Primary peripheral T-cell lymphoma of the cervix with mononeuritis multiplex: an unusual case presentation

Peripheral neuropathy (PN) is characterized by the injury to the peripheral nervous system of varied etiology. Lymphoma is one of the etiologies of PN, presenting various neurological manifestations. Neuropathy associated with peripheral T-cell lymphoma, not otherwise specified (PTCL, NOS) is unusual and fewer cases are documented in the literature. In addition, PTCL, NOS is extremely rare as primary in the female genital tract, especially uterine cervix, and exhibits aggressive clinical course with poor therapy response. We hereby describe a 47-year-old female who presented with fever and chills for 15 days. Clinical examination revealed left-sided lower motor neuron type of facial nerve palsy with Bell's phenomenon. Nerve conduction study of all four limbs illustrated asymmetrical axonal neuropathy (motor > sensory), suggesting mononeuritis multiplex. She developed vaginal bleeding during her hospital stay. Pelvic examination and imaging revealed a 4x3cm polypoidal mass on the posterior lip of the cervix, which was excised and diagnosed as extranodal primary PTCL, NOS based on morphology, immunohistochemistry, and in-situ hybridization findings. Besides, the cerebrospinal fluid (CSF) was infiltrated by the lymphoma cells, detected on cell block preparation. The patient succumbed to her illness within one week despite best efforts and the commencement of chemotherapy. No consent was obtainable for nerve biopsy and autopsy. Thus, we report an extremely rare case of primary extranodal PTCL, NOS of the uterine cervix with unusual presentation of mononeuritis multiplex. Further, we discussed the differentials of PTCL, NOS at this extranodal site.

Linfoma de células NK/T subtipo nasal ­ relato de caso / Nasal subtype NK/T cell lymphoma ­ case report

RESUMO O linfoma de células NK/T subtipo nasal tem baixa incidência e alta agressividade, mostrando-se necessário diagnóstico precoce. Tal se mostra desafiador devido à sua manifestação clínica inicial, semelhante a quadro infeccioso de vias aéreas superiores. Por meio deste estudo observacional descritivo, relatam-se casos deste linfoma acometendo paciente masculino e outro feminino, ambos com diagnóstico tardio e com envolvimento de vias aéreas superiores sem resposta à antibioticoterapia. À exame de imagem, apresentaram lesão expansiva em cavidade nasal e o diagnóstico se deu por meio de estudo histológico com marcadores imuno-histoquímicos de células NK atípicas e presença de vírus Epstein-Barr (EBV). PALAVRAS-CHAVE: Linfoma de células NK/T tipo nasal, Epstein-Barr vírus, linfoma não-hodgkin, linfoma de células T periférico

ABSTRACT Nasal subtype NK/T cell lymphoma has a low incidence and high aggressiveness, and early diagnosis is necessary. This is challenging due to its initial clinical manifestation, similar to an upper airway infection. Through this descriptive observational study, we report cases of this lymphoma affecting a male and a female patient, both with late diagnosis and involvement of the upper airways without response to antibiotic therapy. On imaging scan, they presented an expansive lesion in the nasal cavity and the diagnosis was made through histological study with immunohistochemical markers of atypical NK cells and the presence of Epstein-Barr virus (EBV). KEYWORDS: NK/T-cell lymphoma nasal type, Epstein-Barr virus, Lymphoma non Hodgkin, T-cell lymphoma

Peripheral T-cell lymphoma not otherwise specified with oral manifestation: a case report / Linfoma periférico de células T no especificado con manifestación oral: reporte de caso

Peripheral T-Cell Lymphoma Not Otherwise Specified it is a rare type of Non-Hodgkin t-cells malignant tumor whose oral manifestations are difficult to diagnose. A case of a 48-year-old male with a hemi-maxillary lesion histological and immunohistochemically compatible with Peripheral T-Cell Lymphoma not otherwise specified is presented. A case of a 48-year-old male with a hemi-maxillary lesion histological and immunohistochemically compatible with Peripheral T-Cell Lymphoma not otherwise specified is presented. The patient treatment consisted of chemotherapy, but after the second cycle, died from immunosuppressive complications. Early stage diagnosis of oral lesions is imperative to avoid aggressive treatment and low overall survival rate of such pathologies.

Introducción: El linfoma periférico de células T no especificado es un tipo raro de tumor maligno no Hodgkin de células T cuyas manifestaciones orales son difíciles de diagnosticar. Se presenta el caso de un varón de 48 años con lesión hemimaxilar histológica e inmunohistoquímicamente compatible con linfoma periférico de células T no especificado. El tratamiento del paciente consistió en quimioterapia, pero después del segundo ciclo, falleció por complicaciones inmunosupresoras. El diagnóstico temprano de las lesiones orales es imperativo para evitar un tratamiento agresivo y la baja tasa de supervivencia.

How to manage lymphoid malignancies during novel 2019 coronavirus (CoVid-19) outbreak: a Brazilian task force recommendation

ABSTRACT: The novel Coronavirus (CoVid-19) outbreak is now consider a world pandemic, affecting more than 1,300,000 people worldwide. Cancer patients are in risk for severe disease, including a higher risk of intensive care unit (ICU) admission, need for invasive ventilation or death. Management of patients with lymphoid malignancies can be challenging during the outbreak, due to need of multiple hospital visits and admissions, immunosuppression and need for chemotherapy, radiotherapy and stem cell transplantation. In this article, we will focus on the practical management of patients with lymphoid malignancies during the COVID-19 pandemic, focusing on minimizing the risk for patients.

Adult T-cell leukemia/lymphoma treatment in Bahia, Brazil

Abstract Background: Adult T-cell leukemia/lymphoma is a peripheral disease associated with human T-cell lymphotropic virus type 1. Treatment is carried out according to clinical type with watchful waiting being recommended for less aggressive types. Aggressive adult T-cell leukemia/lymphoma is generally treated with chemotherapy and/or antivirals. The objective of this study was to correlate the survival of patients diagnosed in Bahia, Brazil, with the therapeutic approaches employed and to evaluate what issues existed in their treatment processes. Methods: Eighty-three adult T-cell leukemia/lymphoma patients (26 smoldering, 23 chronic, 16 acute, 13 lymphoma and five primary cutaneous tumoral) with available data were included in this study. Results: Complete response was achieved in seven smoldering patients with symptomatic treatment, in two with chronic disease using antivirals/chemotherapy, in one with acute disease using antivirals and in one lymphoma using the LSG15 regimen [vincristine, cyclophosphamide, doxorubicin, and prednisolone (VCAP); doxorubicin, ranimustine, and prednisolone (AMP); and vindesine, etoposide, carboplatin, and prednisolone (VECP)]. Smoldering patients who received symptomatic treatment presented longer survival. Favorable chronic patients treated with antivirals presented longer survival compared to the unfavorable subtype. However, for the acute form, first-line chemotherapy was better, albeit without significance, than antivirals. Only one of the patients with lymphoma and primary cutaneous tumors responded. Conclusions: Watchful waiting associated with phototherapy represents the best option for smoldering adult T-cell leukemia/lymphoma with survival in Bahia being superior to that described in Japan. There was a trend of better results with zidovudine/interferon-alpha in favorable chronic disease. Excellent results were achieved in the lymphoma type treated with the LSG15 protocol. Patients are diagnosed late probably due to lack of knowledge of adult T-cell leukemia/lymphoma by primary healthcare doctors and a Brazilian treatment protocol needs to be established.

Recurrence of peripheral T-cell lymphoma as granulomas in the lower limbs / Granulomas epitelióides cutâneos, como manifestação de recidiva de linfoma de células T periférico

The presence of granulomas in the skin of T-cell lymphoma patients is a rare but well-known phenomenon. The authors present the case of a 44-year-old Caucasian male, with a previously treated peripheral T-cell lymphoma, with cutaneous infiltration and extensive dyschromia on his lower limbs. Skin biopsies revealed the presence of sarcoid granulomas associated with the recurrence of the lymphoma, confirmed by immunostaining and molecular analysis. Although a new scheme of chemotherapy was started, he died 18 months later. There are two different patterns of skin granulomas associated with lymphoma: granulomatous infiltrates admixed with neoplastic cells and cutaneous granulomas constituting a nonspecific manifestation of the underlying lymphoma, but presently there is no evidence supporting their relationship with lymphoma prognosis.

A presença de granulomas cutâneos associados a linfomas é um fenômeno raro, mas bem conhecido. Um homem, 44 anos de idade, com diagnóstico prévio de linfoma de células T periférico, foi enviado à nossa consulta por infiltração cutânea e extensa discromia nos membros inferiores. A biopsia cutânea revelou a presença de granulomas epitelioides associados à recidiva do linfoma, confirmada pela marcação imuno-histoquímica e estudo molecular. Apesar de iniciado novo esquema de quimioterapia, o doente faleceu 18 meses depois. Na literatura são descritos dois tipos de granulomas cutâneos na presença de linfomas: associados à infiltração cutânea pelo linfoma ou como uma manifestação não específica do linfoma. Contudo, atualmente não há evidência que suporte a sua relação com o prognóstico dos linfomas.

Ruptura esplénica espontánea asociada a linfoma periférico de células T, presentación de un caso y revisión de la literatura / Spontaneous splenic rupture associated with peripheral T-cell lymphoma

Se presenta un caso de ruptura esplénica espontánea asociada a shock hemorrágico en un paciente de 24 años. Después del manejo quirúrgico, se demostró la presencia de un linfoma periférico de células T. Se hace una breve revisión de la literatura actualizada alrededor de las posibilidades diagnósticas de la ruptura esplénica espontánea.

The case of a 24 year old male with hemorrhagic shock associated with spontaneous splenic rupture is presented. After surgical management, a peripherical t cell lymphoma was demonstrated through immunohistochemistry.Up to date literature is reviewed, as well as possible differential diagnosis related to spontaneous splenic rupture.

T-Cell Project: an international, longitudinal, observational study of patients with aggressive peripheral T-cell lymphoma / Projeto de célula-T: estudo internacional, longitudinal de pacientes com linfoma de célula-T periférica agressivo

Peripheral T-cell lymphomas (PTCLs) comprise a heterogeneous group of neoplasms that are derived from post-thymic lymphoid cells at different stages of differentiation and with different morphological patterns, phenotypes, and clinical presentations. PTCLs are highly diverse, reflecting the diverse cells from which they can originate and are currently sub-classified using World Health Organization (WHO) 2008 criteria. Peripheral T-Cell Lymphomas account for 5 percent-10 percent of all lymphoproliferative disorders in the Western hemisphere, with an overall incidence of 0.5-2 per 100,000 individuals per year, and have a striking epidemiological distribution, with higher incidence in Asia. The clinical features of PTCL are extremely heterogeneous. PTCLs express even more clinical diversity than B-cell non-Hodgkin's lymphomas, and there is a close, though not absolute, relationship between some unusual clinical features and certain histological subtypes.

Linfomas T periféricos (PTCLs) compreendem um grupo heterogêneo de neoplasias que derivam das células linfoides pós-tímicas nos diversos estágios de maturação, com diversos padrões histológicos, fenotípicos, e clínicos. PTCLs são muito diversos entre si e refletem diversas células das quais foram originadas e são atualmente subclassificadas, usando-se a classificação da Organização Mundial da Saúde (OMS) 2008, apresentada neste texto na tabela 1. PTCLs compreendem 5 por cento-10 por cento de todas as doenças linfoproliferativas no mundo ocidental, com uma incidência global de 0.5 a 2 a cada 100.000 pessoas por ano e têm uma distribuição epidemiológica diversa com maior incidência na Ásia. Os achados clínicos dos PTCLs são muito heterogêneos. PTCLs expressam muito maior variação de apresentações clínicas do que os linfomas B, e há uma íntima, mas não absoluta, relação entre algum achado clínico não usual e certos subtipos histológicos. O autor faz aqui uma revisão do assunto altamente contemporâneo

Sinusite complicada ou algo mais? Relato de caso / Complicated sinusitis or something else? Case report.

JUSTIFICATIVA E OBJETIVOS: O linfoma periférico de células NK e T tipo nasal é de difícil diagnóstico, em se considerando vários aspectos tais como a relativa raridade da doença, diversidade dos achados clínicos, necessidade de exames complementares sofisticados, amostras adequa­das para biópsia e, ainda, um confuso padrão morfológico celular. Este cenário propicia retardo de tempo para obten­ção do diagnóstico final, tempo este precioso, em se consi­derando a agressividade e refratariedade aos tratamentos. O objetivo deste artigo foi relatar um caso deste tipo de linfoma "mascarado" por sinusite e que, ainda com trata­mento instituído, motivou o óbito do paciente. RELATO DO CASO: Paciente do sexo masculino, 22 anos, apresentando sinusite havia seis meses sem resposta ao tra­tamento com antibióticos e procedimentos cirúrgicos com biópsia. Internado na enfermaria de Clínica Médica, devi­do a persistência de sintomas sugestivos de sinusite crônica, apresentando edema periorbitário à direita, lesão ulcerada na hemiface direita e ausência de linfonodos. Os exames de imagem foram compatíveis com sinusite e biópsia prévia demonstrou processo inflamatório inespecífico, sem células atípicas. Foi realizado ultra-sonografia (US) de abdômen que demonstrou hepatoesplenomegalia. O paciente apresentou pneumonia nosocomial, hemorragia digestiva alta piora da função renal e hepática; após o resultado do exame ­anatomopatológico da biópsia de pele compatível com linfoma, foi iniciado tratamento com imunossupressor, porém sem resposta adequada e consequente evolução ao óbito. CONCLUSÃO: O diagnóstico precoce do linfoma periférico de células NK e T tipo nasal a despeito de sua raridade é de extrema relevância pela necessidade de tratamento precoce.

Presentación clínica inusual del linfoma intestinal: fistulización ileosigmoidea / Unusual clinical presentation of intestine lymphoma: iliosigmoid fistulization

Los linfomas primarios de intestino son infrecuentes, representando aproximadamente el 12 por ciento de los tumores primitivos del intestino delgado y en el colon entre el 0,2 por ciento y 0,65 por ciento. La epidemiología ha cambiado radicalmente en relación al siglo pasado, hecho vinculado particularmente con el advenimiento de la inmunosupresión. Se analiza un caso clínico de un linfoma no Hodgkin primario de intestino, con una presentación clínica no habitual que según la clasificación REAL, modificada por la PDQ se trata de un linfoma agresivo de la variedad T periférico. Inicialmente operada de urgencia, se realiza la resección en bloque de sigmoides, asa delgada, anexo izquierdo, pared anterolateral de abdomen y músculo ilíaco, reconstruyendo el tránsito en forma videoasistido 4 meses después e iniciando posteriormente tratamiento de quimioterapia de consolidación.

Linfomas B y T: diagnóstico inmunofenotípico de los linfomas no hodgkinianos / B-and T-lymphomas: immunophenotypical diagnosis of non-Hodgkin lymphomas

Los linfomas no hodgkinianos son neoplasias linfoides que pueden presentar fenotipo de células B ó T; las de tipo B son más frecuentes, mientras que los linfomas de tipo T tienen peor pronóstico y por lo general son más agresivos. En el ámbito internacional, la sobrevida de estas enfermedades es de aproximadamente 5 años, y en nuestro país se encuentran entre las 10 primeras causas de cáncer en ambos sexos, con prevalencia ligeramente mayor en el sexo masculino (1,3:1). Se destaca además, entre algunos aspectos, la importancia del estudio inmunofenotípico para determinar la naturaleza de estos procesos neoplásicos y distinguir entre enfermedad de Hodgkin, linfomas no hodgkinianos e infiltraciones por otros procesos linfoides, también para poder establecer factores pronósticos y de progresión en estos desórdenes linfoproliferativos.

Diferenciación inmunofenotípica de un sindrome linfoproliferativo crónico y linfoma periférico / Differentiation immunophenotyping of a chronic lymphoproliferative syndrome and peripheral lymphoma

Paciente de 80 años, a quien cuatro años atrás se le trató un linfoma gástrico de células grandes. En la visita actual mostró un hemograma básicamente normal, salvo ligera leucocitosis linfocítica, en la que un 50 por ciento de sus elementos eran de aproximadamente 15 un de diámetro y mostraban características prolinfocíticas de tipo neoplásico, por la presencia de un prominente nucleólo inserto en una matriz cromatínica de textura intermedia. A fin de aclarar la naturaleza del larvado proceso leucémico, se utilizaron técnicas citoquímicas (ANAE, CAE, PX y FAC), y un panel de Ac monoclonales para los estudios en el citómetro de flujo. Los resultados demostraron la índole linfosarcomatosa del proceso, el cual inmunofenotípicamente resultó ser un linfoma periférico de células tumorales B diferenciadas (CD5+/CD19+) tipo kappa

Peripheral T-cell lymphoma in HIV-positive patients

Two cases of peripheral T-cell lymphomas in HIV-positive patients are reported: one case of T-pleomorphic small cellnon-Hodgkin lymphoma in a 27 year-ol bisexual male, and one case of a T-pleomorphic medium and large cell non-Hodgkin lymphoma in a 27 year-old female, whose husband was drug addicted. Both cases were studied on a morphological and immunohistological basis