Human immunodeficiency virus-associated Burkitt lymphoma in a Japanese patient with early submandibular swelling: a case report
© Komatsu et al.; licensee BioMed Central Ltd. 2013
Received: 18 May 2013
Accepted: 16 December 2013
Published: 26 December 2013
Patients infected with the human immunodeficiency virus (HIV) are at risk of developing malignancies and have an increased susceptibility to infection. HIV-associated Burkitt lymphoma (BL) is relatively rare in developed countries, but remains prevalent in developing counties and is sometimes compounded by the fact that patients may be unaware that they are HIV-positive.
A 37-year-old Japanese man was referred to our department for diagnosis and management of submandibular swelling. He was unaware that he was HIV-positive at the initial visit. Here, we describe our diagnostic approach, in which we used hematological and immunological investigations, biopsy, fluorescence-activated cell sorting and fluorescence in situ hybridization to confirm the diagnosis of HIV-associated BL. The patient has no risk factors for HIV infection, and the source of infection remains unclear.
In this case, submandibular swelling was the first clinical sign of pathology and the patient’s HIV-positive status only became evident later. It is highly likely that BL was triggered by HIV infection.
Human immunodeficiency virus (HIV) was first reported in 1981, in a cohort of homosexual men with Pneumocystis carinii pneumonia . By 2011, the Joint United Nations Program on HIV/acquired immunodeficiency syndrome (AIDS) estimated that the number of adults and children living with HIV was between 31.4 million and 35.9 million. In developing countries, especially sub-Saharan Africa, widespread HIV infection has caused severe economic and social problems as a result of decreased life expectancy and increased childhood mortality . HIV-positive patients are more likely to develop malignant disease than healthy individuals because of the immunosuppressive effects of the virus. Moreover, it is not uncommon for patients to be unaware that they are infected with HIV.
Burkitt lymphoma (BL) was first reported in 1958 as a sarcoma of the jaw in a Ugandan patient . Spina et al.  and Straus  have reported that BL accounts for only 1–3% of lymphomas in HIV-seronegative adults, but this figure rises to 15–40% for AIDS-related lymphomas. We report an uncommon case of HIV-associated BL with clinical signs of submandibular swelling in a Japanese man.
HIV infection was treated with highly active anti-retroviral therapy at a specialist center, and BL was treated with four cycles of R-HyperCVAD/R-MA chemotherapy administered without radiotherapy. Two years after diagnosis, he continues to receive treatment at the same hospital with no evidence of recurrence. The patient has no apparent risk factors for HIV infection.
Burkitt lymphoma is a highly aggressive non-Hodgkin lymphoma (NHL) . According to the WHO classification, there are three clinical variants of BL: endemic, sporadic, and immunodeficiency-associated. Endemic BL develops in parts of Africa and New Guinea, in most cases at 4–7 years of age, with boys affected twice as frequently as girls . It can involve the jaw and other facial bones, kidneys, gastrointestinal tract, ovaries, breast, and other extranodal sites. Sporadic BL is a worldwide phenomenon with no specific geographic or climatic association , accounting for 1–2% of adult lymphomas and up to 40% of child lymphomas in the United States and Western Europe . Sporadic BL most commonly presents in the abdomen, ovaries, kidneys, omentum, and Waldeyer’s tonsillar rings. Endemic BL is strongly associated with EBV infection, but the etiology of sporadic BL has yet to be defined . Immunodeficiency-associated BL occurs in HIV-infected patients and allograft recipients . This case is highly likely to be an immunodeficiency-associated BL.
HIV-infected patients have a two-fold increased risk of developing malignant disease; in the head and neck the majority of cancers are Kaposi’s sarcoma or oral Kaposi’s sarcoma (68%), with squamous cell carcinoma and NHL accounting for 17% and 13%, respectively, and only 2% diagnosed as BL . Burkitt lymphoma is strongly associated with HIV infection, and HIV-associated BL accounts for approximately 5–40% of cases of HIV-associated NHL [6, 8, 11]. HIV-associated BL is rare in developed countries, despite being common in developing countries. However, the number of HIV carriers in Japan has recently increased, in part because of internationalization, which may be expected to lead to an increase in HIV-associated BL.
Generally, patients with submandibular swelling visit a dental clinic or are referred to a specialist center for investigation and treatment. In such cases, differential diagnoses that must be considered include inflammation of the submandibular gland, submental ranine, submental lymphadenitis, a tumor of the submandibular gland, a metastatic tumor, and ML. Thus, screening for infectious disease, notably for circulating antibodies against HIV and EBV, is important in cases where suspected BL cannot be differentiated from HIV-associated BL by clinical, radiological, histopathological and immunohistochemical analyses alone. Furthermore, those practicing in oral and maxillofacial medicine should take a history that includes questions regarding sexual history (e.g. sexual intercourse with different partners), travel history (especially to/from countries with many HIV carriers) and history of transfusion of blood products to facilitate differential diagnosis. In this case, the patient is neither homosexual nor has any history of travel to developing countries where HIV is prevalent.
In conclusion, this case emphasizes the importance of an integrated diagnostic approach for the early diagnosis and appropriate treatment of HIV-associated BL in the submandibular region.
Written informed consent was obtained from the patient for publication of this Case Report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
This study was performed in conformity with the Declaration of Helsinki, and was approved by our institutional ethical committee.
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