Response and survival benefit with chemoimmunotherapy in Epstein-Barr virus-positive diffuse large B-cell lymphoma.

Epstein-Barr virus (EBV)-positive diffuse large B-cell lymphoma (DLBCL) is a haematologic malignancy with poor prognosis when treated with chemotherapy. We evaluated response and survival benefits of chemoimmunotherapy in EBV-positive DLBCL patients. A total of 117 DLBCL patients were included in our retrospective analysis; 33 were EBV-positive (17 treated with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone [R-CHOP] and 16 with CHOP), and 84 were EBV-negative (all treated with R-CHOP). The outcomes of interest were complete response (CR) and overall survival (OS) in EBV-positive DLBCL patients (R-CHOP versus CHOP) and in DLBCL patients treated with R-CHOP (EBV-positive vs EBV-negative). There were no differences in the clinical characteristics between EBV-positive and EBV-negative DLBCL patients. Among EBV-positive DLBCL patients, R-CHOP was associated with higher odds of CR (OR 3.14, 95% CI 0.75-13.2; P = .10) and better OS (hazard ratio 0.30, 95% confidence interval [CI] 0.09-0.94; P = .04). There were no differences in CR rate (OR 0.52, 95% CI 0.18-1.56; P = .25) or OS (hazard ratio 0.93, 95% CI 0.32-2.67; P = .89) between EBV-positive and EBV-negative DLBCL patients treated with R-CHOP. Based on our study, the addition of rituximab to CHOP is associated with improved response and survival in EBV-positive DLBCL patients. Epstein-Barr virus status does not seem to affect response or survival in DLBCL patients treated with R-CHOP.

Carcinoma adenoideo quístico de mama: serie de casos / Adenoid cystic carcinoma of the breast: case series

El carcinoma adenoideo quístico (CAQ) de mama es un cáncer poco frecuente y representa entre el 0,1% y el 0,4% de todos los carcinomas de mama. A continuación se presentan dos casos con el objetivo de describir un tipo de carcinoma de mama de presentación poco frecuente con evolución clínica no común. El primero de una mujer de 34 años y el segundo de una mujer de 44 años, ambas con diagnóstico confirmado de CAQ de mama. El CAQ de mama es de predominio en el sexo femenino en la etapa postmenopáusica. Sin embargo, ambas pacientes presentaron el CAQ en edad fértil. Generalmente posee un pronóstico favorable con rara diseminación, a diferencia de estos dos casos presentados donde se observa varias metástasis a distancia y un curso muy agresivo de la enfermedad.

Adenoid cystic carcinoma (ACC) of the breast is a rare neoplasm and accounts for 0.1% to 0.4% of all breast carcinomas. This is a description of two clinical cases that describe a rare presentation with an uncommon clinical course. The patients are 34 and 44 year old women, both with a confirmed diagnosis of ACC of the breast. The ACC of the breast predominantly grows in postmenopausal women. However, both patients are still fertile. Commonly it has a favorable prognosis and a rare presentation of metastasis. Nevertheless, in both cases, distant dissemination and an aggressive course of the disease was observed.

The neutrophil-to-lymphocyte ratio is an independent prognostic factor in patients with peripheral T-cell lymphoma, unspecified.

Peripheral T-cell lymphoma (PTCL) encompasses a group of rare and aggressive lymphomas. PTCL, unspecified (PTCLU) is the most common subtype of PTCL, and carries a poor prognosis. The International Prognostic Index (IPI) and the Prognostic Index for PTCLU (PIT) scoring systems are powerful risk-stratification tools in patients with PTCL. The aim of this study was to evaluate whether the neutrophil-to-lymphocyte ratio (NLR) is a prognostic factor in PTCLU. We retrospectively studied 83 patients with diagnosis of PTCLU. In the univariate analysis, NLR ≥ 4 was associated with worse overall survival (HR 3.96, 95% CI 1.92-8.17; p < 0.001). In the multivariate analysis, NLR ≥ 4 was independently associated with worse overall survival after adjustment for the PIT score (HR 4.30, 95% CI 1.90-9.69; p < 0.001), and for the IPI score (HR 2.60, 95% CI 1. 12-6.04; p = 0.03). Our study suggests the NLR could be helpful in refining the survival prognostication in patients with PTCLU.

[Primary non-polipoid intestinal folicular lymphoma: case report and review of the literature]. / Linfoma folicular primario intestinal no polipósico: reporte de un caso y revisión de la literatura.

The primary intestinal follicular lymphoma is a rare disease described in the last classification of lymphomas from WHO. It is a localized disease with excellent prognosis. We describe in this article ,a 64 year-old Peruvian female with abdominal pain and delayed vomiting for the last two years, has undergone a partial intestinal resection due to bowel obstruction. There was a well-circumscribed annular tumor. A diagnosis of non-polypoid primary intestinal follicular lymphoma was made. We report the case and review the literature in this article.

Linfoma folicular primario intestinal no polipósico: reporte de un caso y revisión de la literatura / Primary non-polipoid intestinal folicular lymphoma: case report and review of the literature

El linfoma folicular primario intestinal es un desorden raro descrito en la última clasificación de linfomas de la WHO. Es una entidad localizada con excelente pronóstico. En el presente artículo, reportamos una mujer peruana de 64 años de edad diagnosticada con linfoma folicular primario intestinal. Ella tuvo dos años con dolor abdominal y vómitos tardíos. Ella desarrolló una obstrucción intestinal y tuvo una resección completa del tumor. Se describe el caso y se realiza una revisión de la literatura de esta entidad.

The primary intestinal follicular lymphoma is a rare disease described in the last classification of lymphomas from WHO. It is a localized disease with excellent prognosis. We describe in this article ,a 64 year-old Peruvian female with abdominal pain and delayed vomiting for the last two years, has undergone a partial intestinal resection due to bowel obstruction. There was a well-circumscribed annular tumor. A diagnosis of non-polypoid primary intestinal follicular lymphoma was made. We report the case and review the literature in this article.

Follicular lymphoma with leukemic phase at diagnosis: a series of seven cases and review of the literature.

Follicular lymphoma (FL) is a prevalent type of non-Hodgkin lymphoma in the United States and Europe. Although, FL typically presents with nodal involvement, extranodal sites are less common, and leukemic phase at diagnosis is rare. There is mounting evidence that leukemic presentation portends a worse prognosis in patients with FL. We describe 7 patients with a pathological diagnosis of FL who presented with a leukemic phase. We compared our cases with 24 additional cases reported in the literature. Based on our results, patients who present with leukemic FL tend to have higher risk disease. Leukemic FL also seems to be associated with a worse prognosis; however, larger studies are needed to confirm our findings. A discrepancy with previously reported cases of FL in leukemic phase raises the possibility of differences attributable to geographic regions.

Diffuse large B-cell lymphoma in human T-lymphotropic virus type 1 carriers.

We describe the clinical and pathological characteristics of seven patients who were human T-lymphotropic virus type 1 (HTLV-1) carriers and had a pathological diagnosis of de novo diffuse large B-cell lymphoma. Interestingly, three of our cases showed positive expression of Epstein-Barr-virus, (EBV-) encoded RNA within the tumor cells indicating a possible interaction between these two viruses. Furthermore, our three EBV-positive cases presented with similar clinical characteristics such as early clinical stage and low-risk indices. To the best of our knowledge, this is the first case series describing the characteristics of HTLV-1-positive DLBCL patients. The potential relationship between HTLV-1 and EBV should be further explored.

Peripheral T-cell lymphoma with a regulatory T-cell phenotype: report of a nodal and an extranodal case from Peru.

T-cell regulatory lymphocytes (T reg) are identified by their reactivity with CD4, CD25, and FOXP3, and are variably present in the background of various neoplasms including hematopoietic tumors, and function modulating the immune response, including control of autoimmunity. Adult T-cell leukemia/lymphoma is an aggressive lymphoma associated with human T-lymphotrophic virus 1 infection characterized by the presence of neoplastic lymphocytes with a T reg phenotype; however, this phenotype is not characteristically found in other lymphomas. Here, we report 2 apparently immunocompetent human T-lymphotrophic virus 1-negative patients with nodal and extranodal peripheral T-cell lymphoma, not otherwise specified with a T reg immunophenotype, based on the strong CD25 and FOXP3 positivity of the neoplastic cells. One patient was a 48-year-old woman with an early stage tumor in the cavum, who despite of chemotherapy subsequently developed systemic disease and died of tumor progression 46 months from diagnosis. The second patient was a 65-year-old male with generalized adenopathy and B symptoms who received chemotherapy achieving a complete remission but had recurrence and died 36 months from diagnosis. The histopathology revealed a diffuse infiltrate with an interfollicular distribution in the second case, with nodal involvement, consisted of large cells with clear cytoplasm associated with vascular proliferation and abundant mitoses. Neoplastic cells of first case showed typical T reg phenotype, whereas the second case had a CD4/CD8 double negative T reg variant. Only a single similar case was found in a review of the literature. We conclude that peripheral T-cell lymphoma, not otherwise specified with a T reg phenotype may represent a distinct category of T-cell lymphoma with an aggressive clinical course and poor prognosis.

EBV-positive diffuse large B-cell lymphoma of the elderly: a case series from Peru.

EBV-positive diffuse large B-cell lymphoma (DLBCL) of the elderly is an entity recently included in the WHO classification of lymphoid tumors. We have reviewed our experience and clinical outcomes of this distinct subtype of DLBCL. Between 2002 and 2009, cases of DLBCL were identified from medical records of the Hospital Nacional Edgardo Rebagliati Martins in Lima, Peru, and underwent pathological evaluation including immunohistochemistry for CD20, CD10, bcl-6, MUM1/IRF4, and EBV-encoded RNA in situ hybridization. Clinical data were gathered, tabulated, and reported descriptively. Survival analyses were performed using Kaplan-Meier estimates. Out of 199 cases of DLBCL, 28 cases of EBV-positive DLBCL of the elderly were identified. The median age was 75 years with male predominance (1.5:1). B-symptoms were present in 43%, advanced stage in 50% and International Prognostic Index (IPI) score > 2 in 57% of patients; 68% of patients had a nongerminal center (NGC) phenotype. The complete response rates to R-CHOP and CHOP were 63% and 33%, respectively. The median overall survival (OS) for the group was 5 months. In the univariate analysis, age ≥70 years, lymphocyte count <1.0 × 10(9) /L, and advanced clinical stage were associated with worse OS in patients treated with chemotherapy with and without rituximab. EBV-positive DLBCL of the elderly is a clinically aggressive entity with a short OS and typically presents with advanced stage, high IPI score, and a NGC phenotype. Further studies are needed to investigate if rituximab-containing regimens are associated with better response and OS rates in EBV-positive DLBCL of the elderly.

Different prognostic factors for survival in acute and lymphomatous adult T-cell leukemia/lymphoma.

INTRODUCTION: Adult T-cell leukemia/lymphoma (ATLL) is a clinically aggressive and heterogeneous entity; hence it is likely that different variants of ATLL have different prognostic factors. METHODS: 95 patients with ATLL seen at our institution between 1987 and 2008 were included. Clinical data were compared, according to ATLL variant, using the Mann-Whitney and the Chi-square tests for continuous and categorical variables, respectively. Kaplan-Meier estimates compared using the log-rank test and Cox proportional-hazard test were used for the univariate and multivariate analysis, respectively. RESULTS: Median age was 61 years with male-to-female ratio of 1.07:1. Patients with acute ATLL were more likely to present with bone marrow, liver and spleen involvement, higher ß2-microglobulin and lower albumin levels. Poor performance status, high IPI score, presence of B symptoms, high LDH and low albumin levels were associated with a worse survival in lymphomatous ATLL. High LDH, high ß2-microglobulin and high PIT score were associated with worse survival in acute ATLL. In the multivariate analysis, low albumin level and presence of B symptoms were independent factors for worse survival in lymphomatous ATLL, and high ß2-microglobulin level was independent factor for worse survival in acute ATLL. CONCLUSIONS: Aggressive ATLL variants have a distinct, almost mutually exclusive profile of prognostic factors.

[Gastrointestinal stromal tumor (GIST) of Duodenum: Case report]. / Tumor estromal gastrointestinal en duodeno.

Female of 64 years old, who two years ago was hospitalized in another institution for treatment of an abscess in the right liver lobe. During that hospitalization, it was found a duodenal tumor by tomography; however, no further studies or follow up on this finding was done. Prior to actual hospitalization, she was seen in a private clinic and treated with antibiotics for a suggestive"organized abscess" in the pancreatic head diagnosed by tomography. She presented with fever, abdominal pain and a palpable abdominal tumor in the right upper quadrant for a month. We realized a three-phase multislice spiral tomography with pancreatic curved reconstruction, which allowed to identified an ovoid tumor of 80 x 60 mm, with an area of central necrosis and intense contrast enhancement (arterial phase), located between the second and third duodenal portion, near the head of the pancreas. An upper endoscopy showed an extrinsic compression of the third duodenal portion. The patient underwent tumor resection without complication and the histologic diagnosis of the surgical specimen revealed a duodenal stromal tumor (GIST) with extraluminal transmural growth. We presented this case because of its unusual presentation of this tumor.

Tumor estromal gastrointestinal en duodeno / Gastrointestinal stromal tumor (GIST) of duodenum

Paciente mujer de 64 años de edad, quien hace dos años estuvo hospitalizada en otra institución para el tratamiento de un absceso en el lóbulo hepático derecho. Durante esa hospitalización se diagnosticó un tumor duodenal por tomografía; sin embargo, no se completó el estudio sobre este hallazgo. Previa a la actual hospitalización, la paciente estuvo internada en una clínica privada, donde fue tratada con antibióticos por una imagen sugestiva de "absceso organizado" en la cabeza del páncreas. Desde hace un mes presentaba fiebre, dolor abdominal y una masa palpable en el cuadrante superior derecho del abdomen. Se le realizó una tomografía espiral multicorte trifásica, con reconstrucción curvada de páncreas que permitió reconocer una tumoración ovoidea de 80 x 60 mm, con área de necrosis central e intenso realce del contraste (fase arterial), localizada entre la segunda y tercera porción duodenal, próxima a la cabeza de páncreas. La endoscopía alta demostró una compresión extrínseca en la tercera porción duodenal. La paciente fue sometida a una resección tumoral sin complicaciones y la histología del espécimen quirúrgico reveló un Tumor Estromal Gastrointestinal (GIST) Duodenal, de crecimiento transmural extraluminal. Presentamos el caso por ser una inusual presentación este tipo de tumores.

Female of 64 years old, who two years ago was hospitalized in another institution for treatment of an abscess in the right liver lobe. During that hospitalization, it was found a duodenal tumor by tomography; however, no further studies or follow up on this finding was done. Prior to actual hospitalization, she was seen in a private clinic and treated with antibiotics for a suggestive "organized abscess" in the pancreatic head diagnosed by tomography. She presented with fever, abdominal pain and a palpable abdominal tumor in the right upper quadrant for a month. We realized a three-phase multislice spiral tomography with pancreatic curved reconstruction, which allowed to identified an ovoid tumor of 80 x 60 mm, with an area of central necrosis and intense contrast enhancement (arterial phase), located between the second and third duodenal portion, near the head of the pancreas. An upper endoscopy showed an extrinsic compression of the third duodenal portion. The patient underwent tumor resection without complication and the histologic diagnosis of the surgical specimen revealed a duodenal stromal tumor (GIST) with extraluminal transmural growth. We presented this case because of its unusual presentation of this tumor.

[Gastric adult T cell leukemia/lymphoma: report of four cases and review of literature]. / Leucemia/linfoma T del adulto gástrico: reporte de cuatro casos y revisión de la literatura.

Adult T-cell leukemia/lymphoma (ATLL) is an aggressive disease associated with human T-cell lymphotropic virus type-I (HTLV-I) with heterogeneous clinical presentation and outcomes. We report four cases with gastric involvement. We describe clinical and endoscopic findings of cases and review literature.

Leucemia/Linfoma T Gástrico del adulto: reporte de cuatro casos y revisión de la literatura / Gastric adult T cell Leukemia/Lynphoma: report of four cases and review of literature

Leucemia/Linfoma T del Adulto (ATLL) es una enfermedad agresiva asociada con el virus linfotrópico de células T tipo I (HTLV-1) con presentación y curso clínico heterogéneo. El reporte describe cuatro casos con compromiso gástrico, considerando características clínicas y hallazgos endoscópicos, con la respectiva revisión de la literatura.

Adult T-cell leukemia/lymphoma (ATLL) is an aggressive disease associated with human T-cell lymphotropic virus type-I (HTLV-I) with heterogeneous clinical presentation and outcomes. We report four cases with gastric involvement. We describe clinical and endoscopic findings of cases and review literature.

Epstein-Barr virus as a prognostic factor in de novo nodal diffuse large B-cell lymphoma.

Although the International Prognostic Index (IPI) score is a valuable prognostic tool in diffuse large B-cell lymphoma (DLBCL), other risk-stratifying factors may be of value. The aim of this study was to define the prognostic value of EBV expression in de novo nodal DLBCL. Seventy-four cases were selected between January 2002 and December 2007. Clinical data were reviewed and tissue samples were evaluated for expression of CD20, CD10, bcl-6, MUM1, and EBV-encoded RNA (EBER). Of 74 evaluated cases, 53 cases (72%) were of non-germinal center-like subtype and 11 cases (15%) were positive for EBER. In a univariate analysis of the 57 patients who received chemotherapy, factors associated with survival were EBV status, performance status, LDH level, and IPI score. Using a multivariate analysis, a prognostic model was developed using IPI score and EBV status, which showed statistical significance. Our study supports EBV status as a powerful prognostic factor in de novo nodal DLBCL. Prospective studies should be carried to validate this hypothesis.

[Corticosteroid refractory colonic Crohn disease with successful remission with infliximab]. / Remisión de Crohn colónico refractario a corticoides con Infliximab.

We review the case of a 14 year old patient complaining of 5 months of bloody and mucous diarrhea, abdominal pain, fever and 10 kg weight loss. At the beginning she received cotrimoxazole and then chloramphenicol with no success. With the suspicious of tuberculosis she was treated for a month with the corresponding drugs with no clinical improvement. Colonoscopy revealed the presence of punch - like ulcers, deep linear ones cobblestoning and skip lesions. The histological analysis showed severe distortion the colonic mucosa, ulcers, granulation tissue, lymphoid follicles and a trend towards the formation of granulomatous lesions. The clinical, endoscopic and histological features were consistent with Crohn disease. The activity index for it was greater than 450. The patient received 60 mg bid hydrocortisone but switched to 20 mg bid methylprednisolone at day 10th. After 10 days and because she was doing worst refractoriness to corticoids was considered and 5 mg / kg Infliximab was started. At the third day of treatment symptoms improved dramatically and one week later was almost asymptomatic. Similar dosage of Infliximab was repeated at weeks 2, 6 and 8. Currently the patient is being treated with 75 mg azathioprine as maintenance therapy.