis/Abbott, Downers Grove USA. FISH using LSI MYC dual-color break-apart rearrangement probe showed 1 orange, 1 green and 1 orange/green fusion (1O1G1F) signal pattern (Figure 3A) indicating the presence of MYC translocation. FISH using IGH/MYC dual color, dual-fusion translocation probe revealed 1 orange, 1 green and 2 orange/ green fusions (1O1G2F) signal pattern (Figure 3B) indicating translocation between MYC and IGH. FISH using BCL2 dual-color break-apart probe and BCL6 dual-color break-apart probe presented 2 two orange/green fusion signals pattern (Figure 3C, 3D) indicating atypical lym-Figure 1. Prominent granulomas of different size are identified (H&E × 100) (A). Langhans giant cells are fairly easy to be observed in granulomas (H&E × 400) (B). “Starry sky” pattern is obviously seen (H&E × 200) (C). Medium sized cells present round or oval nuclei with finely clumped and dispersed chromatins and 2~4 basophilic (H&E ×

[image:2.612.91.525.71.394.2]
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[image:3.612.91.525.70.393.2]

HIV-related Burkitt lymphoma with florid granulomatous reaction

Figure 2. Immunohistochemistry (A-C). Atypical lymphocytes are positive for CD20 (A), CD10 (B), and nearly 100% of atypical lymphocytes are positive for Ki-67 (C) (× 200). EBER-ISH shows positive signal in atypical lymphocytes, while histiocytes are negative (D) (× 200).

phocytes absence of BCL2 and BCL6 translo-cation respectively.

Clinical follow-up

Without radiotherapy and chemotherapy, the patient was alive and well with no evidence of lymphoma during the observation period of 24 months. Follow-up including blood tests, imag -ing examinations and bone marrow biopsy were performed every 6 months.

Discussion

Lymphoma related epithelioid cell granuloma occurs in T-cell-derived NHL, Hodgkin lympho-ma (HL) and some B-cell-derived NHL [8, 9]. Granulomatous reaction indicates different clinicopathological significances in different lymphomas. Granulomatous reaction may reflect the disease progression of follicular lym -phoma (FL) in large B-cell lym-phoma [10]. Conversely, granulomatous reaction suggests

limited stage disease and good prognosis in cases of sporadic BL and HL [3, 9]. Unfortuna-tely, lymphomas with accompanying florid gran -ulomatous reaction are easilymisdiagnosed as benign lesions due to the fact that the primary pathology is obscured by a large number of granulomas. To make the correct diagnosis may be a challenge on fine needle aspiration biopsy. Immunohistochemical staining, special staining and molecular tests are possessed of very important in accurate diagnosis.

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the lack of LMP1 expression in tumor cells [3]. However, a recent study reported a case of flor -id granulomatous reaction in primary gastric BL without EBV infection [6]. Furthermore, Hossain et al. showed that BL was deficient in HLA class II-mediated Ag presentation [11]. The exact causes of granulomas in BL became vaguer. The case under discussion exhibited three par-ticular features which are rare in this entity. First, the patient infected with HIV had subnor -mal CD4 cell count. Owing to EBER positivity was detected only in tumor cells and the CD4 cell count was subnormal, we expect that CD4+helper T cells don’t play the key role for formation of the florid granulomatous reaction. BL related epithelioid cell granuloma is more likely to result from cytokine production by tumor cells. Second, without radiotherapy and chemotherapy, the patient revealed no eviden-ceof BL involvement during the next 24-month. It seems that there is an association between

the granulomatous reaction and the favorable outcome in case of HIV related BL. Third, the granulomas occupied a significant area in the lymph node, which created troubles to make the accurate diagnosis. The presence of atypi-cal lymphocytes in the context of a granuloma-tous reaction aroused our suspicion, and immu-nohistochemical staining, special staining and FISH analysis are useful to establish an accu -rate diagnosis.

In summary, we describe the first case of HIV related BL with accompanying florid granuloma -tous reaction which almost obscured the pri-mary pathology. Pathologists need to be aware of the presence of large number of prominent granulomatous reaction. The first temptation is to think about an infectious disease. Special stains are required to rule out the possibility of infection. Any presence of atypical lymphocytes in the context of a granulomatous reaction should be considered suspicious. The patient Figure 3. Representative interphase FISH results. FISH study using MYC dual-color break-apart rearrangement probe detects a dissociation of the red and green signals (A). IGH/MYC dual color, dual-fusion translocation probe shows two fusion signals (B). FISH study using BCL2 dual-color break-apart probe and BCL6 dual-color break-apart

[image:4.612.90.524.70.394.2]
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HIV-related Burkitt lymphoma with florid granulomatous reaction

had a good prognosis suggesting an associa-tion between the granulomatous reacassocia-tion and the favorable outcome in case of HIV related BL. Many possible mechanisms of genesis of granulomas in BL have been postulated. However, more cases and further research are needed to elucidate the exact causes of granu-lomas in cases of BL which may provide some new insights into the treatment of BL.

Acknowledgements

This work was supported by the National Natural Science Foundation of China (grant number 81272626).

Disclosure of conflict of interest

None.

Address correspondence to: Dr. Wen-Yan Zhang,

Department of Pathology, West China Hospital,

Sichuan University, Chengdu 610041, China. Tel: +86-28-85422699; Fax: +86-28-85422699; E-mail:

zhangwyliu@ hotmail.com

References

[1] Leoncini L, Raphael M, Stein H, Harris NL, Jaffe ES, Kluin PM. Burkitt lymphoma. In: World Health Organization Classifcation of Tumour of Haemato-poietic and Lymphoid Tissues. Lyon: IARC Press; 2008. pp. 262-264.

[2] Noy A. Controversies in the treatment of Burkitt lymphoma in AIDS. Curr Opin Oncol 2010; 22: 443-448.

[3] Haralambieva E, Rosati S, van Noesel C, Boers

E, van Marwijk KM, Schuuring E, Kluin P. Florid

granulomatous reaction in Epstein-Barr virus-positive nonendemic Burkitt lymphomas: re-port of four cases. Am J Surg Pathol 2004; 28: 379-383.

[4] Hollingsworth HC, Longo DL, Jaffe ES. Small noncleaved cell lymphoma associated with

florid epithelioid granulomatous response. A

clinicopathologic study of seven patients. Am J Surg Pathol 1993; 17: 51-59.

[5] Schrager JA, Pittaluga S, Raffeld M, Jaffe ES.

Granulomatous reaction in Burkitt lymphoma:

correlation with EBV positivity and clinical out-come. Am J Surg Pathol 2005; 29: 1115-1116.

[6] El Demellawy D, Otero C, Radhi J. Primary

gas-tric lymphoma with florid granulomatous reac

-tion. J Gastrointestin Liver Dis 2009; 18:

99-101.

[7] Janegova A, Janega P, Ilencikova D, Babal P. Burkitt lymphoma with unusual granuloma-tous reaction. A case report. Cesk Patol 2011; 47: 19-22.

[8] Scarabello A, Leinweber B, Ardigo M, Rutten A,

Feller AC, Kerl H, Cerroni L. Cutaneous lympho -mas with prominent granulomatous reaction: a potential pitfall in the histopathologic diagno-sis of cutaneous T- and B-cell lymphomas. Am J Surg Pathol 2002; 26: 1259-1268.

[9] Macak J, Smyslova O, Krc I, Duskova M,

Zapletal P. Hodgkin's disease with epithelioid

granulomatous reaction. Cesk Patol 1998; 34: 89-93.

[10] Kojima M, Nakamura S, Ichimura K, Suzuki R,

Kagami Y, Kondo E, Motoori T, Hosomura Y,

Oyama T, Itoh H. Centroblastic and centroblas-tic/centrocytic lymphoma associated with a prominent epithelioid granulomatous re-sponse: a clinicopathologic study of 50 cases. Mod Pathol 2002; 15: 750-758.

[11] Hossain A, God JM, Radwan FF, Amria S, Zhao

Figure

Figure 1. Prominent granulomas of different size are identified (H&E × 100) (A). Langhans giant cells are fairly easy to be observed in granulomas (H&E × 400) (B)
Figure 2. Immunohistochemistry (A-C). Atypical lymphocytes are positive for CD20 (A), CD10 (B), and nearly 100% of atypical lymphocytes are positive for Ki-67 (C) (× 200)
Figure 3. Representative interphase FISH results. FISH study using MYC dual-color break-apart rearrangement probe detects a dissociation of the red and green signals (A)

References

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