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BACKGROUND Angiocentric centrofacial lymphomas, now known as nasal-type extranodal natural killer T-cell lymphomas, are neoplasms of highly destructive characteristics that mainly affect the nasal cavity and palate. The most frequent clinical presentation includes fever, weight loss, nasal obstruction, epistaxis, nasal or facial edema, as well as necrotic ulcers in the nasal cavity, gums, and palate. It has been found to have an important association with the Epstein-Barr virus. Diagnostic pathology could be difficult due to the typical widespread tissue necrosis. CASE REPORT A 72-year-old Caucasian male sought medical attention with a chief complaint of nasal obstruction for the past 3 years, which only responded partially to unspecific treatment. He also presented with intermittent fever and nocturnal hyperhidrosis. Physical examination with rhinoscopy demonstrated a deviated septum, congestive turbines, and fragile and pale mucous membrane with yellowish, thick mucus. The pathology report described an angiocentric centrofacial lymphoma and a positive serology for Epstein-Barr virus. CONCLUSIONS The objective of this case report was to show that this illness represents a diagnostic challenge for the treating physician. It may be concluded that despite the poor prognosis of the disease, this particular case showed slower evolution and the patient remained stable despite multiple consecutive complications.
This article was published in the following journal.
Name: The American journal of case reports
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An angiocentric and angiodestructive lymphoproliferative disorder primarily involving the lungs. It is caused by an Epstein-Barr virus-induced transformation of the B-cells, in a T-cell rich environment. Clinically and pathologically it resembles EXTRANODAL NK-T-CELL LYMPHOMA.
A leukemia/lymphoma found predominately in children and young adults and characterized LYMPHADENOPATHY and THYMUS GLAND involvement. It most frequently presents as a lymphoma, but a leukemic progression in the bone marrow is common.
B-cell lymphoid tumors that occur in association with AIDS. Patients often present with an advanced stage of disease and highly malignant subtypes including BURKITT LYMPHOMA; IMMUNOBLASTIC LARGE-CELL LYMPHOMA; PRIMARY EFFUSION LYMPHOMA; and DIFFUSE, LARGE B-CELL, LYMPHOMA. The tumors are often disseminated in unusual extranodal sites and chromosomal abnormalities are frequently present. It is likely that polyclonal B-cell lymphoproliferation in AIDS is a complex result of EBV infection, HIV antigenic stimulation, and T-cell-dependent HIV activation.
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A form of undifferentiated malignant LYMPHOMA usually found in central Africa, but also reported in other parts of the world. It is commonly manifested as a large osteolytic lesion in the jaw or as an abdominal mass. B-cell antigens are expressed on the immature cells that make up the tumor in virtually all cases of Burkitt lymphoma. The Epstein-Barr virus (HERPESVIRUS 4, HUMAN) has been isolated from Burkitt lymphoma cases in Africa and it is implicated as the causative agent in these cases; however, most non-African cases are EBV-negative.