The authors describe a peculiar form of Epstein-Barr virus (EBV)-associated T-cell lymphoma in an HIV-positive patient presenting an aggressive clinical course. Unlike most other EBV-positive T-cell/natural-killer (NK)-cell lymphomas, the disease was characterized by predominant nodal involvement at presentation. T-cell lineage was confirmed by T-cell receptor-rearrangement, and neoplastic cells exhibited strong and diffuse CD56 expression. A marked intravascular component was detected in the skin, the liver, and the lung parenchyma. This entity was not predicted in the WHO 2008 classification, but has been recently identified in immunocompromised patients. This case report refers to a middle-aged man with AIDS, who presented a 4-month history of weight loss, fever, hepatosplenomegaly, peripheral and deep-chain lymphadenopathy. A blood smear showed lymphocytosis with a marked presence of atypia. The outcome was unfavorable and the patient could not be treated. The autopsy revealed multivisceral involvement, including lymph nodes, spleen, bone marrow, liver, lungs, skin, and kidneys.
KiesslingR, KleinE, WigzellH. “Natural” killer cells in the mouse. I. Cytotoxic cells with specificity for mouse Moloney leukemia cells. Specificity and distribution according to genotype. Eur J Immunol. 1975;5(2):112-7. [https://doi.org/10.1002/eji.1830050208]. [PMID:1234049]
KiesslingR, KleinE, ProssH, WigzellH. “Natural” killer cells in the mouse. II. Cytotoxic cells with specificity for mouse Moloney leukemia cells. Characteristics of the killer cell. Eur J Immunol. 1975;5(2):117-21. [https://doi.org/10.1002/eji.1830050209]. [PMID:1086218]
HerbermanRB, NunnME, LavrinDH. Natural cytotoxic reactivity of mouse lymphoid cells against syngeneic acid allogeneic tumors. I. Distribution of reactivity and specificity. Int J Cancer. 1975;16(2):216-29. [https://doi.org/10.1002/ijc.2910160204]. [PMID:50294]
HerbermanRB, NunnME, HoldenHT, LavrinDH. Natural cytotoxic reativity of mouse lymphoid cells against syngeneic acid allogeneic tumors. II. Characterization of effector cells. Int J Cancer. 1975;16(2):230-9. [https://doi.org/10.1002/ijc.2910160205]. [PMID:1080480]
RaphaelM, SaidJ, BorischB, CesarmanE, HarrisNL. Lymphomas associated with HIV infections. In: SwerdlowSH, CampoE, HarrisNL, et al., editors. WHO classification of tumors of haematopoietic and lymphoid tissues. IARC: Lyon; 2008; p. 340-2.
JaffeES. Classification of natural killer (NK) cell and NK-like T-cell malignancies. Blood. 1996;87(4):1207-10. [PMID:8608206].
EmileJF, BoullandML, HaiounC, et al. CD5-CD56+ T-cell receptor silent peripheral T-cell lymphomas are natural killer cell lymphomas. Blood. 1996;87(4):1466-73. [PMID:8608237].
GualcoG, Domeny-DuarteP, ChioatoL, BarberG, NatkunamY, BacchiCE. Clinicopathologic and molecular features of 122 Brazilian cases of nodal and extranodal NK/T-cell lymphoma, nasal type, with EBV subtyping analysis. Am J Surg Pathol. 2011;35(8):1195-203. [https://doi.org/10.1097/PAS.0b013e31821ec4b5]. [PMID:21716086]
AuWY, WeisenburgerDD, IntragumtornchaiT, et al. Clinical differences between nasal and extranasal natural killer/T-cell lymphoma: a study of 136 cases from the International Peripheral T-Cell Lymphoma Project. Blood. 2009;113(17):3931-7. [https://doi.org/10.1182/blood-2008-10-185256]. [PMID:19029440]
ChanJKC, SinVC, WongKF, et al. Nonnasal lymphoma expressing the natural killer cell marker CD56: a clinicopathologic study of 49 cases of an uncommon aggressive neoplasm. Blood. 1997;89(12):4501-13. [PMID:9192774].
ChimCS, MaESK, LoongF, KwongYL. Diagnostic cues for natural killer cell lymphoma: primary nodal presentation and the role of in situ hybridisation for Epstein-Barr virus encoded early small RNA in detecting occult bone marrow involvement. J Clin Pathol. 2005;58(4):443-5. [https://doi.org/10.1136/jcp.2004.022608]. [PMID:15790718]
AttygalleAD, CabeçadasJ, GaulardP, et al. Peripheral T-cell and NK-cell lymphomas and their mimics; taking a step forward - report on the lymphoma workshop of the XVIth meeting of the European Association for Haematopathology and the Society for Hematopathology. Histopathology. 2014;64(2):171-99. [https://doi.org/10.1111/his.12251]. [PMID:24128129]
NagahataY, KatoA, ImaiY, IshikawaT. HIV-related NK/T-cell lymphoma in the brain relapsed during intensive chemotherapy but regressed after chemotherapy discontinuation: the importance of maintaining cellular immunity. Int J Hematol. 2014;100(4):402-6. [https://doi.org/10.1007/s12185-014-1610-2]. [PMID:24938379]
CanioniD, ArnulfB, Asso-BonnetM, RaphaelM, BrousseN. Nasal natural killer lymphoma associated with Epstein-Barr virus in a patient infected with human immunodeficiency virus. Arch Pathol Lab Med. 2001;125(5):660-2. [PMID:11300939].