SMILE en linfoma de células NK/T extranodal en recaída / SMILE in relapsed extranodal NK / T cell lymphoma

Introducción: El linfoma extranodal de células NK/T, tipo nasal es una variedad agresiva que se presenta con mayor frecuencia en Asia Oriental y América Latina. El régimen de tratamiento SMILE, basado en dexametasona, metotrexato, ifosfamida, L-asparaginasa y etopósido, mostró tasas de respuestas objetivas adecuadas en estudios de fase 1/2. Objetivo: presentar el primer reporte de la aplicación del protocolo SMILE en Cuba. Presentación del caso: paciente de 47 años de edad, blanca que presentó recaída temprana de linfoma extranodal de células NK/T tipo nasal en partes blandas de la pared anterior izquierda del tórax. Recibió quimioterapia SMILE por 4 ciclos y en la evaluación al final del tratamiento se comprobó que hubo una respuesta completa de la enfermedad(AU)

Introduction: Extranodal lymphoma of NK / T cells, nasal type is an aggressive variety that occurs most frequently in East Asia and Latin America. The SMILE treatment regimen, based on dexamethasone, methotrexate, ifosfamide, L-asparaginase and etoposide, showed adequate objective response rates in phase 1/2 studies. Objective: to present the first report of the application of the SMILE protocol in Cuba. Case presentation : 47-year-old white patient who presented early relapse of extranodal lymphoma of nasal NK / T cells in soft parts of the left anterior wall of the thorax. He received SMILE chemotherapy for 4 cycles and the evaluation at the end of the treatment showed that there was a complete response of the disease(AU)

A Case of Angiocentric T-cell Lymphoma / 대한피부과학회지

A 40-year-old female patient, known angiocenteric T-cell lymphoma on nasal cavity, was transfered from the department of hemato-oncology for the evaluation of egg sized pinkish-yellow indurated mass with central necrotic tissue on the left medial elbow. Histopathologic findings revealed angiocentric, angioinvasive, and angiodestructive infiltrate containing atypical lymphocytes. The infiltrative cells were positivly stained with antibody to CD45RO, LCA, but not with antibody CD20. The atypical infiltrated perivascular lymphoid cells were positive to Epstein-Barr virus in situ polymerase chain reaction. She was treated with combined chemotherapy and radiation therapy, but she died due to sepsis. We herein report a rare case of angiocentric T-cell lymphoma on the nasal cavity and skin associated with Epstein-Barr virus.

Angiocentric T cell Lymphoma associated with Epstein-Barr Virus

Angiocentric T-cell lymphomas have been described as a distinctive clinicopathologic entity in the spectrum of peripheral T-cell lymphomas, with a prominent invasion of blood vessels by lymphomatous cells. In these conditions, the presence of Epstein-Barr virus (EBV) genomes has been demonstrated, suggesting that EBV might play a major role in their cause. Herein, we report a case of cutaneous angiocentric T cell lymphoma associated with the EBV. The patient was diagnosed with nasal angiocentric T cell lymphoma 5 months ago, and treated with cyclophosphamide, doxorubicin, vincristine, and prednisolone (CHOP) combination chemotherapy. After three cycles of CHOP, skin lesions developed. A skin biopsy specimen showed an angiocentric and angioinvasive infiltrate containing some atypical lymphocytes. EBV encoded RNA (EBER) was demonstrated in lesional skin by in situ hybridization.

Epstein Barr Virus-Associated Angiocentric T Cell Lymphoma in Nasal Cavity of Renal Transplantation Recipient / 대한이비인후과학회지

Posttransplant lymphoproliferative disorder (PTLD)is recognized as a significant and morbid complication of organ transplantation. The majority of PTLD is of B cell origin and strongly associated with Epstein Barr virus (EBV). T cell origin is uncommon and has a poor prognosis. There are only a few cases of PTLD of T cell origin, associated with EBV. We have experienced a case of angiocentric T cell lymphoma in nasal cavity, associated with EBV in a renal transplanted recipient. The patient was 25 years old woman who received transplant from her brother in 1995. Angiocentric T cell lymphoma was developed 33 months after the transplantation. We detected EBV mRNA in the neoplastic cells by in situ hybridization. She was treated by radiotherapy and is in complete remission state at present.

A Case of Angiocentric T-cell Lymphoma of the Esophagus / 대한소화기내시경학회지

In this study we present a case of an angiocentric T-cell lymphoma (ACTL) which involve the esophagus. A 37-year-old male was admitted with dysphagia, odynophagia, and weight loss for 3 months. Two months before his admission, he had undergone on endoscopy with a biopsy. The first endoscopic findings revealed a large ulcerative lesion on the esophagus, although there had been no evidence of lymphoma upon microscopic examination. On the follow up endoscopic examination, this ulcerative lesion revealed progressive and destructive change. Histopathology of the third biopsied specimens showed large atypical lymphocyte infiltrates with angiocentric and angioinvasive features. Immunohistochemical studies revealed that the atypical (large) lymphocytes had T-cell phenotypes (CD3+, CD5+, and CD43+) and contained few reactive B-cells (CD20+), We assume that this is the first case of ACTL involving the esophagus and larynx.

A Case of Angiocentric T Cell Lymphoma Accompanied with Multiple Erythematous Nodules, Subcutaneous Mass on the Right Face and Peripheral Polyneuropathy / 대한혈액학회지

Angiocentric T cell lymphoma is a variant of peripheral T cell lymphoma characterized by an angiocentric and angioinvasive infiltrates, invasion of vascular walls, and occulsion of lumen by atypical lymphoid cells with varying degrees of cytologic atypia. We recently experienced a woman with angiocentric T cell lymphorma, who presented with multiple erythematous nodules of skin, subcutaneous mass on right face and peripheral polyneuropathy. At the skin biopy specimen, we could find angiocentric and angioinvasive infiltrate by atypical lymphoid cells which showed a positive immunohistochemical reaction for CD45RO that is one of the T cell markers and a negative reaction for B cell marker. She was treated with two cycles of combination chemotherapy composed of CVP (cyclophosphamide, vincristine, prednisolone), that resulted in complete remission.

Peripheral T - Cell Lymphoma Involving Skin / 대한피부과학회지

BACKGROUND: Peripheral T-cell lymphomas(PTCL) frequently involve the skin and may occur as distinct clinicopathologic entities. However, clinicopathologic features of PTCLs involving the skin other than mycosis fungoides and Sezary syndrome have been poorly understood. OBJECT: This study aimed to characterize the nature of PTCL involving the skin in the Korean population. METHODS: Twenty-three cases of PTCL which involved the skin were collected during period from July, 1991 to June, 1995 In addition to reviewing the clinical records and routine histologic slides of t.hese cases, the immunohistochemical studies with several antibodies and in situhybridization for Epstein-Barr virus(EBV) early RNAs were performed on selected cases. RESULTS: We recognized five subtypes; mycosis fungoides, 5 cases, angiocentric T-cell lympho-ma, 9 cases, diffuse large cell lymphoma, 4 cases(Ki-1 positive, 3 cases, Ki-1 negative, 1 case), subcutaneous T-cell lymphoma, 1 case, and unspecified PTCL, 4 cases. Mycosis fungoides tends to involve papillary dermis with epidermotropism, where as angiocentric T-cell lymphoma and unspecified PTCI. predominantly affact adnexae, venules, and subcutis. Among 9 cases of angiocentric T-cell lymphoma, EBV RNAs were detected in 5 cases. In the prognostic point of view, mycosis fungoides and Ki- 1 positive large cell lymphoma were notably favorable and angiocentric T-cell lymphoma, especially EBV related, was guarded. CONCLUSION: A variety of the distinct entities of PTCL which frequently involve the skin were identified in the Korean population. In addition to the clinicopathologic information, immunophenotyping and EBV detection are thought to be crucial in diagnosing and predicting the behavior of T-cell neoplasms.

Angiocentric T - cell Lymphoma: Clinical and Histopathologic Features of Four Patients / 대한피부과학회지

BACKGROUND: Angiocentric T-cell lymphoma(AL) is a rare subtype of cutaneous T-cell lymphoma and recently many cases have been reported from Taiwan and Japan. But little is known about the clinical and histopathologic features of AL and few data are available in Korea. OBJECTIVE: Our purpose was to characterize the clinical and histopathologic features of the specific cutaneous manifestauons of AL and to investigate the association with Epstein-Barr virus (EBV). METHODS: Clinical records and histopathologic evaluation of skin biopsy specimens from four patients with AL were reviewed. We also performed immunohistochemical staining using monoclonal antibodies against, surface markers of lymphocytes and in situ hybridization for EBV-encoded RNA(EBER) in lesional skin. RESULTS: 1. The most common clinical finding in our patients was indurated plaque predominantly on the trunk. 2. The common histopathologic finding in skin biopsy specimens from all four patients was angiocentric, angioinvasive, and angiodestructive infiltrate containing atypical lymphocytes. 3. In three of four patients, EBER was demonstrated in lesional skin by in situ hybridization. CONCLUSION: AL is an unique clinicopathologic entity showing strong association with EBV in Korea.