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 Table of Contents  
CASE REPORT
Year : 2020  |  Volume : 45  |  Issue : 2  |  Page : 115-117

Burkitt lymphoma presenting with nasopharyngeal mass and generalized lymphadenopathy in a HIV-positive patient


1 Department of Laboratory Medicine, Basvatarakam Indo American Cancer Hospital and Research Institute, India
2 Department of Medical Oncology, Basvatarakam Indo American Cancer Hospital and Research Institute, Hyderabad, Andhra Pradesh, India

Date of Submission22-Aug-2019
Date of Acceptance03-Apr-2020
Date of Web Publication29-Dec-2020

Correspondence Address:
Manasi Mundada
Department of Laboratory Medicine, Basvatarakam Indo American Cancer Hospital and Research Institute, Road No. 14 Banjara Hills, Hyderabad, Andhra Pradesh 500 034
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ejh.ejh_29_19

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  Abstract 


Burkitt lymphoma is an aggressive B-cell neoplasm arising from the germinal center B cells. Clinically, it exists in three forms: endemic, sporadic, and immunodeficiency associated. Immunodeficiency-associated Burkitt lymphoma is usually extranodal and occurs with a CD4 count more than 200 μl. We report a retrospective study of a 47-year-old male patient, seropositive for HIV infection, presenting with nasopharyngeal mass and generalized lymphadenopathy. His CD4 count was 192 cells/μl, and lactate dehydrogenase was high. Diagnosis was established by biopsy, immunohistochemistry, and fluorescence in-situ hybridization. He was treated with chemotherapy. The disease progressed to involve central nervous system despite treatment. He received palliative radiotherapy and was stable at 11-month follow-up. This report highlights the uncommon clinical presentation, heavy disease burden, and progression of the disease in a HIV seropositive patient.

Keywords: Burkitt lymphoma, HIV, nasopharyngeal mass


How to cite this article:
Mundada M, Ahmed F, Khera R, Murthy S, Boyella PK. Burkitt lymphoma presenting with nasopharyngeal mass and generalized lymphadenopathy in a HIV-positive patient. Egypt J Haematol 2020;45:115-7

How to cite this URL:
Mundada M, Ahmed F, Khera R, Murthy S, Boyella PK. Burkitt lymphoma presenting with nasopharyngeal mass and generalized lymphadenopathy in a HIV-positive patient. Egypt J Haematol [serial online] 2020 [cited 2021 Jul 31];45:115-7. Available from: http://www.ehj.eg.net/text.asp?2020/45/2/115/305398




  Introduction Top


Burkitt lymphoma (BL) is an aggressive B-cell neoplasm arising from the germinal center B cells [1]. Clinically it exists in three forms: endemic, sporadic, and immunodeficiency associated. All the subtypes are similar on morphology and at the genetic level but differ from each other in clinical presentation. Endemic BL is more common in children with more common involvement of the facial/jaw bone, whereas sporadic and immunodeficiency-related BL are more common in adults with involvement of gastrointestinal tract [1].

Immunodeficiency-associated BL is generally seen at CD4 count more than 200 μl, and risk of developing BL increases with increasing duration of immunodeficiency [2]. Herein, we report a patient with HIV-related BL presenting with nasopharyngeal mass and generalized lymphadenopathy.


  Case report Top


A 47-year-old male presented with the complaints of nasal block since 15 days. The patient was diagnosed as HIV positive 7 years back but was not taking highly active antiretroviral therapy (HAART). The CD4 count at presentation was 192 cells/μl. MRI showed a large lobulated mass measuring 3.8×2.7×2.7 cm in the nasopharynx extending anteriorly into nasal cavity ([Figure 1]). Extensive nodal involvement was noted in bilateral cervical, right axillary, and retroperitoneal regions. Lactate dehydrogenase levels were 1032 U/l (120–242 U/l). The vitals were stable, and the patient underwent endoscopy-guided biopsy. The hematoxylin and eosin-stained section from the nasopharyngeal biopsy showed monomorphic population of intermediate sized lymphoid cells arranged in sheets. The cells showed vesicular nucleus with clumped chromatin and scanty cytoplasm ([Figure 2]). Immunohistochemistry showed positivity for CD20, CD10, PAX5, and BCL6, whereas CD3, BCL2, and TdT were negative. Ki67 index was 98%. Fluorescence in-situ hybridization for CMYC was positive for the characteristic translocation ([Figure 3]). On evaluation, the patient was in stage III and was started on R-DA EPOCH (Rituximab-550 mg/kg i.v., dose adjusted Doxorubicin-18 mg i.v./kg, Vincristine-0.7 mg/kg i.v. day 1-4, Etoposide 90 mg/kg day 1–4, Endoxan-1300 mg i.v., and hydrocortisone 50 mg).
Figure 1 MRI T1 post-contrast sagittal image showed a well-defined mildly enhancing homogeneous mass lesion at the posterior nasopharyngeal wall.

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Figure 2 (a) Section shows nasal mucosa with underlying neoplastic lesion (×100, hematoxylin and eosin). (b) Section shows monomorphic lymphoid cells with intervening tingible body macrophages’ starry sky pattern (×400, hematoxylin and eosin).

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Figure 3 (a) Diffuse CD20 membrane positive in neoplastic cells (×400). (b) CD3 negative neoplastic cells (×400). (c) Diffuse CD10 membrane positive (×400). (d) KI67 index 98% (×400).

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PET-CT at 3 months showed partial response with disappearance of nasopharyngeal mass but persistence of nodal disease. At the end of fourth cycle, the patient developed headache, and on evaluation was found to have central nervous system (CNS) involvement. The chemotherapy was changed to R-HDMTx (Rituximab with high dose Methotrexate), and whole brain palliative radiotherapy was added. At the end of three cycles, patient improved symptomatically, but opted for palliative treatment owing to financial constraints. The patient is stable under regular follow-up and oral chemotherapy (Methotrexate 12.5 mg with Cytarabine 40 mg and hydrocortisone 50 mg) ([Figure 1],[Figure 2],[Figure 3]).


  Discussion Top


BL is one of the AIDS-associated lymphomas with a frequency of 2.4–20% [3] Immunodeficiency-related BL is seen most commonly in adults, with predominantly extranodal presentation. The common sites are gastrointestinal tract, liver, and bone marrow. HIV-associated BL shows high frequency of involvement of oral cavity, clinically presenting as large intraoral masses with swelling involving the gingiva, jaw, or maxilla [3]. Our patient presented with a nasopharyngeal mass with lymph node involvement, both of which were uncommon. Bone marrow and CNS involvement is commonly described in immunodeficiency-related BL, and our patient developed CNS involvement over the course of the disease, despite treatment [1].

Immunodeficiency-related BL is known to present at late stages of disease with extensive disease burden, and the risk of developing lymphoma increases with the duration of immunosuppression [4]. The disease is known to manifest in immunocompetent host with CD4 counts more than 200 μl, suggesting the role of CD4 counts in disease pathogenesis [2]. Our patient was seropositive for HIV since 7 years, not receiving HAART, and the CD4 count was 192/μl. The patient presented with high disease burden and high lactate dehydrogenase levels. The morphology, immunohistochemistry, and molecular tests were classical and confirmatory for the diagnosis.

Intensive chemotherapy regimens with HAART are the mainstay of treatment. Regimens like CODOX-M/IVAC developed by Corti [3] were proven to be clinically efficient with 90% cure rates. Chemotherapy with EPOCH-R was started in our patient; he is stable and on regular follow-up.

This report is to highlight the uncommon synchronous nodal and extranodal presentation of BL in a patient with HIV infection. This uncommon clinical scenario raises the suspicion of metastatic nasopharyngeal carcinoma and requires prompt biopsy and conscientious use of ancillary techniques to arrive at the correct diagnosis. The patient presented with widespread disease and hence associated with poor prognosis.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Leoncini L, Campo E, Stein H, Harris NL, Jaffe ES, Kluin PM. Burkitt lymphoma. In Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H et al. eds. WHO classification of tumours of haematopoietic and lymphoid tissues. Revised 4th edn. Lyon:IARC Press; 2017. 330–334.  Back to cited text no. 1
    
2.
Said J, Cesarman E, Rosenwald A, Harris NL. Lymphomas associated with HIV infection. In Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H et al. eds. WHO classification of tumours of haematopoietic and lymphoid tissues. Revised 4th edn. Lyon: IARC Press; 2017. 449–452.  Back to cited text no. 2
    
3.
Corti M. Burkitt’s lymphoma associated with HIV Infection. Clin Microbiol 2016; 5:232.  Back to cited text no. 3
    
4.
Stroup JS, Bransteitter BA, Stephens JR, Hendrickson SE. Burkitt lymphoma in an adult HIV positive patient. Infect Dis Clin Pract 2007; 15:116–118.  Back to cited text no. 4
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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