Linfomas de células T/NK das regiões oral e maxilofacial: um estudo multicêntrico / T/NK cell lymphomas of the oral and maxillofacial regions: a multicenter study

Os linfomas representam um grupo importante, complexo e heterogêneo de distúrbios proliferativos malignos originados a partir das células do tecido linfoide. Os linfomas de células T/NK maduras representam 12,7% a 26,2% dos linfomas não-Hodgkin, são condições relativamente raras, e a incidência da maioria das neoplasias aumenta com a idade. Portanto, o objetivo desse estudo é avaliar as características clinicopatológicas e imunoistoquímicas de uma série de linfomas de células T/NK maduras que acometem as regiões oral e maxilofacial e fornecer uma revisão atualizada da literatura sobre as bases biológicas desse grupo de doenças malignas. Casos diagnosticados como linfomas maduros de células T/NK afetando a região oral e maxilofacial foram recuperados retrospectivamente de seis centros de patologia oral e maxilofacial, e seus diagnósticos foram confirmados por meio de lâminas coradas com hematoxilina e eosina, reações imuno-histoquímicas e hibridização in situ para detecção do vírus Epstein-Barr (EBV). Um total de 22 casos foram incluídos neste estudo. Onze (50%) consistiam em linfomas extranodais de células T/NK, tipo nasal; oito (36,4%) eram linfomas periféricos de células T, sem outra especificação; dois (9,1%) eram leucemia/linfomas de células T, tipo adulto e um (4,5%) era um linfoma anaplásico de grandes células ALK-positivo. No geral, houve predomínio do sexo masculino, com média de idade de 55 anos. O palato foi o local mais acometido (50%), e os tumores geralmente se apresentavam como úlceras destrutivas e dolorosas. O EBV estava presente em todos os casos de linfoma de células T/NK extranodal tipo nasal, mas estava ausente nos outros subtipos.

Lymphomas represent an important, complex, and heterogeneous group of malignant proliferative disorders arising from lymphoid tissue cells. Mature T/NK cell lymphomas represent 12.7% to 26.2% of non-Hodgkin lymphomas, are relatively rare conditions, and the incidence of most neoplasms increases with age. Therefore, the aim of this study is to evaluate the clinicopathological and immunohistochemical characteristics of a series of mature T/NK cell lymphomas that affect the oral and maxillofacial regions and to provide an updated review of the literature on the biological basis of this group of malignancies. Cases diagnosed as mature T/NK cell lymphomas affecting the oral and maxillofacial region were retrospectively retrieved from six oral and maxillofacial pathology centers, and their diagnoses were confirmed by hematoxylin-and-eosinstained slides, immunohistochemical reactions, and in situ hybridization for detection of Epstein-Barr virus (EBV). A total of 22 cases were included in this study. Eleven (50%) consisted of extranodal T/NK cell lymphomas, nasal type; eight (36.4%) were peripheral T-cell lymphomas, not otherwise specified; two (9.1%) were T-cell leukemia/lymphomas, adult type and one (4.5%) was an ALK-positive anaplastic large cell lymphoma. Overall, there was a predominance of males, with a mean age of 55 years. The palate was the most affected site (50%), and the tumors usually presented as destructive and painful ulcers. EBV was present in all cases of nasal type extranodal T/NK cell lymphoma but was absent in the other subtypes.

EBER+ extranodal NK/T lymphoma in patient with idiopathic CD4 lymphocytopenia.

Idiopathic CD4 lymphocytopenia (ICL) not related to HIV is an infrequent and severe condition with no etiology defined until now. The concomitant presence of an underlying disease, especially an oncohematological process, could be related to the immune physiopathology and the development of the immunosuppressive state. On the other hand, Epstein Barr virus is a well-known oncogenic pathogen described in the development of several types of lymphoma which might be reactivated in the ICL. There is still no specific treatment for this syndrome, so the therapeutic scope for these patients is the treatment of opportunistic diseases and the administration of specific antimicrobials as prophylaxis. We present a patient with an uncommon association of an ICL and an extranodal T/NK lymphoma with detection of VEB nuclear RNA by in situ hybridization (EBER). Diagnosis was challenging which led the health team to carry out many studies over several months.

La linfocitopenia CD4 idiopática (ICL) no relacionada al HIV es una condición grave e infrecuente sin una etiología aún definida. La presencia de una enfermedad subyacente, especialmente un proceso oncohematológico, podría tener relación en la fisiopatología del proceso inmunológico. Por otro lado, el virus Epstein Barr (VEB) es bien conocido por ser un patógeno oncogénico descrito en el desarrollo de diversos tipos de linfomas, el cual podría ser reactivado en estados de inmunosupresión severa. No existe aún un tratamiento específico para este síndrome, por lo que el objetivo terapéutico en estos pacientes radica en el manejo profiláctico y activo de las distintas enfermedades oportunistas ante las cuales son susceptibles. Se presenta un paciente con un déficit grave de linfocitos CD4 de causa idiopática, y un diagnóstico posterior de linfoma T/NK extraganglionar con detección de RNA nuclear de VEB por hibridización in situ (EBER), una asociación poco descrita en la literatura médica.

EBER + extranodal NK/T lymphoma in patient with idiopathic CD4 lymphocytopenia / Linfoma T/NK EBER positivo en paciente con linfocitopenia CD4 idiopática

Abstract Idiopathic CD4 lymphocytopenia (ICL) not related to HIV is an infrequent and severe condition with no etiology defined until now. The concomitant presence of an underlying disease, especially an oncohematological process, could be related to the immune physiopathology and the development of the im munosuppressive state. On the other hand, Epstein Barr virus is a well-known oncogenic pathogen described in the development of several types of lymphoma which might be reactivated in the ICL. There is still no specific treatment for this syndrome, so the therapeutic scope for these patients is the treatment of opportunistic diseases and the administration of specific antimicrobials as prophylaxis. We present a patient with an uncommon asso ciation of an ICL and an extranodal T/NK lymphoma with detection of VEB nuclear RNA by in situ hybridization (EBER). Diagnosis was challenging which led the health team to carry out many studies over several months

Resumen La linfocitopenia CD4 idiopática (ICL) no relacionada al HIV es una condición grave e infrecuente sin una etiología aún definida. La presencia de una enfermedad subyacente, especialmente un proceso oncohematológico, podría tener relación en la fisiopatología del proceso inmunológico. Por otro lado, el virus Epstein Barr (VEB) es bien conocido por ser un patógeno oncogénico descrito en el desarrollo de diversos tipos de linfomas, el cual podría ser reactivado en estados de inmunosupresión severa. No existe aún un tratamiento específico para este síndro me, por lo que el objetivo terapéutico en estos pacientes radica en el manejo profiláctico y activo de las distintas enfermedades oportunistas ante las cuales son susceptibles. Se presenta un paciente con un déficit grave de linfocitos CD4 de causa idiopática, y un diagnóstico posterior de linfoma T/NK extraganglionar con detección de RNA nuclear de VEB por hibridización in situ (EBER), una asociación poco descrita en la literatura médica.