Mycosis fungoides refractory to treatment - importance of a multidisciplinary approach.

We report a case of difficult-to-control mycosis fungoides (MF), where the role of the dental surgeon was crucial for the control and prognosis of the disease. A 62-year-old female patient diagnosed with MF had a previous record of red patches and small raised bumps on the face, along with a cancerous growth in the cervical and vulvar region. The patient was initially treated with methotrexate and local radiotherapy without resolution. Chemotherapy with cyclophosphamide, doxorubicin, vincristine, and prednisone was then started (CHOP protocol). The dental team of a reference hospital was consulted to evaluate swelling in the anterior region of the palate, which had been developing for two months, reporting discomfort when eating. The role of the dentistry team was fundamental in the differential diagnosis of oral lesions with dental infections, second neoplasia, or even a new site of disease manifestation, in addition to controlling mucosal changes resulting from chemotherapy. After ruling out dental infection, the dentistry team performed a lesion biopsy to confirm the diagnosis. The histopathological and immunohistochemical analysis showed atypical lymphoid infiltration of T cells (CD3+/CD4+/CD7-/CD8-), coexpression of CD25, and presence of CD30 cells, corresponding to the finding for MF. Identifying CD30 + allowed for a new chemotherapy protocol with brentuximab vedotin (BV) combined with gemcitabine. This protocol effectively controlled MF, which previous protocols had failed to do. The diagnosis by the dental team was essential for therapeutic change and improvement of the patient's clinical condition without the need for invasive medical procedures.

The Epidemiological Particularities of Malignant Hemopathies in French Guiana: 2005-2014.

French Guiana is a French Overseas territory with singular features: it has a high prevalence of HIV and HTLV-1, its population is ethnically mixed, with widespread poverty, and up to 20% of the population lives in geographic isolation. In this context, we used registry data to estimate incidence and mortality due to hematological malignancies and to compare them with France and tropical Latin America. ICD codes C90 and C88 were compiled between 2005 and 2014. The direct standardization of age structure was performed using the world population. Survival analysis was performed, and Kaplan-Meier curves were drawn. The overall standardized incidence rate was 32.9 per 100,000 male years and 24.5 per 100,000 female years. Between 2005 and 2009, the standardized incidence rate was 29.6 per 100,000 among men and 23.6 per 100,000 among women, and between 2010 and 2014, it was 35.6 per 100,000 among men and 25.2 per 100,000 among women. Multiple myeloma/plasmocytoma and mature t/NK cell lymphomas, notably adult t-cell lymphoma/leukemia due to HTLV-1 infection, were the two most common hematologic malignancies and causes of death. Non-Hodgkin's lymphoma incidence estimates were greater than global estimates. After adjusting for age, sex, and type of malignancy, people born in a foreign country independently had a poorer case-fatality rate, presumably reflecting difficulties in accessing care. The epidemiology of hematological malignancies in French Guiana has features that distinguish it from mainland France or from Latin America. The incidence of multiple myeloma and adult t-cell lymphoma/leukemia was significantly greater in French Guiana than in France or other Latin American countries.

Extranodal natural killer/T cell lymphoma nasal type simulating osteoradionecrosis with metachronic B lymphoma in the pelvis: Case report.

Extranodal Natural Killer/T Cell Lymphoma Nasal Type (EN-NK/T-CL-NT) is a non-Hodgkin extranodal lymphoma of unfavorable prognosis due to its aggressive nature. This neoplasm mainly affects the paranasal sinuses, nasopharynx, oropharynx, oral cavity, palate, and rarely intestinal, gastric and skin regions. 50-year-old female with a history of lymphoma in nasal and pelvic region. At four years of tumors-free, has facial asymmetry, accompanied by sub-palpebral, nasal and lip edema. Intraoral examination revealed a large ulceration suggestive of osteoradionecrosis. Gum biopsy shows Extranodal NK/T Cell Lymphoma Nasal Type (EN-NK/T-CL-NT). In this case we highlight the characteristics of EN-NK/T-CL-NT with a presentation of osteoradionecrosis-like. Unfortunately, the nature of this tumor led to the patient's death. Clinical follow-up of patients with cancer is imperative to mend and/or decrease treatment complications, as well as to identify second primary tumors or the spread of the underlying disease.

Decrease of 5-hydroxymethylcytosine in primary cutaneous CD4+ small/medium sized pleomorphic T-cell lymphoproliferative disorder

Abstract Background: Primary cutaneous CD4+ small/medium-sized pleomorphic T-Cell lymphoproliferative disorder (PC-SMTLD) has been considered as a controversial dermatological disease that has been included in cutaneous T-cell lymphoma group, presenting most commonly as a solitary nodule and/or plaque with a specific and characteristic head and neck predilection. Due to the considerable overlap between PC-SMTLD and pseudolymphoma (PL), the differential diagnosis is often challenging. Methylation of DNA at position 5 of cytosine, and the subsequent reduction in intracellular 5-hydroxymethylcytosine (5-hmC) levels, is a key epigenetic event in several cancers, including systemic lymphomas. However, it has rarely been studied in cutaneous lymphomas. Objectives: The authors aimed to explore the role of differential 5-hmC immunostaining as a useful marker to distinguish PC-SMTLD from PL. Methods: Retrospective case series study with immunohistochemical and immunofluorescence analysis of 5-hmC was performed in PL and PC-SMTLD. Results: Significant decrease of 5-hmC nuclear staining was observed in PC-SMTLD when compared with PL (p<0.0001). By semi-quantitative grade integration, there were statistical differences in the final 5-hmC scores in the two study groups. The IF co-staining of 5-hmC with CD4 revealed a decrease of 5-hmC in CD4+ lymphocytes of PC-SMTLD. Study limitations: The small clinical sample size of the study. Conclusions: The immunorreactivity of 5-hmC in CD4+ lymphocytes was highly suggestive of a benign process as PL. Furthermore, the decrease of 5-hmC nuclear staining in PC-SMTLD indicated its lymphoproliferative status and helped to make the differential diagnosis with PL. © 2023 Sociedade Brasileira de Dermatologia. Published by Elsevier España, S.L.U. This is an open access article under the CC BY license (https://creativecommons.org/licenses/by/4.0/).

Integration of T-cell clonality screening using TRBC-1 in lymphoma suspect samples by flow cytometry.

BACKGROUND: The diagnosis of T-cell non-Hodgkin lymphomas (NHL) is challenging. The development of a monoclonal antibody specific for T-cell receptor ß constant region 1 (TRBC1) provides an alternative to discriminate clonal T cells. The aim of this study was to evaluate the diagnostic potential of an anti-TRBC1 mAb for the identification of T-NHL. METHODS: We performed a cross-sectional diagnostic analytic study of samples tested for lymphoma. All samples sent for lymphoma screening were first evaluated using the standard Euroflow LST, to which a second additional custom-designed T-cell clonality assessment tube was added CD45/TRBC1/CD2/CD7/CD4/TCRγδ/CD3. Flow cytometry reports were compared with morphological and molecular tests. RESULTS: Fifty-nine patient samples were evaluated. Within the T-cell population, cut-off percentages in the CD4+ cells were from 29.4 to 54.6% and from 23.9 to 52.1% in CD8+ cells. Cut-off ratios in CD4+ T cells were from 0.33 to 1.1, and in CD8+ cells between 0.22 and 1.0. Using predefined normal cut-off values, 18 of 59 (30.5%) samples showed a restricted expression of TRBC1. A final diagnosis of a T-NHL was confirmed clinically and/or by histopathological studies in 15 of the 18 cases (83.3%). There were no cases of T-NHL by morphology/IHC with normal TRBC1 expression. Non-neoplastic patient samples behaved between predefined TRBC1 cut-off values. CONCLUSIONS: Expression of TRBC1 provides a robust method for T-cell clonality assessment, with very high sensitivity and good correlation with complementary methods. TRBC1 can be integrated into routine lymphoma screening strategies via flow cytometry.

Decrease of 5-hydroxymethylcytosine in primary cutaneous CD4+ small/medium sized pleomorphic T-cell lymphoproliferative disorder.

BACKGROUND: Primary cutaneous CD4+ small/medium-sized pleomorphic T-Cell lymphoproliferative disorder (PC-SMTLD) has been considered as a controversial dermatological disease that has been included in cutaneous T-cell lymphoma group, presenting most commonly as a solitary nodule and/or plaque with a specific and characteristic head and neck predilection. Due to the considerable overlap between PC-SMTLD and pseudolymphoma (PL), the differential diagnosis is often challenging. Methylation of DNA at position 5 of cytosine, and the subsequent reduction in intracellular 5-hydroxymethylcytosine (5-hmC) levels, is a key epigenetic event in several cancers, including systemic lymphomas. However, it has rarely been studied in cutaneous lymphomas. OBJECTIVES: The authors aimed to explore the role of differential 5-hmC immunostaining as a useful marker to distinguish PC-SMTLD from PL. METHODS: Retrospective case series study with immunohistochemical and immunofluorescence analysis of 5-hmC was performed in PL and PC-SMTLD. RESULTS: Significant decrease of 5-hmC nuclear staining was observed in PC-SMTLD when compared with PL (p < 0.0001). By semi-quantitative grade integration, there were statistical differences in the final 5-hmC scores in the two study groups. The IF co-staining of 5-hmC with CD4 revealed a decrease of 5-hmC in CD4+ lymphocytes of PC-SMTLD. STUDY LIMITATIONS: The small clinical sample size of the study. CONCLUSIONS: The immunorreactivity of 5-hmC in CD4+ lymphocytes was highly suggestive of a benign process as PL. Furthermore, the decrease of 5-hmC nuclear staining in PC-SMTLD indicated its lymphoproliferative status and helped to make the differential diagnosis with PL.

Comparative analysis of upper aerodigestive tract and non-upper aerodigestive tract in NK/T-cell lymphoma.

OBJECTIVE: Nasal or extranasal natural killer/T-cell lymphoma (NKTCL) is a very rare aggressive lymphoma, but it is increasingly diagnosed. To evaluate some specificity by comparative analysis between primary upper aerodigestive tract (UAT) and non-upper aerodigestive tract (NUAT)NKTCL. METHODS: A retrospective analysis was performed on NKTCL patients from January 2013 to November 2022 in our cancer center. RESULTS: The majority of the lesions were UAT-NKTCL 70 cases (92.1%), the primary NUAT occurred in 6 cases. Patients in the UAT group were mainly in the early stage and in the low and medium risk, while those in the NUAT group were late stage and in high risk (p = 0.000). The expressions of CD3 and TIA-1 in UAT group were higher than those in NUAT group (p = 0.031, p = 0.003), while CD7 was dominant in NUAT group (p = 0.009). For early stage NKTCL, multivariate analysis suggested that gender and PINK score were independent factors affecting PFS and OS (p < 0.05). The 3 year OS rate in initial CR group was 90.1% versus 46.4% in non-CR group (p = 0.000). In advanced stage, KI67% and bone marrow involvement were independent factors affecting OS (p = 0.022, p = 0.038). CONCLUSION: It was difficult to distinguish between UAT and NUAT-NKTCL from histopathology. NUAT-NKTCL patients did have advanced stage and poor outcome. The prognostic value of PINK score and bone marrow involvement was proposed. We aimed to improve initial CR rates, as well as to find new predictive models to predict the whole population.

Autologous, allogeneic hematopoietic cell transplantation and CAR-T/NK therapy: what is their real importance in PTCL?

Peripheral T cell lymphoma (PTCL) is a rare and aggressive type of non-Hodgkin's lymphoma that affects mature T cells. This type of cancer is characterized by the abnormal growth of T cells, which can accumulate in the lymph nodes, spleen, bone marrow, and other organs, leading to a variety of symptoms. PTCLs are often difficult to diagnose and treat, and they have a poorer prognosis than other types of lymphoma. However, recent advancements in treatment options, such as targeted therapies have shown promise in improving outcomes for patients with PTCL. Here, we discuss the use of autologous and allogeneic hematopoietic cell transplantation (HCT) as a treatment strategy for patients with PTCL, as well as the recent treatment approaches based on advanced cellular therapy. The current evidence for the use of HCT in PTCL is mainly derived from registry data, retrospective studies, and expert opinion, as randomized trials are limited due to the low incidence and histological heterogeneity of PTCL subtypes.

Extranodal NK-/T-cell lymphoma, nasal type: what advances have been made in the last decade?

Extranodal NK-/T-cell lymphoma (ENKTCL) is a rare and highly aggressive malignancy with significant racial and geographic variations worldwide. In addition to the formerly "nasal-type" initial description, these lymphomas are predominantly extranodal in origin and typically cause vascular damage and tissue destruction, and although not fully understood, Epstein-Barr virus (EBV) has an important role in its pathogenesis. Initial assessment must include a hematopathology review of representative and viable tumor areas without necrosis for adequate immunohistochemistry studies, including EBV-encoded small RNA (EBER) in situ hybridization (ISH). Positron emission tomography with 18-fluorodeoxyglucose (18F-FDG-PET/CT) for accurate staging is essential, and most patients will have localized disease (IE/IIE) at diagnosis. Apart from other T-cell malignancies, the best treatment even for localized cases is combined modality therapy (chemotherapy plus radiotherapy) with non-anthracycline-based regimens. For advanced-stage disease, l-asparaginase-containing regimens have shown improved survival, but relapsed and refractory cases have very poor outcomes. Nowadays, even with a better understanding of pathogenic pathways, up-front therapy is completely based on chemotherapy and radiotherapy, and treatment-related mortality is not low. Future strategies targeting signaling pathways and immunotherapy are evolving, but we need to better identify those patients with dismal outcomes in a pre-emptive way. Given the rarity of the disease, international collaborations are urgently needed, and clinical trials are the way to change the future.

Blistering and aggressive paniculite cutaneous T-cell lymphoma.

BACKGROUND: Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is characterized by atypical T-cells expressing the α/ß T-cell receptor in the subcutaneous fat. Although it is usually indolent, some cases can show an aggressive course. It is usually a disease of the middle-aged, but can rarely affect children. CASE REPORT: We describe the case of a 12-year-old male, previously healthy, who presented a dermatosis disseminated to the four segments consisting of vesicles, blisters, erythematous and hematonecrotic plaques, atrophic scars, associated with edema. The biopsy confirmed limited cutaneous panniculitic T-cell lymphoma with extensive epidermal necrosis. CONCLUSIONS: We report the case of a SPTCL in a child. Although rare in this age group, the diagnosis should be considered in children who present similar conditions and who do not respond to treatment. Diagnosis is made on clinical suspicion and confirmed by histology. We discuss the challenges in its management and how timely diagnosis influences patient survival.

INTRODUCCIÓN: El linfoma de células T tipo paniculitis subcutánea (LCCTP) se caracteriza por la presencia de linfocitos T atípicos que expresan el receptor de células T α/ß en el tejido celular subcutáneo. Aunque generalmente es indolente, algunos casos presentan un curso agresivo. Es mayormente una enfermedad de la mediana edad, rara vez afecta a los niños. CASO CLÍNICO: Se describe el caso de un paciente de sexo masculino de 12 años de edad, previamente sano que presentó una dermatosis diseminada a los cuatro segmentos constituida por vesículas, ampollas, placas eritematocostrosas y hematonecróticas, además de atróficas, asociadas con edema. La biopsia confirmó linfoma cutáneo de células T paniculítico con extensa necrosis epidérmica. CONCLUSIONES: Reportamos el caso de un LCCTP en un paciente pediátrico. Aunque es raro en este grupo de edad, se debe considerar en los niños que presentan cuadros similares y que no responden a tratamiento. El diagnóstico se realiza por sospecha clínica y se confirma por histología. Se discuten los desafíos en su manejo y cómo el diagnóstico oportuno influye en la sobrevida del paciente.

Linfoma gástrico primario a células T, no asociado a HTLV1, con presentación inicial de fractura patológica. Reporte de caso

El linfoma de células T en adultos (ATLL) es una neoplasia agresiva de linfocitos T, por lo general asociada con el virus linfotrópico T humano tipo 1 (HTLV-1), de presentación clínica abigarrada. Los linfomas gástricos primarios son generalmente linfoma no Hodgkin (NH) tipo B, y un mínimo porcentaje por linfocitos T. Es escasa la literatura sobre linfomas gástricos primarios por células T con HTLV-1 Negativo y que hacen metástasis ósea. Para ilustrar esta situación, se presenta el caso de un adulto de 41 años, que ingresa por una fractura patológica. A los 15 días presenta diarrea, distensión abdominal, y una endoscopia alta encuentra "Lesiones ulceradas gástricas, aspecto neoproliferativo". La biopsia informó Linfoma No Hodgkin a células maduras linfocito T; y la prueba de HTLV1 fue negativa. Se realizaron tres sesiones de quimioterapia con esquema CHOEP. Hubo respuesta favorable, saliendo de alta; sin embargo, no retorna para proseguir la terapia. El paciente regresó dos meses después en mal estado general; luego presentó falla multiorgánica, produciéndose su deceso.

Adult T-cell lymphoma (ATLL) is an aggressive T-cell neoplasm, usually associated with human T-lymphotropic virus type 1 (HTLV-1), with a variegated clinical presentation. Primary gastric lymphomas are generally non-Hodgkin lymphoma (NH) type B, and a minimal percentage are due to T lymphocytes. There is little literature on primary gastric lymphomas due to HTLV-1 Negative T cells that metastasize to bone. To illustrate this situation, the case of a 41-year-old adult who is admitted for a pathological fracture is presented. 15 days later, he developed diarrhea and abdominal distension, and an upper endoscopy found "gastric ulcerated lesions, neoproliferative appearance". The Biopsy reported Non-Hodgkin Lymphoma to mature T lymphocyte cells; and the HTLV1 test was negative. Three chemotherapy sessions were performed with the CHOEP scheme. There was a favorable response, and he was discharged; However, he did not return to continue therapy. The patient returned 2 months later in poor general condition. He then presented multiple organ failure, resulting in his death.

Angioimmunoblastic T-cell lymphoma and correlated neoplasms with T-cell follicular helper phenotype: from molecular mechanisms to therapeutic advances.

Angioimmunoblastic T-cell lymphoma (AITL) is the second most frequent subtype of mature T-cell lymphoma (MTCL) in the Western world. It derives from the monoclonal proliferation of T-follicular helper (TFH) cells and is characterized by an exacerbated inflammatory response and immune dysregulation, with predisposition to autoimmunity phenomena and recurrent infections. Its genesis is based on a multistep integrative model, where age-related and initiator mutations involve epigenetic regulatory genes, such as TET-2 and DNMT3A. Subsequently, driver-mutations, such as RhoA G17V and IDH-2 R172K/S promote the expansion of clonal TFH-cells ("second-hit"), that finally begin to secrete cytokines and chemokines, such as IL-6, IL-21, CXCL-13 and VEGF, modulating a network of complex relationships between TFH-cells and a defective tumor microenvironment (TME), characterized by expansion of follicular dendritic cells (FDC), vessels and EBV-positive immunoblasts. This unique pathogenesis leads to peculiar clinical manifestations, generating the so-called "immunodysplastic syndrome", typical of AITL. Its differential diagnosis is broad, involving viral infections, collagenosis and adverse drug reactions, which led many authors to use the term "many-faced lymphoma" when referring to AITL. Although great advances in its biological knowledge have been obtained in the last two decades, its treatment is still an unmet medical need, with highly reserved clinical outcomes. Outside the setting of clinical trials, AITL patients are still treated with multidrug therapy based on anthracyclines (CHOP-like), followed by up-front consolidation with autologous stem cell transplantation (ASCT). In this setting, the estimated 5-year overall survival (OS) is around 30-40%. New drugs, such as hypomethylating agents (HMAs) and histone deacetylase inhibitors (HDAi), have been used for relapsed/refractory (R/R) disease with promising results. Such agents have their use based on a biological rationale, have significant potential to improve the outcomes of patients with AITL and may represent a paradigm shift in the therapeutic approach to this lymphoma in the near future.

Mycosis fungoides and Sézary syndrome: clinical presentation, diagnosis, staging, and therapeutic management.

Mycosis fungoides (MF) and Sézary syndrome (SS) are cutaneous T-cell lymphomas. MF is the most common cutaneous lymphoma, and it is classified into classic Alibert-Bazin MF, folliculotropic MF, pagetoid reticulosis, and granulomatous slack skin, each with characteristic clinical presentation, histopathological findings, and distinct clinical behaviors. SS is an aggressive leukemic variant of cutaneous lymphoma, and it is characterized by erythroderma, lymphadenopathy, and peripheral blood involvement by malignant cells. There is a wide range of dermatological manifestations of MF/SS, and prompt recognition is essential for early diagnosis. Skin biopsy for histopathology and immunohistochemical analysis is imperative to confirm the diagnosis of MF/SS. Histopathology may also provide information that may influence prognosis and treatment. Staging follows the TNMB system. Besides advanced stage, other factors associated with poorer prognosis are advanced age, male gender, folliculotropism in histopathology of patients with infiltrated plaques and tumors in the head and neck region, large cell transformation, and elevated lactate dehydrogenase. Treatment is divided into skin-directed therapies (topical treatments, phototherapy, radiotherapy), and systemic therapies (biological response modifiers, targeted therapies, chemotherapy). Allogeneic bone marrow transplantation and extracorporeal photopheresis are other treatment modalities used in selected cases. This review discusses the main clinical characteristics, the histopathological/immunohistochemical findings, the staging system, and the therapeutic management of MF/SS.

Long-time follow-up of patients with untreated peripheral T cell lymphoma following chidamide combined with cyclophosphamide, epirubicin, vindesine, prednisone, and etoposide therapy: a single-center propensity score-matching study.

PURPOSE: This is a retrospective, single-center PSM study evaluating the efficacy and safety of chidamide combined with the CHOEP (C-CHOEP) regimen versus the single CHOEP regimen in patients with untreated peripheral T cell lymphomas (PTCL). PATIENTS: Patients newly diagnosed with PTCL between January 2015 and June 2021 were recruited, and were 1:1 divided into C-CHOEP and CHOEP groups according to their first-line chemotherapy regimens. The PSM method was used to match the baseline variables to balance the confounding factors. RESULTS: A cohort of 33 patients each in the C-CHOEP and CHOEP groups was generated after propensity score-matching (PSM). The complete remission (CR) rates of the C-CHOEP regimen were higher than that of the CHOEP regimen (56.3 vs. 25.8%, p = 0.014), whereas the duration of response of the C-CHOEP group was shorter (median DOR 30 vs. 57 months), resulting in roughly similar progression-free survival (PFS) and (overall survival) OS between the two groups. The responding patients who received chidamide maintenance therapy showed a trend of superior PFS and OS compared with patients who did not receive maintenance therapy. CONCLUSIONS: The C-CHOEP regimen was well tolerated but failed to show advantages over the CHOEP regimen in patients with untreated PTCL; however, the chidamide maintenance may contribute to a more durable response and stable long-term survival.

Infección por virus linfotrópico de células T humanas: Síndrome de Sezary asociado a paraparesia espástica tropical apropósito de un caso / Human T-cell lymphotropic virus infection: Sezary syndromeassociated with tropical spastic paraparesis: a case report

El virus linfotrópico de células T humanas tipo 1(HTLV-1, por sus siglas en inglés) es parte de la familia de los Retroviridae, perteneciente al género de los Delta retrovirus, está compuesto por una envoltura lipídica, obtenida de la célula huésped, en la superficie expresa proteínas transmembrana que le permite el anclaje e internalización por endocitosis al citoplasma celular. En su interior cuenta con una hebra de ARN de cadena simple en sentido positivo, además de las enzimas integrasa y transcriptasa inversa que forman la núcleo cápside icosaédrica. El virus linfotrópico de células T humanas está ampliamente distribuido a nivel mundial. Existen múltiples vías de transmisión (Transmisión vertical, interacciones sexuales, transfusiones sanguíneas, uso de drogas ilícitas endovenosas y el contacto de fluidos cargados de viriones con las mucosas). El 90% de los pacientes expuestos no desarrollaran síntomas, pero existe un 10% de los pacientes que desarrollaran el cuadro clínico. El HTLV-1 se asocia a dos cuadros clínicos bien establecidos: la paraparesia espática tropical y el linfoma cutáneo-T-leucemia de células T. Al ser inusual, presentándose en 1 de cada 100.000 habitantes, se discute el caso de una paciente femenina de 63 años de edad, con antecedentes de acalasia corregida quirúrgicamente, quien consulta con cuadro clínico de 2 meses de duración, caracterizado por debilidad progresiva simétrica en miembros inferiores que le impide la deambulación, incontinencia urinaria, lesiones cutáneas extensas y la presencia de hiperleucocitosis con más de 20% de blastos en sangre periférica, se realiza inmunofenotipo expresando que el 85% de linfocito T neoplásicos, resultando en leucemia de células T o síndrome de Sezary, posteriormente se confirma el diagnóstico al realizar Elisa de cuarta generación positivo para HTLV-1(AU)

The human T-cell lymphotropic virus is part of the Retroviridae family, belonging to the Delta retrovirus genus, thisvirus is composed of a lipid envelope, obtained from the hos tcell, on the surface it expresses transmembrane proteins that allow it to anchor and internalization by endocytosis into the cell cytoplasm. Inside it has a single-stranded RNA strand the positive direction, in addition to the enzymes integrase andreverse transcriptase that form the icosahedral nucleo capsid. Human T-cell T-cell lymphotrophic virus is widely distribute dworldwide. There are multiple routes of transmission (vertical transmission, sexual interactions, blood transfusions, use of intravenous illicit drugs and contact of virion-laden fluidswith mucous membranes). 90% of exposed patients will not develop symptoms, but there is 10% of patients who will develop the clinical picture, HTLV-1 is associated with twowell-established clinical pictures: tropical spastic paraplegia and cutaneous T-cell lymphoma. T-cell leukemia. As it is unusual, occurring in 1 out of every 100,000 inhabitants, the caseof a 63-year-old female patient with a history of surgically corrected achalasia is discussed, who consults with a clinical picture of 2 months duration, characterized due to progressive symmetrical weakness in the lower limbs that prevent walking, urinary incontinence, extensive skin lesions and the presence of hyperleukocytosis with more than 20% of blasts in peripheralblood, an immunophenotype is performed, expressing that 85% of neoplastic T lymphocytes, resulting in (T-cell leukemia) Sesary syndrome, diagnosis is later confirmed by performing afourth generation Elisa positive for HT LV-1(AU)

Linfoma de células T/natural killer extranodal de tipo nasal enmascarado en una sinusopatia

Introducción: El linfoma de células T citotóxico/natural killer extranodal de tipo nasal es poco frecuente, pero con alta tasa de mortalidad. Las manifestaciones clínicas de la enfermedad pueden simular una infección de senos paranasales. Objetivo: Presentar las manifestaciones clínicas de un paciente de 34 años de edad con diagnóstico de linfoma de células T citotóxico/natural killer extranodal de tipo nasal. Caso clínico: Se presenta un paciente masculino de 34 años de edad con rinorrea verdosa fétida recurrente y obstrucción en fosa nasal derecha. En la evaluación inicial sugiere sinusitis crónica, sin embargo, debido al empeoramiento de las manifestaciones clínicas se realiza una tomografía computarizada que muestra lesiones sugestivas de infiltración neoplásica, una biopsia de la lesión confirma el diagnóstico de linfoma de células T/natural killer extranodal de tipo nasal. Conclusiones: Los linfomas de células T citotóxico/natural killer extranodal de tipo nasal son considerados neoplasias poco frecuentes, caracterizadas por el patrón rápidamente progresivo con afectación ósea; en su etapa inicial presenta manifestaciones clínicas similares a una sinusitis. La tomografía computarizada y la histopatología, son indispensables en el diagnóstico de la enfermedad.

Introduction: Nasal-type extranodal natural killer/cytotoxic T-cell lymphoma is rare but has a high mortality rate. The clinical manifestations of the disease can mimic a paranasal sinus infection. Objective: To present the clinical manifestations of a 34-year-old patient diagnosed with nasal-type extranodal natural killer/cytotoxic T-cell lymphoma. Clinical case: A 34-year-old male patient with recurrent greenish fetid rhinorrhea and obstruction in the right nostril is presented. In the initial evaluation, it suggests chronic sinusitis, however, due to the worsening of the clinical manifestations, a computed tomography is performed that shows lesions suggestive of neoplastic infiltration, a biopsy of the lesion confirms the diagnosis of T-cell lymphoma/extranodal natural killer. Conclusions: Nasal-type extranodal natural killer/cytotoxic T-cell lymphomas are considered rare neoplasms characterized by a rapidly progressive pattern with bone involvement; in its initial stage it presents clinical manifestations similar to sinusitis. Computed tomography and histopathology are essential in the diagnosis of the disease.

Linfoma de células T/NK primario de testículo. Caso clínico y revisión de la literatura / Primary testicular NK/T cell lymphoma. Case report and review of literature

Natural killer/T cell lymphomas chiefly involving the midline facial structures including the nasal cavity or nasopharyns are a relatively rare type of non-Hodgkin's lymphoma. Apart from the upper respiratory tract, the disease occasionally presents in certain extranodal sites, such as the central nervous system, skin, gastrointestinal tract, or testes. We report a case of natural killer NK/T cell lymphoma as a testicular tumor in a 36-year-old man with a history of progressive swelling of his right testicle. Histologically, the testicular mass showed a diffuse infiltrate of medium-sized and atypical large lymphoid cells with angiocentric infiltration and areas of coagulative necrosis. Immunohistochemical studies demonstrated tumor cells staining positively with CD3, TIA-1, and Granzyme B. The Epstein-Barr virus genoma was detected by in situ hybridization. There were no abnormal findings in the nasal and nasopharyngeal regions. Classified as stage IEA, the patient received involved-field irradiation to contralateral testis (45 Gy), followed by systemic chemotherapy with a combination regimen ofL-asparaginase, methotrexate and dexamethasone. Relevant literature is reviewed, and the clinicopathologic features, natural history, and treatment options for primary testicular NK/T cell lymphoma are discussed.

Gastric perforation secondary to T-cell lymphoma.

Introduction: Malignant primary lymphoma represents only 1%-5% of all gastric tumours. Spontaneous gastric perforation in the absence of chemotherapy in these cases is extremely rare. The vast majority of primary gastric lymphomas have a B-cell phenotype that originates from mucosa-associated lymphoid tissue and primary gastric lymphomas with a T-cell phenotype are rarely reported. This report describes a case of a primary gastric T-cell malignant lymphoma associated to spontaneous perforation and peritonitis. Case presentation: An 80-year-old woman referring 24 hours of abdominal pain associated to cognitive impairment consulted to our Emergency Department. Her past medical history revealed smoking, hypothyroidism, dilated cardiomyopathy, hypertension, celiac disease with poor adherence to gluten-free diet and a Non-Hodgkin T cell lymphoma associated to enteropathy in 2010. At physical examination, she presented with tachycardia, hypotension and abdominal tenderness. Lab test revealed low red cell count and an abdomen computed tomography scan showed pneumoperitoneum secondary to a large gastric perforation located in the anterior wall of the antrum. Urgent surgery was performed. At exploratory laparoscopy, a 5 cm perforation of the anterior wall of prepyloric antrum was observed associated to a 4-quadrant peritonitis. Conversion to open surgery was decided to perform an open antrectomy and Billroth II gastro-jejunostomy. The patient was transferred to ICU after surgery under mechanical respiratory assistance for closed monitoring but evolved with a cardiogenic shock and deceased on the first postoperative day. The final histopathological and immunohistochemical analysis reported enteropathy-associated T-cell lymphoma of gastric localisation with concomitant celiac disease. Discussion: We present a rare case of a patient with a history of celiac disease who developed a gastric perforation secondary to an enteropathy-associated T-cell lymphoma of gastric localisation. To the best of authors' knowledge, there have been reported less than 30 cases of spontaneous perforation of gastric lymphoma in the absence of chemotherapy in the last 35 years. Malignant gastric lymphoma, accounting only for 1% of primary gastric malignancies, is usually a diffuse large B-cell lymphoma. Incidence of perforation of gastric lymphomas in patients receiving chemotherapy rounds 0.9%-1.1%. However, it is a rare condition in patients not receiving chemotherapy. Conclusion: This is a rare case of a patient with an enteropathy-associated T-cell lymphoma of gastric localisation, who developed a spontaneous gastric perforation in the absence of chemotherapy. Despite it is a rare condition, it must be suspected in patients with a history of lymphoma in the context of acute abdominal pain.

Linfoma cutáneo de células T paniculítico, ampolloso y agresivo / Blistering and aggressive paniculite cutaneous T-cell lymphoma

Resumen Introducción: El linfoma de células T tipo paniculitis subcutánea (LCCTP) se caracteriza por la presencia de linfocitos T atípicos que expresan el receptor de células T α/β en el tejido celular subcutáneo. Aunque generalmente es indolente, algunos casos presentan un curso agresivo. Es mayormente una enfermedad de la mediana edad, rara vez afecta a los niños. Caso clínico: Se describe el caso de un paciente de sexo masculino de 12 años de edad, previamente sano que presentó una dermatosis diseminada a los cuatro segmentos constituida por vesículas, ampollas, placas eritematocostrosas y hematonecróticas, además de atróficas, asociadas con edema. La biopsia confirmó linfoma cutáneo de células T paniculítico con extensa necrosis epidérmica. Conclusiones: Reportamos el caso de un LCCTP en un paciente pediátrico. Aunque es raro en este grupo de edad, se debe considerar en los niños que presentan cuadros similares y que no responden a tratamiento. El diagnóstico se realiza por sospecha clínica y se confirma por histología. Se discuten los desafíos en su manejo y cómo el diagnóstico oportuno influye en la sobrevida del paciente.

Abstract Background: Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is characterized by atypical T-cells expressing the α/β T-cell receptor in the subcutaneous fat. Although it is usually indolent, some cases can show an aggressive course. It is usually a disease of the middle-aged, but can rarely affect children. Case report: We describe the case of a 12-year-old male, previously healthy, who presented a dermatosis disseminated to the four segments consisting of vesicles, blisters, erythematous and hematonecrotic plaques, atrophic scars, associated with edema. The biopsy confirmed limited cutaneous panniculitic T-cell lymphoma with extensive epidermal necrosis. Conclusions: We report the case of a SPTCL in a child. Although rare in this age group, the diagnosis should be considered in children who present similar conditions and who do not respond to treatment. Diagnosis is made on clinical suspicion and confirmed by histology. We discuss the challenges in its management and how timely diagnosis influences patient survival.