Hydroa vacciniforme-like lymphoproliferative disorder in Ecuadorian children: A case series

We report the clinical and histopathological features of hydroa vacciniforme-like lymphoproliferative disorder in five indigenous and Mestizo children. All the children resided at higher altitudes, experiencing maximal solar exposure. All cases presented with prurigo along with Epstein-Barr virus infection. Histopathologic examination showed an atypical, CD30 + lymphocytic infiltrate with angiocentricity in all, while three cases demonstrated panniculitis-like infiltrate

Clinical Features and Prognostic Factors of Patients with Primary Cutaneous Lymphoma / 中国实验血液学杂志

OBJECTIVE@#To retrospectively analyze the clinical characteristics and prognostic factors of patients with primary cutaneous lymphoma.@*METHODS@#The clinical data of 22 patients with primary cutaneous lymphoma admitted to Xinjiang Hotan District People's Hospital, Heji Hospital affiliated to Changzhi Medical College and the Fifth Medical Center of PLA General Hospital from January 2013 to June 2021 were retrospectively analyzed.@*RESULTS@#The incidence of primary cutaneous T cell and NK/T cell lymphoma was about 91.9/100 000, and the incidence of primary cutaneous B cell lymphoma was about 14.5/100 000. The overall survival (OS) of patients aged ≥65 years was significantly shorter than that of patients younger than 65 years (P <0.05). Patients with elevated β2-microglobulin (β2-MG) had shorter OS and progression-free survival (PFS) (both P <0.05). Patients who achieved complete/partial response after initial treatment had longer OS than those with stable or progressive disease (P <0.05). There were significant differences in OS and PFS among patients with different pathological types of primary cutaneous lymphoma that originated from T and NK/T cells, the OS and PFS of patients with mycosis fungoides were longer than those of patients with other pathological types (both P <0.05). In addition, disease stage might also affect the PFS of the patients (P =0.056).@*CONCLUSION@#The age, disease stage, β2-MG level, pathological type and remission state after treatment of the patients were related to the clinical prognosis.

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.

Linfoma cutâneo primário difuso de grandes células B, tipo perna ­ rápida evolução e desfecho desfavorável: relato de caso / Diffused primary cutaneous lymphoma of large B cells, leg type - quick evolution and unfavorable outcome: case report

Linfomas cutâneos primários são a segunda forma mais comum de linfomas extranodais, sendo os linfomas de células B, (CBCLs) representantes de 20 a 30% dos casos. O linfoma cutâneo difuso de grandes células B, Tipo Perna (PCDLBCL-LT), representa o tipo mais agressivo de CBCLs. Na maioria dos casos, a apresentação clínica é caracterizada por placas ou tumores solitários, ora ulcerados, em uma ou ambas as pernas, de rápido crescimento. O diagnóstico é confirmado através do estudo histopatológico e imunohistoquímico. O tratamento é realizado por meio de quimioterapia e seu prognóstico é reservado com uma sobrevida de 50% a 60% em 05 anos. O objetivo deste trabalho é relatar um caso atendido de linfoma cutâneo primário difuso de grandes células B, tipo perna em um paciente de 75 anos, do sexo masculino com apresentação clínica clássica e desfecho desfavorável, realizar uma revisão bibliográfica do período de 2010 a 2020 na base de dados PUBMED sobre o assunto, dada sua raridade e agressividade ímpar. As informações foram obtidas através de revisão do prontuário, registro fotográfico e revisão da literatura. Por tudo isso, pode-se concluir a importância de estudos multidisciplinares, envolvendo dermatologistas, hematologistas, oncologistas e patologistas para que o diagnóstico e tratamento sejam instituídos o mais precoce possível, visto a raridade e agressividade do PCDLBCL-LT. [au]

Primary cutaneous lymphomas are the second most common form of extranodal lymphomas; with B cell lymphomas (CBCLs) representing 20 to 30% of cases. Diffuse cutaneous large B cell lymphoma, leg type (PCDLBCL-LT), represents the most aggressive type of CBCLs. In most cases, the clinical presentation is characterized by solitary plaques or tumors, sometimes ulcerated, on one or both legs, of rapid growth. The diagnosis is confirmed through histopathological and immunohistochemistry studies. Treatment is carried out through chemotherapy and its prognosis is reserved with a 50% to 60% survival in 5 years. The objective of this work is to report a case of diffuse primary B-cell cutaneous lymphoma, leg type in a 75-year-old male patient with a classic clinical presentation and unfavorable outcome, to perform a literary review from 2010 to 2020 in the PUBMED database on the subject, given its rarity and unique aggressiveness. The data was obtained by reviewing the medical record, photographic record and literature review. For all this, it is possible to conclude the importance of multidisciplinary studies, involving dermatologists, hematologists, oncologists and pathologists so that the diagnosis and treatment are instituted as early as possible, given the rarity and aggressiveness of the PCDLBCL-LT. [au]

Linfoma primario cutáneo de células T epidermotropo agresivo CD8+ / Primary cutaneous aggressive epidermotropic CD8 + T-cell lymphoma

El linfoma de células T epidermotropo agresivo CD8+ o linfoma de Berti es un subtipo infrecuente de los linfomas primarios cutáneos, descripto en 1999. Aún se considera una entidad provisional en la última clasificación de linfomas cutáneos primarios de la Organización Mundial de la Salud. Predomina en los hombres adultos y se manifiesta con pápulas, placas hiperqueratósicas y tumores ulcerados, diseminados y de inicio agudo. En la histopatología, es característica la presencia de un infiltrado de linfocitos atípicos medianos CD8 +, con epidermotropismo de patrón pagetoide. Tiene un comportamiento agresivo y es de mal pronóstico, con una sobrevida a los 5 años menor del 40%. Si bien esta entidad es un reto terapéutico, el tratamiento inicial se basa en poliquimioterapia y/o trasplante de células hematopoyéticas.

The aggressive epidermotropic CD8+ T-cell lymphoma or Berti's lymphoma, is a rare subtype of primary cutaneous lymphomas, first described in 1999. It is still considered a provisional entity by the latest World Health Organization classification of cutaneous lymphomas. Patients are commonly adults with a male predominance and it is characterized by widespread rapid evolving papules, hyperkeratotic annular plaques and ulcerated tumors. Histopathologicfindingsarecharacteristic,withaninfiltrate of medium-sized CD8+ atypical lymphocytes involving the epidermis with a pagetoid pattern. Ithasanaggressivebehaviorandtheprognosisispoor with a 5-yearsurvival less than 40%. Instead these disease represents a therapeutic challenge, the initial treatment consists on polychemotherapy and hematopoietic stem cell transplantation.

Variedades clínicas de micosis fungoide en una cohorte / Clinical variants of mycosis fungoides in a cohort

Resumen Introducción: La micosis fungoide es el linfoma primario de células T en piel más frecuente, con expresividad clínica heterogénea. Objetivo: Reportar las variedades clínicas y las características sociodemográficas de pacientes con micosis fungoide tratados en un hospital dermatológico. Métodos: Se incluyeron 290 pacientes con diagnóstico clínico e histopatológico de micosis fungoide atendidos en el transcurso de 11 años. Se realizó descripción sociodemográfica de los pacientes, quienes se clasificaron conforme las variantes clínicas e histopatológicas. Resultados: 58 % de los casos de micosis fungoide se presentó en mujeres y 42 % en hombres. La variedad clínica más común fue la clásica en 46.2 %; la discrómica representó 35.2 %, del cual la hipopigmentada fue la más representativa (7.6 %); la poiquilodérmica constituyó 4.1 % y la foliculotrópica, 3.1 %. La variedad papular se presentó en seis pacientes (2.1 %), la de placa única en tres (1 %) y la ictiosiforme, siringotrópica y la piel laxa granulomatosa, en un paciente cada una. La variedad granulomatosa se encontró en 0.7 % y 1.4 % presentó eritrodermia. Conclusiones: La variedad clínica más frecuente de micosis fungoide fue la clásica en fase de placa, seguida de las variedades discrómicas. Otras variedades clínicas representaron 18.6 %.

Abstract Introduction: Mycosis fungoides (MF) is the most common primary skin T-cell lymphoma, which is characterized for a heterogeneous clinical expressivity. Objective: To report clinical variants and sociodemographic characteristics in patients with MF under the care of a dermatological hospital. Methods: 290 patients with MF clinical and histopathological diagnosis attended to over the course of 11 years were included. Sociodemographic description of patients was made, who were classified according to clinical and histopathological variants. Results: MF was recorded in 57.9 % of women and 42 % of men. The most common clinical variant was the classic type in 46.2 %; dyschromic variants accounted for 35.2 %, out of which hypopigmented MF was the most representative (17.6 %); poikilodermatous MF accounted for 4.1 %, and folliculotropic, for 3.1%. The papular variant occurred in six patients (2.1 %), the single-plaque variety in three (1%), and the ichthyosiform, syringotropic and granulomatous slack skin varieties occurred in one patient each. The granulomatous variant was found in 0.7 %, and 1.4 % had erythroderma. Conclusions: The most common MF clinical variant was classic plaque stage, followed by dyschromic variants. Other clinical variants accounted for 18.6 %.

Linfoma anaplásico de células T grandes primario cutáneo CD30+. Serie de nueve casos / Primary cutaneous CD30+ anaplastic large T-cell lymphoma. Series of nine cases

Resumen Introducción: El linfoma anaplásico de células T grandes CD30+ es un linfoma primario cutáneo en el cual no hay evidencia de enfermedad sistémica; para su diagnóstico es necesario el estudio histopatológico. Objetivo: Presentar los casos diagnosticados en el Departamento de Dermatología del Hospital General "Dr. Manuel Gea González" con linfomas anaplásicos de células T grandes primarios cutáneos CD30+ durante un periodo de 24 años. Método: Estudio retrospectivo en el que realizó estadística descriptiva. Se recopiló información de sexo, edad, características clínicas, resultados de pruebas complementarias, tratamientos previos y actuales, reportes de los estudios histopatológicos y de inmunohistoquímica. Resultados: Entre 29 309 expedientes, se encontraron nueve casos (0.000034 %) con diagnóstico de linfoma anaplásico de células T CD30+. Se hizo la confirmación del diagnóstico histopatológico e inmunohistoquímico por dos dermatopatólogos certificados. La edad promedio fue de 61.2 años, hubo predominio del sexo femenino y de lesión papular o nodular y topografía variada como presentación clínica inicial. Conclusiones: El pronóstico del linfoma anaplásico de células T grandes CD30+ en la población estudiada fue dependiente del estadio clínico. El tratamiento en etapas tempranas tiene resultados favorables.

Abstract Introduction: CD30+ anaplastic large T cell lymphoma is a cutaneous primary lymphoma in which there is no evidence of systemic disease; histopathological study is required for its diagnosis. Objective: To present the cases diagnosed with primary cutaneous CD30+ anaplastic large T-cell lymphoma over a 24-year period in Hospital General "Dr. Manuel Gea González" Department of Dermatology. Method: Retrospective study. Descriptive statistics was carried out. Information was collected on gender, age, clinical characteristics, complementary test results, previous and current treatments, histopathological studies reports and immunohistochemistry test results. Results: Of 29 309 records, nine patients (0.000034%) with a diagnosis of CD30+ anaplastic T cell lymphoma were found. Histopathological and immunohistochemical diagnosis was confirmed by two certified dermatopathologists. Average age was 61.2 years, and there was a predominance of the female gender, with initial clinical presentation as a papular or nodular lesion and varied topography. Conclusions: The prognosis of CD30+ anaplastic large T cell lymphoma in the studied population was dependent on clinical stage. The treatment at early stages has favorable results.

A Case of Multifocal Primary Cutaneous Anaplastic Large Cell Lymphoma Managed without Surgical Treatment / 대한두경부종양학회지

Primary cutaneous anaplastic large cell lymphoma (C-ALCL) is rare among skin malignancies. C-ALCL usually manifests as reddish or violet nodules. Surgical excision or radiation therapy is generally considered as first-line therapy, but a clinically aggressive disease may require multiagent chemotherapy. Establishing a proper diagnosis of C-ALCL is challenging but should be made to avoid inappropriate treatment and its consequences. The authors report a case of medically resolved C-ALCL in an 81-year-old man presented with well-defined nodular lesions on the forehead.

Primary Cutaneous Intravascular Large B-cell Lymphoma / 대한피부과학회지

No abstract available.

Case of Secondary Cutaneous Peripheral T Cell Lymphoma, NOS / 대한피부과학회지

No abstract available.

Composite Cutaneous Lymphoma (Iatrogenic Immunodeficiency-Associated Lymphoproliferative Disorder) in a Patient with Rheumatoid Arthritis Treated with Methotrexate: Staging and Evaluation of Response to Therapy with ¹⁸F-FDG PET/CT / 대한핵의학회잡지

A 67 year old woman with a 10 year history of rheumatoid arthritis (RA) treated with methotrexate and prednisone, presented with a 2 year history of worsening multiple cutaneous plaques of variable appearance. Two distinct skin lesions were biopsied to reveal a composite cutaneous lymphoma, possibly caused by long term methotrexate therapy. An [18F] fluoro-2-deoxy-D-glucose (¹⁸F-FDG) positron emission tomography/computed tomography (PET/CT) was performed to stage the malignancy, and was later repeated to evaluate response to chemotherapy, which guided subsequent management. We present the PET/CT imaging findings of this very rare iatrogenic (methotrexate induced) immunodeficiency-associated lymphoproliferative disorder.

A Case of Primary Cutaneous Anaplastic Large Cell Lymphoma on Palm / 대한피부과학회지

Primary cutaneous anaplastic large cell lymphoma (PCALCL) is a rare primary cutaneous lymphoma that is predominantly composed of large lymphoid cells that express the CD30 antigen. The skin lesion of PCALCL is usually single, ulcerative, and located on the trunk or extremities and rarely the palm. A 25-year-old woman presented with a plaque on the left palm for 20 days. The plaque was walnut-sized and purple to gray colored with erosion in the center. Histopathologic examination showed infiltration of large atypical cells in the dermis. The large tumor cells showed positivity for CD3, CD4, and CD30 and negativity for CD8, CD20, epithelial membrane antigen, and anaplastic lymphoma kinase. PET-CT showed no other hypermetabolic lesion except that on the left palm, and we finally arrived at a diagnosis of PCALCL. The patient was treated with an intralesional injection of methotrexate (25 mg/mL, 0.45 cc). After 3 months of treatment, the walnut-sized plaque had disappeared and a peripheral hyperpigmented patch remained.

Type D lymphomatoid papulosis simulating aggressive epidermotropic cytotoxic lymphoma.

Three histological subtypes of lymphomatoid papulosis (LyP), type A (histiocytic), type B (mycosis fungoides like) and type C (anaplastic large cell lymphoma like) are well recognized. Two new histological variants, type D (simulating an aggressive epidermotropic cytotoxic lymphoma) and type E (angioinvasive type) has been described recently. We describe a 27‑year‑old man presented with a history of asymptomatic erythematous papules on both upper and lower limbs noted since 10 years of age. There were no systemic symptoms. Biopsy revealed an atypical dermal lymphoid infiltrate with epidermotropism, and the immunohistochemical markers showed a diffuse positivity for CD3, CD8, CD56, T1A and granzyme B with the focal positivity of CD30. All other relevant tests were normal. In this case report of a recently described delineated variant of LyP we emphasize the indolent course of this entity although the histology would suggest a more aggressive disease.

Clinical analysis of 14 cases of rare non-Hodgkin lymphoma in children / 中华实用儿科临床杂志

Objective To analyze the clinical characteristics and prognosis of 4 rare types of non -Hodgkin lymphoma(NHL)in children,and to discuss the progress in treatment.Methods Clinical data of 1 4 patients with rare types of NHL at Shanghai Children′s Medical Center,Shanghai Jiaotong University School of Medicine between January 2004 and December 201 4 were retrospectively analyzed,and their clinical features,treatment and prognosis were dis-cussed.Results Fourteen cases were reported including 6 subcutaneous panniculitis -like T -cell lymphoma (SPTCL),3 hydroa vacciniforme -like cutaneous lymphoma(HVLL),2 pediatric follicular lymphoma(PFL)and 3 ex-tranodal NK/T -cell lymphoma,and nasal type(ENKTL).Ten patients (71 .4%)primarily presented with skin lesions and underwent a long course of illness before they were finally diagnosed (the median was 1 0 months),71 .4%(1 0 /1 4 cases)of them associated with fever and 50.0%(7 /1 4 cases)with liver and spleen enlargement,and no evidence of central nervous system (CNS)and bone marrow (BM)involvement was observed,while 28.6% patients (4 /1 4 cases) had more than two lines′abnormalities in peripheral blood examination.Since there were no standard treatment guide-lines,most patients received CHOP (Cyclophosphamide +Adriamycin +Vincristine +Prednisone)and /or mature B -cell NHL -like therapy,and 50.0%(7 /1 4 cases)of them received interferon therapy in addition,while 1 patient re-ceived allogeneic hematopoietic stem cell transplantation after recurrence.The complete remission was achieved in 71 .4%(10 /14 cases)of all the patients.Two of them died,3 lost follow -up,and 1 relapsed.The 3 -year overall survi-val and event free survival rates were 0.84 and 0.57,respectively,after a median follow -up of 26 months (range 12 -64 months).Conclusions Pediatric rare types of NHL show atypical clinical manifestation,low incidence of CNS /BMinfil-tration and long course.It is hard to make pathological diagnosis and differentiation.It is also inappropriate to apply the commonly used staging system to these rare types of NHL.No standard treatment has been found by now.SPTCL,HVLL and PFL have relatively good outcomes when treated with mature B -cell NHL -type therapy plus interferon therapy.

Clinical and laboratorial evaluation of dogs with cutaneous lymphoma treated with lomustine / Avaliação clínica e laboratorial de cães com linfoma cutâneo tratados com lomustina

The aim of this prospective study was to evaluate the clinical response of dogs with cutaneous lymphoma treated with lomustine (CCNU) and to identify possible adverse effects and toxicity during treatment. Fifteen dogs, seven females and eight males aged between five and 17 years old, diagnosed with cutaneous lymphoma by histopathological analysis were selected and treated with lomustine at 90 mg/m² every three weeks. Monitoring was carried out and consisted of the assessment of laboratory hematology and serum chemistry before and during treatment. Partial response was observed in 53.3% of the animals. None of the animals achieved a complete response and seven dogs (46.6%) had progressive disease. The median survival time was 22 days. The major hematological and biochemical changes found after therapy were leukopenia (73.3%), thrombocytopenia (60%) and anemia (46.1%). Renal and liver toxicity was observed in 40% and 73.3% of dogs, respectively. Hematocrit, total protein, leukocyte count, neutrophil count, serum creatinine, ALT, GGT, alkaline phosphatase and urine specific gravity were affected during therapy. The use of lomustine as a monotherapy in the treatment of canine cutaneous lymphoma was effective; however, adverse effects occurred and compromised the quality of life of the majority of dogs in this study. Therefore, lower doses of lomustine should be considered in future studies...

O objetivo deste estudo prospectivo foi avaliar a resposta clínica de cães com linfoma cutâneo tratados com lomustina (CCNU) e identificar possíveis efeitos adversos e toxicidade durante o tratamento. Quinze cães, sendo 7 fêmeas e 8 machos, com idades entre 5 e 17 anos diagnosticados com linfoma cutâneo por avaliação histopatológica foram selecionados e tratados com lomustina na dose de 90 mg/m2 a cada três semanas. Os cães foram monitorados por avaliação hematológica e bioquímica sérica antes e durante o tratamento. A resposta parcial foi observada em 53,3% dos animais. Nenhum dos animais apresentou resposta completa e sete animais (46,6%) apresentaram progressão da doença. O tempo médio de sobrevida foi de 22 dias. As principais alterações hematológicas e bioquímicas observadas após o tratamento foram leucopenia (73,3%), trombocitopenia (60%) e anemia (46,1%). Sinais de toxicidade renal e hepática foram observados em 40% e 73,3% dos cães, respectivamente. Durante o tratamento foram afetados os parâmetros hematócrito, proteínas séricas totais, contagem de leucócitos, contagem de neutrófilos, creatinina sérica, ALT, GGT, fosfatase alcalina e densidade urinária. O uso de lomustina como monoterapia no tratamento do linfoma cutâneo canino foi efetivo; entretanto, efeitos adversos ocorreram e comprometeram a qualidade de vida da maioria dos animais neste estudo. Assim, sugere-se que doses mais baixas de lomustina sejam consideradas em estudos futuros...

Linfoma cutáneo primario de células B centrofolicular: un desafío diagnóstico / Primary cutaneous follicular B-cell lymphoma: a diagnostic challenge

El linfoma cutáneo primario de células B (LCPCB) centrofolicular corresponde a una proliferación neoplásica infrecuente e indolente de células del centro germinal confinadas a la piel. Se reporta y revisa un caso de LCPCB centrofolicular. Paciente femenino de 76 años, con antecedentes de hipertensión arterial y resistencia a la insulina. Consultó por aumento de volumen frontal de dos años de evolución. Al examen físico destacaba un nódulo único en región frontal derecha. La ecografía de partes blandas fue compatible con quiste epidérmico. La histopatología demostró en dermis profunda, tejido adiposo subcutáneo y tejido muscular estriado una proliferación linfoide sólida dispuesta en patrón nodular y difuso. A la inmunohistoquímica (IHQ), los linfocitos fueron CD20 y BCL-6 positivo, con un Ki-67 de 60% y BCL-2, CD3, CD5 y CD10 negativo. El estudio de diseminación tumoral resultó negativo. El LCPCB centrofolicular concentra el 60% de todos los LCPCB. Se presenta en promedio a los 51 años. Se ha descrito asociación con Borrelia burgdorferi, VIH, virus hepatitis C y virus Epstein-Barr. Clínicamente corresponde a un nódulo eritematoso y asintomático, localizado preferentemente en cabeza, cuello y tronco. La IHQ es fundamental para diferenciarlo de otros tipos de LCPCB. Habitualmente, el tratamiento es con radioterapia o cirugía escisional. La supervivencia es de un 95% a 5 años. Se presenta este caso dado que el LCPCB centrofolicular corresponde a un tumor cutáneo infrecuente, con múltiples diagnósticos diferenciales, que requiere de un alto índice de sospecha para lograr un diagnóstico y tratamiento oportuno.

The primary cutaneous follicle center lymphoma (PCFCL) corresponds to an infrequent and indolent neoplastic proliferation of germinal center cells confined to the skin. A case of PCFCL is reported and revised. Results: A female patient, 76 years old, with arterial hypertension and insulin resistance. Sough attention for an increase in size of the frontal region over the course of two years. Upon physical examination, a single nodule was noted in the right frontal region. A soft tissue ultrasound identified results indicative of an epidermal cyst. The histopathology revealed a proliferation of solid lymphoid arrayed in a diffuse and nodular pattern in the deep dermis, subcutaneous adipose tissue, and striated muscular tissue. Immunohistochemistry (IHQ) revealed CD20 and BCL-6 positive lymphocytes, with 60% of Ki-67 and BCL-2, CD3, CD5, and CD10 negative. A study of tumor dissemination resulted negative. The PCFCL concentrates 60% of all primary cutaneous B cell lymphomas (PCBCL). It presents at an average of 51 years of age. It has been described association with Borrelia burgdorferi, HIV, hepatitis C virus and Epstein-Barr virus. Clinically it corresponds to an erythematous and asymptomatic nodule, found frequently on the head, neck and trunk. The IHQ is essential to differentiate it from other types of PCBCL. It is usually treated with radiotherapy or excisional surgery. Survival is 95% over 5 years. This case is presented because the PCFCL corresponds to an infrequent cutaneous tumor, with multiple differential diagnoses, requiring a high index of suspicion to achieve an opportune diagnostic and treatment.

Primary Cutaneous T Cell Lymphoma (Gamma Delta subtype)

Primary cutaneous T-cell lymphoma gamma-delta subtype is an extremely rare entity of all the cutaneous T-cell lymphomas. Our case provides an insight on clinical behavior and treatment response with feasible effective combination chemotherapy. We believe this will be of great interest to clinicians when facing this difficult clinical entity. We present a case of a 66-year-old Malay man with a threeweek history of rapidly growing skin nodules and plaques which spread throughout his body. He was commenced on combination chemotherapy gemcitabine, etoposide, and carboplatin with near complete remission on completion of second cycle but he defaulted. He relapsed within a month and he progressed despite treatment with the same regime. He was salvaged with fludarabine, cytarabine, and vinblastine combination chemotherapy but progressed with brain metastasis and died. However, more investigations and studies need to be done in this relatively unknown rare entity. A rare lymphoma registry might be of help to better understand and treat similar conditions.

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)

Neoplasia blástica de células dendríticas plasmocitoides: reporte de un caso y revisión de la literatura / Plasmacytoid dendritic cell blast neoplasia: case report and literature review

La neoplasia de células blásticas plasmocitoides dendríticas esun linfoma cutáneo poco frecuente y de mal pronóstico, que característicamenteexpresa antígenos CD4 y CD56. Clínicamentepresenta placas o nódulos de coloración violácea, únicos o múltiples.El diagnóstico se confirma con el estudio anatomopatológicoque evidencia células linfoblástica y fenotipo CD4+ y CD56+, conausencia de marcadores para células mieloides, linfoides B y T ycélulas NK. Presentamos el reporte de un caso de un paciente desexo masculino de 65 años de edad y una revisión de la literatura.

Blastic plasmacytoid dendritic cell neoplasm is a rare andhighly aggressive cutaneous lymphoma that characteristicallyexpress CD4 and CD56 antigens. Clinically presents violet colouredplaques or nodules, in a unique or multiple presentation.The diagnosis is confirmed with the anatomophatologic studythat evidence linfoblastic cells with CD4+ and CD56+ phenotype,and no mieloids, linfoids B and T and natural killers cellsmarkers. We present a case report of a 65-years-old male anda literature review.

Characteristics of Cutaneous Lymphomas in Korea According to the New WHO-EORTC Classification: Report of a Nationwide Study

BACKGROUND: Previously, cutaneous lymphomas were classified according to either the European Organization for the Research and Treatment of Cancer (EORTC) or the World Health Organization (WHO) classification paradigms. The aim of this study was to determine the relative frequency of Korean cutaneous lymphoma according to the new WHO-EORTC classification system. METHODS: A total of 517 patients were recruited during a recent 5 year-period (2006-2010) from 21 institutes and classified according to the WHO-EORTC criteria. RESULTS: The patients included 298 males and 219 females, and the mean age at diagnosis was 49 years. The lesions preferentially affected the trunk area (40.2%). The most frequent subtypes in order of decreasing prevalence were mycosis fungoides (22.2%), peripheral T-cell lymphoma (17.2%), CD30+ T-cell lymphoproliferative disorder (13.7%), and extranodal natural killer/T (NK/T) cell lymphoma, nasal type (12.0%). Diffuse large B-cell lymphoma accounted for 11.2% of cases, half of which were secondary cutaneous involvement; other types of B-cell lymphoma accounted for less than 1% of cases. CONCLUSIONS: In comparison with data from Western countries, this study revealed relatively lower rates of mycosis fungoides and B-cell lymphoma in Korean patients, as well as higher rates of subcutaneous panniculitis-like T-cell lymphoma and NK/T cell lymphoma.