Neoplasia de células dendríticas plasmocitoides blásticas, un reporte de caso / Blast plasmacytoid dendritic cell neoplasia, a case report

Introducción: La neoplasia de células dendríticas plasmocitoides blásticas (NCDPB) es una patología agresiva y representa menos del 1% de neoplasias hematológicas, se caracteriza por lesiones cutáneas nodulares violáceas sin evidencia de adenopatías en la mayoría de casos. Estudios observacionales demuestran que el Protocolo de quimioterapia Hyper-CVAD y la consolidación con tras-plante de células progenitoras hematopoyéticas se han asociado con una mayor supervivencia general. Caso Clínico: mujer de 82 años con antecedentes de Diabetes Mellitus tipo con cinco meses de lesiones hiperpigmentadas, elevadas, induradas, violáceas no dolorosas en mejilla, brazos, tórax anterior y posterior y piernas. Evolución: En citometría de flujo se determinó un fenotipo compatible con células patológicas (5.86%) con CD123++, HLADR+++, NG2++, CD56+++, CD4++, que sugiere una NCDPB. La biopsia de médula ósea presentó infiltración. PET CT posterior a terapia corticoide: no evidencia enfermedad tumoral macroscópica metabólicamente activa. Se inicia tratamiento con Dexametasona, con lo que las lesiones cutáneas disminuyeron en un 80%. Se inició Quimioterapia Protocolo CHOP like, ha recibido 6 ciclos hasta octubre del 2021, actualmente en remisión completa. Conclusión: En el presente caso el curso clínico de la NCDPB no fue agresivo hasta el momento del cierre del caso presentando disminución del 80% de las lesiones.

Introduction: blast plasmacytoid dendritic cell neoplasia (BPDCN) is an aggressive pathology and represents less than 1% of hematological neoplasms, it is characterized by violaceous nodular skin lesions without evidence of adenopathy in most cases. Observational studies show that the Hyper-CVAD chemotherapy protocol and consolidation with transplantation of hematopoietic progenitor cells have been associated with greater overall survival. Clinical case: a 82-year-old woman with a history of type Diabetes Mellitus with five months of hyperpigmented, raised, indurated, non-painful violaceous lesions on the cheek, arms, anterior and posterior thorax and legs. Evolution: Flow cytometry determined a phenotype compatible with pathological cells (5.86%) with CD123 ++, HLADR +++, NG2 ++, CD56 +++, CD4 ++, which suggests a BPDCN. The bone marrow biopsy showed infiltration. PET CT after corticosteroid therapy: there is no evidence of metabolically active macroscopic tumor disease. Dexamethasone treatment was started, with which skin lesions decreased by 80%. The CHOP-like Chemotherapy Protocol was started, she has received 6 cycles until October 2021, currently in complete remission. Conclusion: In the present case, the clinical course of NCDPB was not aggressive until the moment of closure of the case, presenting a decrease of 80% of the lesions.

Mycosis fungoides and Sézary syndrome: focus on the current treatment scenario

Abstract Cutaneous T-cell lymphomas are a heterogeneous group of lymphoproliferative disorders, characterized by infiltration of the skin by mature malignant T cells. Mycosis fungoides is the most common form of cutaneous T-cell lymphoma, accounting for more than 60% of cases. Mycosis fungoides in the early-stage is generally an indolent disease, progressing slowly from some patches or plaques to more widespread skin involvement. However, 20% to 25% of patients progress to advanced stages, with the development of skin tumors, extracutaneous spread and poor prognosis. Treatment modalities can be divided into two groups: skin-directed therapies and systemic therapies. Therapies targeting the skin include topical agents, phototherapy and radiotherapy. Systemic therapies include biological response modifiers, immunotherapies and chemotherapeutic agents. For early-stage mycosis fungoides, skin-directed therapies are preferred, to control the disease, improve symptoms and quality of life. When refractory or in advanced-stage disease, systemic treatment is necessary. In this article, the authors present a compilation of current treatment options for mycosis fungoides and Sézary syndrome.

Linfomas cutáneos: serie de casos en México / A case series of cutaneous lymphomas in Mexico

Resumen Introducción: Los linfomas cutáneos primarios son un grupo heterogéneo de neoplasias de células T y B que se presentan en la piel, sin ninguna evidencia de enfermedad extracutánea en el momento del diagnóstico, y muestran diferencias considerables en histologia, fenotipo y pronóstico. Se consideran neoplasias poco frecuentes. Casos clínicos: Se presentan cinco casos de linfomas cutáneos diagnosticados en el Hospital Infantil de México Federico Gómez durante el periodo de 2010 a 2018. Las presentaciones clínicas más frecuentes en estos pacientes fueron dermatitis, costras hemáticas y úlceras necróticas. El inmunofenotipo más común fue el linfoma cutáneo no Hodgkin T/NK extranodal nasal primario. El esquema de tratamiento que se utilizó en la mayoría de los pacientes fue SMILE. El promedio de tiempo al diagnóstico fue de 7 meses. Conclusiones: El pronóstico depende del estadio de la enfermedad al diagnóstico, grado de afectación de la piel y presencia o ausencia de enfermedad extracutánea. Los linfomas cutáneos primarios son neoplasias poco frecuentes. Debido al diagnóstico tardío, el estadio de la enfermedad suele ser avanzado, por lo que, generalmente, el comportamiento es agresivo.

Abstract Background: Primary cutaneous lymphomas are a rare heterogeneous group of T and B cell skin neoplasms without any evidence of extracutaneous disease at the time of diagnosis, which show considerable differences in histology, phenotype and prognosis. Case reports: Five cases of cutaneous lymphomas treated at the Hospital Infantil de México Federico Gómez from 2010 to 2018 are described. The most frequent clinical presentations in these patients were dermatitis, blood scabs, and necrotic ulcers. The most common immunophenotype was non-Hodgkin T/NK primary nasal extranodal cutaneous lymphomas. The treatment scheme used in most patients was SMILE. The average time to diagnosis was 7 months. Conclusions: The prognosis depends on the stage of the disease at diagnosis, the degree of skin involvement, and the presence of extracutaneous disease. As primary cutaneous lymphomas are infrequent neoplasms, the stage of the disease is usually advanced and generally shows an aggressive behavior due to a late diagnosis.

Case for diagnosis. Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder

Abstract: Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder is a rare disease, with an indolent evolution and benign course. The classic presentation is a solitary nodule on the face or trunk. The disorder's rarity and clinical and histopathological characteristics, can make the diagnosis difficult. We present the case of a 36-year-old Caucasian woman with a purplish erythematous nodule, hardened, shiny, asymptomatic, on the left nasal ala, which had grown progressively for 45 days. Histopathological examination and immunohistochemistry panel demonstrated alterations consistent with primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder. There was complete remission of the condition within 60 days of treatment with potent occlusive corticosteroids.

Cutaneous T-cell lymphomas in the revised 4th edition of World Health Organization classification of tumors of hematopoietic and lymphoid tissues (2017)

Abstract: Recently, the World Health Organization published the revised 4th edition of its classification of tumors of hematopoietic and lymphoid tissues. The present paper is a concise comparative review of the main primary cutaneous T-cell hematopoietic tumors, with emphasis on their immunohistochemical profiles.

Evaluation of the Cutaneous Lymphoma International Prognostic Index in patients with early stage mycosis fungoides

Abstract: Background: Mycosis fungoides (MF) is the most common subtype of cutaneous T-cell lymphoma. TNMB system is the staging method used in MF, and it not only guides therapeutic management, but represents the main prognostic factor. In order to improve the prognostic evaluation, the Cutaneous Lymphoma International Prognostic Index (CLIPi) was proposed. Objective: To evaluate the performance of CLIPi score for prognostic analysis in patients with early stage MF. Methods: This is a retrospective cross-sectional observational study, with exploratory analysis. The outcome variables were disease progression and related death. Results: One hundred and two patients were stratified according to CLIPi score, being the majority classified as low risk. Patients with intermediate or high risk presented disease progression more frequently than those with low risk (PR: 1.2 / p = 0.004 / 95%CI: 1.0 - 1.6). The same did not occur with the variable related death. In addition, survival rates were not consistent with risk stratification. Study Limitations: Small sample and its retrospective analysis. Conclusions: Since CLIPi score was proposed, four other studies that we could consult showed conflicting results, similar to the present study. Further studies are necessary for a recommendation of its use.

Epitheliotropic lymphoma with heart and kidney metastasis in a dog: case report / Linfoma epiteliotrópico em cão com metástase cardíaca e renal: relato de caso

A female adult dog, with a four-month history of pain and intense pruritus, which eventually resulted in sudden death, was referred for necropsy. Postmortem examination showed thoracic and abdominal serum-sanguineous exudates, multifocal infiltrative renal masses, and similar tumors in the heart. Histopathology revealed midsize infiltrative neoplastic proliferation composed of round cells, sparse cytoplasm, and large hyperchromatic nuclei. Immunohistochemistry revealed CD3+ and CD20-immunoexpression. Histopathological and immunohistochemical findings confirmed the diagnosis of epitheliotropic lymphoma with cardiac and renal metastasis.(AU)

Foi encaminhado para necropsia um cão adulto do sexo feminino, com histórico de dor e prurido intenso com evolução de quatro meses, que acabou resultando em morte súbita. O exame post mortem mostrou presença discreta de exsudato serossanguinolento em cavidades torácica e abdominal, massas renais infiltrativas multifocais e tumores semelhantes no coração. O exame histopatológico revelou proliferação neoplásica infiltrativa composta de células redondas, com citoplasma escasso, e grandes núcleos hipercromáticos. A análise imuno-histoquímica mostrou imunoexpressão CD3+e CD20. Os achados histopatológicos e imuno-histoquímico confirmaram o diagnóstico de linfoma epiteliotrópico com metástase cardíaca e renal.(AU)

Primary cutaneous T-cell lymphoma: experience from the Peruvian National Cancer Institute

Abstract: Background: Primary cutaneous T-cell lymphomas constitute a heterogeneous and rare group of diseases with regional particularities in Latin America. Objective: To determine the clinicopathological features, relative frequency and survival among patients from a Peruvian institution. Methods: Primary cutaneous T-cell lymphomas were defined based on the absence of extracutaneous disease at diagnosis. Classification was performed following the 2008 World Health Organization Classification of Neoplasms of the Hematopoietic and Lymphoid tissues. Risk groups were established according to the 2005 World Health Organization-EORTC classification for cutaneous lymphomas. Data of patients admitted between January 2008 and December 2012 were analyzed. Results: 74 patients were included. Mean age was 49.5 years. In order of frequency, diagnoses were: mycosis fungoides (40.5%), peripheral T-cell lymphoma not otherwise specified (22.95%), adult T-cell lymphoma/leukemia (18.9%), CD30+ lymphoproliferative disorders (6.8%), hydroa vacciniforme-like lymphoma (5.4%), extranodal NK/T-cell lymphoma (4.1%) and Sézary syndrome (1.4%). Predominant clinical patterns were observed across different entities. Mycosis fungoides appeared mainly as plaques (93%). Peripheral T-cell lymphoma not otherwise specified and adult T-cell lymphoma/leukemia presentation was polymorphic. All patients with hydroa vacciniforme-like lymphoma presented with facial edema. All cases of extranodal NK/T-cell lymphoma appeared as ulcerated nodules/tumors. Disseminated cutaneous involvement was found in 71.6% cases. Forty-six percent of patients were alive at 5 years. Five-year overall survival was 76.4% and 19.2%, for indolent and high-risk lymphomas, respectively (p<0.05). High risk group (HR: 4.6 [2.08-10.18]) and increased DHL level (HR: 3.2 [1.57-6.46]) emerged as prognostic factors for survival. Study limitations: Small series. Conclusion: Primary cutaneous T-cell lymphomas other than mycosis fungoides or CD30+ lymphoproliferative disorders are aggressive entities with a poor prognosis.

Granulomatous slack skin: a rare subtype of mycosis fungoides

Abstract: We report a case of granulomatous slack skin, a rare and indolent subtype of mycosis fungoides. It affects mainly men between the third and fourth decades. It is characterized by hardened and erithematous plaques that mainly affect flexural areas and become pedunculated after some years. Histological examination shows a dense infiltrate of small atypical lymphocytes involving the dermis (and sometimes the subcutaneous tissue) associated with histiocytic and multinucleated giant cells containing lymphocytes and elastic fibers (lymphophagocytosis and elastophagocytosis, respectively). Patients affected by this entity can develop secondary lymphomas. There are several but little effective therapeutic modalities described. Despite the indolent behavior of granulomatous slack skin, its early recognition and continuous monitoring by a dermatologist becomes essential for its management and prevention of an unfavorable outcome.

Proliferación linfoide indolente cutánea CD8 positiva a propósito de tres casos con compromiso facial / Indolent CD8-positive t-cell lymphoid proliferation: on purpose three cases of facial commitment

Resumen La proliferación linfoide indolente cutánea CD8 positiva es una variante recientemente descrita de linfoma T cutáneo que se caracteriza por un nódulo, pápula o placa eritematosa de crecimiento lento que puede afectar la región facial o extrafacial. En el estudio de patología se caracteriza por un infiltrado monomorfo de linfocitosTalo largo de la dermis con presencia de zona de Grenz y ausencia de epidermotropismo. El infiltrado es característicamente CD8+ así como CD3+, TIA-1+, CD4-, CD56- CD30-, PD-1-, Granzima B- y EBER negativo. El índice de proliferación Ki-67 es inferior al 10% y se observan reordenamientos clonales de los genes del receptor de antígeno de la célula T, TCR. El seguimiento clínico es favorable y no se ha observado compromiso sistémico. Se presentan tres casos con compromiso facial (dos casos en pabellón auricular y un caso con compromiso nasal), con presentación clínica y hallaz gos histopatológicos típicos (curiosamente un caso con cambio de célula clara), y además se realizaron estudios de clonalidad.

Abstract Primary cutaneous indolent CD8-positive lymphoid proliferation is a recent variant of cutaneous T lymphoma that is characterized by nodule, papule or plaque erythematous with slow growth that can affect the facial or extrafacial region. In the histopathology study it is characterized by an infiltration of monomorphic T lymphocytes throughout the dermis with presence of Grenz zone and absence of epidermotropism. The infiltrate is characteristically CD 8+ and CD3+ TIA-1+ CD4-, CD56- CD30, PD-1, Granzyme B- and negative EBER. Ki-67 Proliferación linfoide indolente cutánea CD8 positiva a propósito de tres casos proliferation index is less than 10% and clonal T-cell receptor gene rearrangements. Clinical follow-up is favorable and has not been observed systemic involvement. We present three cases with facial involvement (two cases in ear and one case with nasal commitment) with typical clinical presentation, histopathological findings (curiously a case with clear cell change) and clonality studies.

Micosis fungoide ampollar de presentación en palmas y plantas / Mycosis fungoide blister presentation in palms and plants

La micosis fungoide es el linfoma cutáneo primario de células-T más común y se caracteriza por presentar un amplio rango de variantes clínicas e histopatológicas [1]. La presentación ampollar de esta patología es muy rara y se han encontrado menos de una veintena de reportes en la literatura [2], por lo que se estima que la asociación con una presentación palmaris et plantaris sea aún menos común. A continuación, se presenta el caso clínico de una paciente de la ciudad de Quito-Ecuador quien cursaba con manifestaciones cutáneas de esta enfermedad sin afectación extracutánea.

Methotrexate and etanercept-induced primary cutaneous CD4 positive small/medium-sized pleomorphic T-cell lymphoma

Abstract: Immunosuppressive drugs and biological agents may represent a potential risk of lymphoma development in patients with rheumatoid arthritis. But most cases are diffuse, large B-cell lymphomas. Primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoma, a provisional entity in the 2005 WHO-EORTC classification of cutaneous lymphomas, is only described in a limited number of reports. To our knowledge, our case is a rare instance of primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoma, after associated treatment with methotrexate and etanercept, in a patient with moderate rheumatoid arthritis who had undergone an orchidectomy incorrectly.

Análise comparativa da expressão e atividade das metaloproteinases 2 e 9 e de seus inibidores teciduais nas lesões cutâneas das variantes poiquilodérmica e clássica da micose fungoide / A comparative analysis of the expression and activity of metalloproteinases 2 and 9 and their tissue inhibitors in cutaneous lesions of poikilodermatous and classic variants of mycosis fungoides

Micose fungoide poiquilodérmica (MFp) é uma variante clínica de micose fungoide (MF). É mais indolente e caracterizada pela presença da poiquilodermia. As metaloproteinases (MMP) e seus inibidores específicos TIMP (Tissue Inhibitors of Metaloproteinases) estão envolvidos na oncogênese. Especificamente as MMP2 e MMP9 e seus inibidores, TIMP-2 e TIMP-1, respectivamente, foram relacionados ao prognóstico em tumores. Poucos trabalhos estudaram MMP e nenhum estudou a ação dos TIMP na MF. Objetivos: avaliar a relação entre MMP2 e MMP9 e seus inibidores TIMP2 e TIMP1 e a agressividade da MF e descrever a casuística de micose fungoide poiquilodérmica no ambulatório de linfomas cutâneos da Divisão de Clínica Dermatológica do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo. Métodos: análise retrospectiva de 54 casos de MFp, sendo 25 de MFp localizada 14 de MFp generalizada e 15 de MFp mista. Para análise das MMP e TIMP, os grupos de MFp foram comparados com 7 amostras de pele normal (PN), 10 casos de MF clássica inicial (MFi), 9 casos de MF tumoral não-transformada (MFT nt) e 10 de MF tumoral transformada (MFT t). Resultados: A proporção de mulheres: homens foi 2,44. MFp apresentou maior tempo entre os primeiros sintomas e o diagnóstico. MFpG apresentou maior prevalência de lesões do tipo pitiríase liquenoide crônica (PLC) (79%). Houve alta prevalência de MF hipocromiante (62%) no grupo MFp mista. A histologia da MFp apresentou características típicas de MF e, adicionalmente, atrofia, telangectasias e derrame pigmentar, específicos da forma poiquilodérmica. Na imuno-histoquímica predominou o fenótipo CD3+, CD4+, CD7-, CD8- em todos os grupos, e MFp apresentou significantemente menor predomínio do fenótipo CD8+ que o grupo MFi. O grupo MFpG apresentou baixa positividade para pesquisa de clonalidade T da pele (12,5%). A MMP2 esteve mais presente na epiderme em MFi e MFp relativamente a MFT. Na derme superficial, os grupos MFi e MFp...

Poikilodermatous mycosis fungoides (pMF) is a clinical variant of mycosis fungoides (MF). It is more indolent than classic MF and is characterized by the presence of poikiloderma. The matrix metalloproteinases (MMPs) and their specific inhibitors TIMP (Tissue Inhibitors of Metalloproteinases) are involved in oncogenesis. Specifically, MMP2 and MMP9 and their inhibitors, TIMP-2 and TIMP-1, respectively, have been related to prognosis in tumors. There are few studies on MMP and none on the role of TIMPs in MF. Objectives: To evaluate if there is a relationship between the presence and activity of MMP2 and MMP9 and their inhibitors TIMP2 and TIMP1, and the aggressiveness of MF. To describe a casuistic of poikilodermatous mycosis fungoides in an outpatient clinic in the Dermatological Division of Hospital das Clinicas of University of Sao Paulo Medical School. Methods: Retrospective analysis of 54 cases of pMF, this included 25 localized pMF (LpMF), 14 generalized pMF (GpMF) and 15 mixed pMF. For the analysis of MMPs and TIMPs, the pMF groups were compared with 7 normal skin samples (NS), 10 cases of initial classical MF (cMF), 9 cases of non-transformed tumor MF (nt MFT) and 10 transformed tumor MF (t MFT). Results: The proportion of women : men was 2.44. The pMFs groups showed a longer period of time from the first symptoms to the diagnosis than the cMF group. The GpMF group had a higher incidence of pityriasis lichenoides chronica-like lesions (PLC) (79%) than the other groups. There was a high incidence of hypopigmented MF (62%) in the mixed pMF group. Histology showed typical characteristics of MF and, additionally, atrophy, telangiectasia and pigmentary alterations compatible with pMF. At immunohistochemistry the cases were predominantly CD3+, CD4+, CD7-, CD8- phenotype in all groups, and the pMF groups had a significantly lower prevalence of CD8+ phenotype than the cMF group. The GPMF group showed low positivity for clonality of the T-cell...

Micosis fungoide hiperpigmentada, una variante inusual, a propósito de un caso / Hyperpigmented mycosis fungoides, an unusual variant: a case report

La Micosis Fungoide (MF) hiperpigmentada es un subtipo de linfoma cutáneo de células T infrecuente, la cual podría presentar un curso más indolente y mejor pronóstico que a la MF clásica.Se reporta una caso de MF hiperpigmentada en un sujeto adulto.Paciente masculino de 60 años, con antecedentes de hipertensión arterial. Consulta por múltiples máculas pruriginosas, localizadas principalmente en tronco, de quince años de evolución. Al examen físico, paciente con fototipo de piel Fitzpatrick IV, se observan máculas y placas café oscuro, bien delimitadas en tronco y extremidades. Los estudios histopatológicos, inmunohistoquímicos (IHQ) y de clonalidad de linfocitos son concordantes con MF. El estudio de diseminación es negativo. El paciente es manejado fototerapia con UVB-nb con el diagnóstico de MF Hiperpigmentada. La MF hiperpigmentada afecta a sujetos de edad media y fototipos altos. Clínicamente se caracteriza por parches y placas hiperpigmentadas, localizadas en tronco y extremidades. En la histopatología (HP), además de los hallazgos de la MF clásica, se describen abundantes melanófagos en dermis superior y gránulos de melanina en queratinocitos. Los linfocitos T son de predominio CD8 (+) a diferancia de la MF clásica. Dentro de los diagnósticos diferenciales, se incluyen la hiperpigmentación postinflamatoria, eritema discrómico persistente, pigmentación macular eruptiva idiopática, entre otras. Se presenta este caso de MF hiperpigmentada por su baja frecuencia. Esta MF podría presentar un mejor pronóstico que a la MF clásica. El diagnóstico se realiza por sospecha clínica y se confirma con estudio HP e IHQ.

Hyperpigmented Mycosis Fungoides (MF) is a subtype of an uncommon cutaneous T cell lymphoma. It may have an indolent course and better prognosis than the classic MF. Methods: A case of an adult patient with hyperpigmented MF is reported Male patient of 60 years old, with hypertension history, presented with multiple itchy macules, located mainly on the trunk, for the last fifteen years. On physical examination, the patient has Fitzpatrick type IV skin phenotype; well defined dark brown macules and plaques, are observed on the trunk and extremities. Histopathology (HP), immunohistochemistry (IHC) and lymphocyte clonality studies are consistent with MF. The dissemination study is negative. The patient is treated with narrow-band ultraviolet B (UVB-nb) phototherapy. Hyperpigmented MF affects mid-aged adults and dark phototype skin. It is characterized by hyperpigmented patches and plaques, located on the trunk and extremities. In addition to the classical findings of MF, HP adds abundant melanophages in the upper dermis and melanin granules in keratinocytes. Unlike classical MF, T lymphocytes are mainly CD8 (+). Differential diagnoses include postinflamatory hyperpigmentation, erythema dyschromicum perstans, idiopathic eruptive macular pigmentation, and others. This case of hyperpigmented MF is presented for being infrequent. It may have a better prognosis than the classic MF. The diagnosis is made by clinical examination and confirmed with HP and IHC study.

Linfomas cutáneos primarios: frecuencia de los tipos histológicos en una serie de 500 pacientes / Primary cutaneous lymphomas frequency of histological types in a series of 500 patients

Objetivo: analizar las frecuencias de los tipos histopatológico de Linfomas Cutáneos Primarios (LCP) registrados por un grupo colaborativo multicéntrico (redlinfomacutaneo.org.ar). Metodología: analizamos 500 casos provenientes de 24 centros dermatológicos (públicos y privados) de Argentina y uno de Colombia, reportados entre 2010 y 2015. Se incluyeron únicamente casos histológicamente confirmados y estadificados. La información registrada cumple con la Declaración de Helsinki. Resultados: el 94,2% fueron LCP de células T (LCCT) distribuidos en: Micosis fungoide (MF), 75,4%; Desórdenes Linfoproliferativos CD30+, 5,8%; variantes de MF, 4,6%; Síndrome de Sezary, 2,6%; Linfomas T Periféricos tipo NOS, 1,0%; Linfomas de células T-NK Extranodal tipo nasal, 1,0%; Linfomas CD8+ Epidermotropo Agresivo, 1,0%; Linfomas T Pleomórfico CD4+, 0,2%; Leucemia Linfoma T del Adulto, 0,4%. Los LCP de células B (LCCB) fueron el 5,8% y se distribuyeron en: Linfomas Centrofoliculares, 2.4%; Linfomas Marginales, 1,8%; Linfomas B difusos de Células Grandes tipo pierna, 0,4% y tipo NOS, 1%. Conclusiones: confirmamos el predominio de los LCCT pero con una frecuencia de LCCT superior y de LCCB inferior a las reportadas en series europeas o de EE.UU y similar a las de países asiáticos pudiendo obedecer a un sesgo del grupo aportante que sub-registra los LCCB o a factores etiológicos y/o étnicos. (AU)

Aims: to analize the frequency of histopathological types in Primary Cutaneous Lymphoma (PCL) recorded by a multicenter collaborative group (redlinfomacutaneo.org.ar). Methodology: we analized 500 cases from 24 dermatological centers (public and private) of Argentina and one of Colombia, reported between 2010 and 2015 and only being included histological confirmed and staged cases. Recorded information complies with the Declaration of Helsinki. Results: 94,2% were PCL of T cells (CTCL) distributed as follow: Mycosis Fungoides (MF), 75,4%; CD30+ Lymphoproliferative Disorders, 5,8%; MF variants, 4,6%; Sezary Syndrome, 2,6%; Peripheral T cells Lymphoma unspecified, 1,%; Extranodal NK/T cell Lymphoma nasal type, 1,%; CD8+ aggressive epidermotropic lymphoma 1,0%; CD4+ pleomorphic lymphoma, 0,2%; Adult T-cell leukemia/lymphoma, 0, 4%. The PCL B cell (LCCB) were 5.8% and distributed into: follicle center lymphoma, 2.4%; marginal zone lymphoma, 1,8%; diffuse large B-cell lymphoma, leg type, 0,4% and others type, 1%. Conclusions: we confirmed the prevalence of CTCL but with a higher frequency of CTCL and lower of LCCB to those reported in European or US series and similar to those of Asian countries, may be due to a bias of the contributor who underreport LCCB or to etiological and / or ethnic factors. (AU)

Granulomatous slack skin T-cell lymphoma: an important differential diagnosis with giant cell tumor of soft tissue

Abstract: Granulomatous slack skin is an indolent T-cell lymphoma, considered to be a variant of mycosis fungoides. Clinically it is characterized by areas of redundant skin, wrinkled, inelastic, with variable erythema and infiltration besides a poikilodermic surface. A differential diagnosis unknown to most dermatologists is the giant cell tumor of soft tissue, which is an extremely rare low-grade sarcoma. The authors report a patient who had undergone extensive surgery because of a primary diagnosis of giant cell tumor of soft tissue, but which proved to be granulomatous slack skin after a second interventional procedure with confirmatory histopathology.

Un caso de nódulos cutáneos múltiples: linfoma cutáneo de células T / A case of multiple skin nodules: cutaneous T-cell lymphoma

La micosis fungoide es la variante más común de linfoma cutáneo de células T y se caracteriza por un curso asintomático con evolución posterior de placas y tumores. A medida que la enfermedad progresa se observa mayor compromiso cutáneo y diseminación a ganglios linfáticos, bazo, pulmones e hígado...

Epitheliotropic cutaneous T-cell lymphoma associated with melanoma in a dog: case report / Linfoma cutâneo epiteliotrópico de células T associado a melanoma em cão: relato de caso

Several types of tumors affect dogs' skin. Simultaneously occurring neoplasms with different histological patterns might be rarely present in the same animal. This paper describes the occurrence of epitheliotropic cutaneous T-cell lymphoma and melanoma in a dog. The animal had nodular lesions in the abdominal region and serpiginous plaques on the dorsal region of the trunk. Cytology evidenced malignant fusiform cells from the abdominal lesions as well as few round cells from the dorsal. The histopathological examination of the abdominal lesions showed dermis with polygonal to spindle-shaped neoplastic cells. The lesion of the dorsal region evidenced neoplastic round cells with generally distinct cell borders and a moderate amount of eosinophilic cytoplasm. Abdominal lesions were positive for Melan A. Dorsal and forelimb lesions were positive for CD3. This study reports the occurrence of epitheliotropic cutaneous T-cell lymphoma and malignant melanoma in a crossbred Boxer dog and discusses the importance of performing immunohistochemical profile to confirm the phenotype of the tumor.

Diferentes tipos de tumores podem ocorrer na pele de cães. É rara, porém, a ocorrência simultânea de neoplasias com origens histológicas diferentes no mesmo animal. Este trabalho descreve a ocorrência de linfoma cutâneo epiteliotrópico de células T e melanoma em um cão. O animal apresentava lesões nodulares na região abdominal e placas serpiginosas na região dorsal do tronco e membros. A citologia evidenciou células fusiformes malignas das lesões abdominais, bem como algumas células redondas nas dorsais. O exame histopatológico das lesões abdominais mostrou derme com células neoplásicas poligonais a fusiformes. A lesão da região dorsal evidenciou células redondas neoplásicas com citoplasma eosinofílico. Lesões abdominais foram positivas para Melan A. Lesões dorsais e de membros anteriores foram positivas para CD3. Este estudo relata a ocorrência de linfoma cutâneo de células T epitheliotropic e melanoma maligno em um cachorro Boxer, e discute a importância da realização de perfil imuno-histoquímico para confirmar o fenótipo do tumor. A importância do perfil imuno-histoquímico para confirmar o tipo de neoplasia também é discutida.