Linfoma no Hodgkin difuso de células B grande como linfoma primario de ovario. Presentación de caso / Diffuse large B-cell non-Hodgkin's lymphoma as primary ovarian lymphoma. Case presentation

Fundamento: Los linfomas primarios de ovario son poco frecuentes; el 1 % de estos se presenta en ovario y el 1.5 % de los tumores malignos de ovario son linfomas. Los tipos histológicos más frecuentes es el linfoma no Hodgkin difuso de células B grande y el BurKitt; el tratamiento consiste en cirugía combinada con quimioterapia. Objetivo: Reportar un caso de un linfoma no Hodgkin difuso de células B grande primario de ovario. Presentación de caso: Se presentó el caso de una paciente de 39 años de edad, con antecedentes patológicos personales de salud; la cual fue al cuerpo de guardia de ginecología por presentar dolor abdominal difuso que no se aliviaba con analgésicos. En la exploración física presentaba dolor a la palpación superficial y profunda en hipocondrio y fosa ilíaca derecha con masa tumoral palpable. Ecografía hacia proyección anexial derecha se observó una imagen de baja ecogenicidad y en la laparoscopia de urgencia se concluyó como una formación de aspecto tumoral que parecía corresponderse con ovario derecho. Se le realizó una histerectomía con doble anexectomía. El diagnóstico anatomopatológico fue un linfoma no Hodgkin primario de ovario. Conclusiones: La paciente del caso presentado tuvo una clínica oligosintomática y la confirmación de la enfermedad fue a partir de una muestra quirúrgica, lo que expresa que el diagnóstico del linfoma no Hodgkin de células B es difícil y aunque es poco frecuente siempre se debe tener en cuenta en el diagnóstico diferencial de las tumoraciones unilaterales de ovario.

Background: Primary ovarian lymphomas are uncommon, 1% of these malignancies occur in the ovary, and 1.5% of all ovarian malignancies are lymphomas. The most common histologic types are diffuse large B-cell non-Hodgkin's lymphoma and BurKitt's lymphoma; treatment consists of surgery combined with chemotherapy. Objective: To report a case of primary ovarian diffuse large B-cell non-Hodgkin lymphoma. Case presentation: A 39-year-old female case is presented, with a personal pathological history; she went to the gynecology emergency service because she presented diffuse abdominal pain that was not relieved by analgesics. Physical examination revealed superficial and deep pain on palpation in the hypochondrium and right illiac fossa with a palpable tumor mass. Right adnexal ultrasound showed an image of low echogenicity and at the emergency laparoscopy, it was diagnosed as a tumor-like formation that appeared to correspond to the right ovary. She underwent a hysterectomy with double adnexectomy. The anatomopathologic diagnosis was primary ovarian non-Hodgkin's lymphoma. Conclusions: The patient in the presented case had an oligosymptomatic clinical presentation. Confirmation of the disease was obtained from a surgical sample, which means that B-cell non-Hodgkin's lymphoma is difficult to diagnose and although it is uncommon, it should always be considered in the differential diagnosis of unilateral ovarian tumors.

[Cardiovascular complications in pregnant woman with Primary Mediastinal B-Cell Lymphoma]. / Complicaciones cardiovasculares en gestante con linfoma primario mediastinal de células B.

A 26-year-old pregnant woman with 29 weeks of gestational age reported two months of cough, dyspnea, orthopnea, and palpitations. Chest tomography revealed a 10x12cm solid mass in the right lung. Echocardiography showed that the tumor compromised the right atrium and ventricle, and was diagnosed by transcutaneous biopsy as Primary Mediastinal B-Cell Lymphoma (PMCBL). The patient presented 2:1 atrial flutter, sinus bradycardia, and ectopic atrial bradycardia. Due to the rapid poor evolution, it was decided to terminate the pregnancy by cesarean section and start chemotherapy, after which the cardiovascular complications resolved. PCML is a very rare lymphoma that can affect pregnant women in any trimester, its symptoms are related to its rapid growth and compromise of the heart, causing various cardiovascular manifestations (heart failure, pericardial effusion, cardiac arrhythmias). PCMLC is characteristically chemosensitive and has a good prognosis.

Central nervous system relapse in diffuse large B lymphoma: a retrospective cohort study / [Recaída en sistema nervioso central en linfoma difuso de células B grandes: estudio de cohorte retrospectiva]

Introduction: CNS relapse in patients with LDCGB is a poor prognosis event. The incidence of relapse is variable according to the literature. Data in Latin America is lacking. Methods: In order to establish the incidence of CNS relapse in our cohort, time to CNS relapse and the impact of CNS relapse risk factors, a retrospective cohort study was performed, from January 2012 to June 2017. Results: One hundred and forty seven patients were analyzed. The median age was 66 years (ICR 56-76); 76 patients (51.70%) were men. The IPI was low or intermediate/low in 115 (78.2%) cases. The CNS IPI was intermediate in 77 (52.4%) and high in 14 (9.5%) of cases. Thirty-five (23.81%) patients received intrathecal prophylaxis. No patient received systemic prophylaxis. During the follow-up, 8 (4.59%) patients had CNS relapse, none of them with high IPI. The median time to relapse was 6.5 months (ICR 5.5-10). Seven (87.5%) patients relapsed within the year of diagnosis. We found no risk factors for CNS involvement in the bivariate analysis. The incidence of relapse was 2.7% (CI 0.2% -4.6%), 4.8% (CI 1.8% -8.9%) and 5.4% (CI 4.5- 8.9%) at 6, 12 and 24 months, respectively. Discussion: The incidence of CNS relapse was similar to that described in the international series. Our study confirms that the majority of patients relapse during the first year of follow up. We must carry out broader collaborative work to better establish the risk factor for CNS relapse.

Introducción: La recaída del SNC en pacientes con LDCBG en un evento de mal pronóstico. La incidencia de recaída  es variable según la bibliografía. Los datos en América Latina son escasos. Metodología: Para establecer la incidencia de recaída en SNC, el tiempo a la recaída en SNC y el impacto de los factores de riesgo de recaída del SNC, se realizó un estudio de cohorte retrospectiva, de enero del 2012 a junio del 2017. Resultados: Se analizaron 147 pacientes. La mediana de edad fue de 66 años (IIC 56-76); 76 pacientes (51,70%) fueron hombres. El IPI fue bajo o intermedio/bajo en 115 (78,2%) casos. El CNS IPI fue intermedio en 77 (52,4%) y alto en 14 (9,5%) casos. Treinta y cinco (23.81%) pacientes recibieron profilaxis intratecal del SNC, ninguno profilaxis sistémica. Durante el seguimiento, 8 (4,59%) pacientes tuvieron recaída del SNC. La mediana de tiempo a la recaída fue de 6,5 meses (IIC 5,5 - 10). Siete (87,5%) pacientes recayeron dentro del año. No encontramos factores de riesgo para el compromiso del SNC en el análisis bivariado. La incidencia de recaída fue del 2,7% (IC 0,2%-4,6%), 4,8% (IC 1,8%-8,9%) y 5,4% (IC 4,5-8,9%) a los 6, 12 y 24 meses, respectivamente. Discusión: La incidencia de recaída del SNC fue similar a la descrita en las series internacionales. Nuestro estudio confirma que la mayoría de los pacientes que recaen, lo hacen dentro del primer año del diagnóstico. Debemos realizar trabajos colaborativos más amplios para establecer mejor factores de riesgo asociados.

Linfoma testicular primario tipo B de célula grande: aspectos clínicos, histopatológicos, diagnóstico terapéutico y pronósticos / Large cell type B primary testicular lymphoma: clinical, histopathological, therapeutic diagnosis and prognostic aspects

RESUMEN Fundamento: el linfoma testicular constituye entre el 1 y el 9 % de los tumores testiculares, es el tumor testicular maligno más frecuente en los varones mayores de 50 años. El pronóstico es reservado debido a una gran tendencia a la propagación sistémica temprana. La presentación clínica más habitual, dolor testicular a la palpación y espontáneo, con aumento del volumen y de la consistencia testicular. Su tratamiento se sustenta en tres pilares fundamentales: orquiectomía, quimioterapia y radioterapia. Objetivo: describir un enfermo con linfoma testicular tipo B de célula grande. Presentación del caso: paciente masculino de 55 años de edad, hipertenso, fumador 10 cigarrillos al día, bebedor ocasional. Acude a consulta por aumento de volumen testicular izquierdo. En la analítica se observa elevación del lactato deshidrogenasa. Ecografía: teste izquierdo con ecogenicidad muy heterogénea, parénquima desestructurado, sin lesiones nodulares. Engrosamiento difuso del epidídimo. Vascularización incrementada. Se realiza orquiectomía radical inguinal, al ser el diagnóstico anatomopatológico linfoma difuso de células grandes tipo B, en la actualidad ha sido tratado con quimioterapia, por presentar además afectación ósea. Pero ha evolucionado favorablemente. Conclusiones: el linfoma testicular es una enfermedad poco frecuente, a pesar de ser considerado el tumor testicular más común en mayores de 60 años. En su mayoría se trata de linfomas no Hodgkin difusos de grado intermedio alto de malignidad e inmunofenotipo B. El pronóstico siempre es reservado debido a su gran tendencia a la propagación sistémica. El tratamiento está basado en la cirugía (orquiectomía radical inguinal), quimioterapia y radioterapia.

ABSTRACT Background: testicular lymphoma constitutes between 1 and 9 % of testicular tumors; it is the most frequent malignant testicular tumor in men over 50 years of age. The prognosis is reserved due to a great tendency to early systemic spread. The most common clinical presentation is testicular pain on palpation and spontaneous, with increased volume and testicular consistency. Its treatment is based on three fundamental pillars: orchiectomy, chemotherapy and radiotherapy. Objective: to describe a patient with large cell type B testicular lymphoma. Case report: 55-year-old male patient, hypertensive, smoker 10 cigarettes a day, occasional drinker. He goes to consultation due to left testicular volume increase. In the laboratory tests, the lactate dehydrogenase is elevated. Ultrasound Scand: Left testicle with very heterogeneous echogenicity, unstructured parenchyma, no nodular lesions. Diffuse thickening of the epididymis. Increased vascularization. Inguinal radical orchiectomy is performed, the anatomic-pathological diagnosis being diffuse large B cell type lymphoma, it has now been treated with chemotherapy, because he alsopresented bone involvement. But he has evolved favorably. Conclusions: testicular lymphoma is a very rare entity, despite being considered the most common testicular tumor in people over 60 years of age. The majority are diffuse non-Hodgkin lymphomas of high intermediate degree of malignancy and immune-phenotype B. The prognosis is always reserved due to its great tendency to systemic spread. The treatment is based on surgery (radical inguinal orchiectomy), chemotherapy and radiotherapy.

Absolute monocyte count as a prognostic parameter in diffuse large B cell lymphoma / Recuento absoluto de monocitos como parámetro pronóstico en linfoma difuso de células B grandes

ABSTRACT Background Prognosis of patients with Diffuse Large B Cell Lymphoma (DLBCL) is highly variable, and despite the use of modern immunochemotherapy regimens, almost 50% of patients will eventually relapse. Standard risk models, like the International Prognostic Index or the Revised International Prognostic Index (R_IPI) incorporate patient and tumor characteristics but do not consider variables related to host adaptive immunity which have been shown to be of significant prognostic value in non-Hodgkin lymphomas. Aim To analyze the prognostic significance of the absolute monocyte count at diagnosis in diffuse large-B-cell lymphoma in a retrospective setting. Material and Methods We reviewed data of 171 patients with DLBCL treated with Rituximab-based immunochemotherapy at two reference public Hospitals in Montevideo-Uruguay. The outcome measures were overall and relapse free survival. Results The absolute monocyte count, analyzed as a dichotomized variable predicted progression-free and overall survival in low risk patients according to the R-IPI score. Worse outcomes were observed in those with high monocyte count al diagnosis. Conclusions Absolute monocyte count could help in the identification of high-risk patients otherwise expected to have a good prognosis according to traditional scores.

Antecedentes El pronóstico de pacientes con Linfoma Difuso de Células B Grandes (DLBCL) es muy variable y el 50% de los pacientes recae a pesar de uso de regímenes actualizados de inmuno-quimioterapia. Los modelos pronósticos clásicos como el International Prognostic Index o el Revised International Prognostic Index (R_IPI) incorporan características del paciente o del tumor pero no incorporan variables asociadas a la inmunidad adaptativa que tienen valor en linfomas no Hodgkin. Objetivo Analizar retrospectivamente el valor pronóstico del recuento absoluto de monocitos al momento del diagnóstico en pacientes con DLBCL. Material y Métodos Se revisó información de 171 pacientes con DLBCL tratados con inmuno-quimioterapia basada en rituximab en dos centros de referencia públicos de Montevideo, Uruguay. Las variables de resultado fueron la sobrevida global y libre de recaída. Resultados El recuento absoluto de monocitos, tratado como una variable dicotómica, predijo la sobrevida libre de recaída en pacientes de bajo riesgo, de acuerdo al puntaje R-IPI. El pronóstico fue peor en pacientes con altos recuentos al momento del diagnóstico. Conclusiones El recuento absoluto de monocitos puede identificar pacientes de alto riesgo, clasificados como de bajo riesgo por los puntajes tradicionales.

Primary diffuse large B-cell lymphoma of the mandible.

Primary intraosseous non-Hodgkin lymphoma in the mandible is uncommon, representing about 0.6% of all extranodal lymphomas. We present the case of a 51-year-old male with a 4-month complaint of mandibular swelling and paresthesia, which had been previously submitted to an unsuccessful periodontal treatment. The intra-oral evaluation showed an extensive swelling with teeth mobility in the right mandible body. The panoramic radiography and computed tomography images showed an extensive osteolytic lesion. An incisional biopsy was performed and the histopathological and immunohistochemical analysis established the diagnosis of diffuse large B-cell lymphoma. The treatment included six cycles of chemotherapy with complete remission. The patient is under the seventh month of follow-up with no evidence of relapse. Although uncommon in the oral cavity, lymphoma should be considered in the differential diagnosis.

High-grade B-Cell lymphoma with MYC and BCL6 rearrangements associated with Richter transformation of chronic lymphocytic leukemia

Richter transformation (RT), or Richter syndrome, is defined as the transformation of chronic lymphocytic leukemia (CLL) to an aggressive B-cell lymphoma. The vast majority, up to 99%, transform into diffuse large B-cell lymphoma (DLBCL), with a small subset (<1%) becoming classical Hodgkin lymphoma. Approximately half of RT cases progress through a pathway involving dysregulation of C-MYC. High-grade B-cell lymphoma (HGBL) is a recent diagnostic category of aggressive B-cell lymphomas set forth in the updated 2017 WHO Classification of Hematopoietic and Lymphoid Tissues. HGBL with MYC and BCL2 and/or BCL6 rearrangements, formerly "double-hit" and "triple-hit" lymphomas, comprise the majority of HGBL cases. Patients with HGBL have a worse prognosis than those with diffuse large B-cell lymphoma. We present a case of RT with rearrangements of MYC and BCL6. To our knowledge, there are no reported cases of RT with a "double-hit" lymphoma genotype.

Primary diffuse large B-cell lymphoma of the mandible

Primary intraosseous non-Hodgkin lymphoma in the mandible is uncommon, representing about 0.6% of all extranodal lymphomas. We present the case of a 51-year-old male with a 4-month complaint of mandibular swelling and paresthesia, which had been previously submitted to an unsuccessful periodontal treatment. The intra-oral evaluation showed an extensive swelling with teeth mobility in the right mandible body. The panoramic radiography and computed tomography images showed an extensive osteolytic lesion. An incisional biopsy was performed and the histopathological and immunohistochemical analysis established the diagnosis of diffuse large B-cell lymphoma. The treatment included six cycles of chemotherapy with complete remission. The patient is under the seventh month of follow-up with no evidence of relapse. Although uncommon in the oral cavity, lymphoma should be considered in the differential diagnosis.

High-grade B-Cell lymphoma with MYC and BCL6 rearrangements associated with Richter transformation of chronic lymphocytic leukemia.

Richter transformation (RT), or Richter syndrome, is defined as the transformation of chronic lymphocytic leukemia (CLL) to an aggressive B-cell lymphoma. The vast majority, up to 99%, transform into diffuse large B-cell lymphoma (DLBCL), with a small subset (<1%) becoming classical Hodgkin lymphoma. Approximately half of RT cases progress through a pathway involving dysregulation of C-MYC. High-grade B-cell lymphoma (HGBL) is a recent diagnostic category of aggressive B-cell lymphomas set forth in the updated 2017 WHO Classification of Hematopoietic and Lymphoid Tissues. HGBL with MYC and BCL2 and/or BCL6 rearrangements, formerly "double-hit" and "triple-hit" lymphomas, comprise the majority of HGBL cases. Patients with HGBL have a worse prognosis than those with diffuse large B-cell lymphoma. We present a case of RT with rearrangements of MYC and BCL6. To our knowledge, there are no reported cases of RT with a "double-hit" lymphoma genotype.

Primary infratentorial diffuse large b-cell lymphoma: a challenging diagnosis in an immunocompetent patient

SUMMARY We describe the case of a female patient, 52 years old, with dizziness and left motor incoordination for 2 weeks. Brain MRI magnetic resonance imaging) revealed a hyperintense lesion on T2-weighted images, without restricted diffusion, in the left middle cerebellar peduncle. Spectroscopy demonstrated peak of lipids and perfusion did not show any elevation in relative cerebral blood volume (rCBV). The patient underwent an open biopsy and resection, and the diagnosis of diffuse large B-cell lymphoma (DLBCL) was established. The patient received intravenous dexamethasone with symptoms remission, followed by four cycles of methotrexate plus cytarabine. After 3 months, the patient returned with decreased consciences level and a new MRI revealed a right superior frontal gyrus lesion with features suggesting a lymphomatous lesion. The patient died five days after her relapse.

RESUMO Descrevemos o caso de uma paciente do sexo feminino, de 52 anos, apresentando história de tontura e perda da coordenação motora do lado esquerdo há duas semanas. A RM (ressonância magnética) de crânio revelou uma lesão hiperintensa nas imagens ponderadas em T2, sem restrição à difusão, localizada no pedúnculo cerebelar médio esquerdo. A espectroscopia demonstrou pico de lipídeos, sem elevação do volume sanguíneo cerebral relativo (rCBV) à perfusão. A paciente foi submetida à biópsia a céu aberto, estabelecendo o diagnóstico de linfoma difuso de grandes células B (DLBCL). Houve remissão dos sintomas após o início do tratamento com dexametasona endovenosa, seguida de quatro ciclos de metotrexato associado à citarabina. Após três meses, a paciente retornou apresentando rebaixamento do nível de consciência, e a RM de crânio revelou uma nova lesão de origem linfomatosa no giro frontal superior direito. A paciente faleceu após cinco dias.

PRIMARY BONE LYMPHOMAS: RETROSPECTIVE ANALYSIS OF 42 CONSECUTIVE CASES.

OBJECTIVE: It is difficult to define parameters for management and factors associated with primary bone lymphoma (PBL). This article presents the experience in a single institution with 42 patients with PBL over a 16-year period (2000-2016). METHODS: Fifty-five patients were retrospectively evaluated, and forty-two were included (76.3%). RESULTS: Median age at diagnosis was 51.5 years, and median follow-up was 102.7 months. One patient had HIV. Pain in the affected site was the most prevalent symptom. The average time between symptom onset and diagnosis was 5.4 months. The vertebrae were most affected (n=16, 33.3%). According to the International Prognostic Index Score (IPI), 64.3% of the patients were classified as having low-grade lymphoma and 25.7% as low-intermediate. The most common histology was diffuse large B cell lymphoma (DLBCL) (85.7%). Immunophenotyping was CD20 positive in 93.5% of patients, and 11 patients had pathological fracture. All patients received chemotherapy and 30% of the regimens included rituximab. Thirty-eight percent of patients received radiation therapy. Overall survival was 50%, and survival median time was 80 months. Age and chemotherapy regimen influenced patient survival. Younger patients and patients who received RCHOP had better prognoses. CONCLUSIONS: The choice of chemotherapy regimen associated with age influenced survival for patients with PBL. Level of Evidence IV; Case series.

OBJETIVO: É difícil definir parâmetros para o tratamento e os fatores associados ao prognóstico de linfoma ósseo primário (LOP). Apresentamos a experiência de uma única instituição, com 42 casos de LOP durante 16 anos (2000-2016). MÉTODOS: Cinquenta e cinco pacientes avaliados retrospectivamente e quarenta e dois incluídos (76,3%). RESULTADOS: A mediana da idade foi 51,5 anos e a mediana do seguimento foi 102,7 meses. Um paciente era portador do vírus da imunodeficiência humana. Dor no membro foi o sintoma mais prevalente. O tempo médio entre os sintomas e o diagnóstico foi de 5,4 meses. O sítio anatômico mais afetado foram as vértebras (n = 16, 33,3%). Em relação à International Prognostic Index Score (IPI), 64,3% dos pacientes tinham baixo grau e 25,7% tinham baixo-intermediário. O diagnóstico histológico mais comum foi linfoma difuso de grandes células B (LDGCB), com 85,7% dos casos. A imunofenotipagem foi positiva para CD20 em 95,3%. Onze pacientes tinham fratura patológica. Todos receberam quimioterapia, sendo que 30% dos regimes incluíam rituximabe. A radioterapia foi utilizada em 38% dos pacientes. A porcentagem total de sobrevida foi de 50% e o tempo médio de sobrevida foi de 80 meses. A idade e o regime de quimioterapia juntos influenciaram diretamente a sobrevida dos pacientes. Os pacientes jovens, assim como os que receberam RCHOP, tiveram prognóstico de sobrevida melhor. CONCLUSÃO: A escolha do regime de quimioterapia associada à idade do paciente influenciou a sobrevida dos pacientes com LOP. Nível de Evidência IV; Série de casos.

Primary bone lymphomas: retrospective analysis of 42 consecutive cases / Linfoma ósseo primário: análise retrospectiva de 42 casos consecutivos

ABSTRACT Objective: It is difficult to define parameters for management and factors associated with primary bone lymphoma (PBL). This article presents the experience in a single institution with 42 patients with PBL over a 16-year period (2000-2016). Methods: Fifty-five patients were retrospectively evaluated, and forty-two were included (76.3%). Results: Median age at diagnosis was 51.5 years, and median follow-up was 102.7 months. One patient had HIV. Pain in the affected site was the most prevalent symptom. The average time between symptom onset and diagnosis was 5.4 months. The vertebrae were most affected (n=16, 33.3%). According to the International Prognostic Index Score (IPI), 64.3% of the patients were classified as having low-grade lymphoma and 25.7% as low-intermediate. The most common histology was diffuse large B cell lymphoma (DLBCL) (85.7%). Immunophenotyping was CD20 positive in 93.5% of patients, and 11 patients had pathological fracture. All patients received chemotherapy and 30% of the regimens included rituximab. Thirty-eight percent of patients received radiation therapy. Overall survival was 50%, and survival median time was 80 months. Age and chemotherapy regimen influenced patient survival. Younger patients and patients who received RCHOP had better prognoses. Conclusions: The choice of chemotherapy regimen associated with age influenced survival for patients with PBL. Level of Evidence IV; Case series.

RESUMO Objetivo: É difícil definir parâmetros para o tratamento e os fatores associados ao prognóstico de linfoma ósseo primário (LOP). Apresentamos a experiência de uma única instituição, com 42 casos de LOP durante 16 anos (2000-2016). Métodos: Cinquenta e cinco pacientes avaliados retrospectivamente e quarenta e dois incluídos (76,3%). Resultados: A mediana da idade foi 51,5 anos e a mediana do seguimento foi 102,7 meses. Um paciente era portador do vírus da imunodeficiência humana. Dor no membro foi o sintoma mais prevalente. O tempo médio entre os sintomas e o diagnóstico foi de 5,4 meses. O sítio anatômico mais afetado foram as vértebras (n = 16, 33,3%). Em relação à International Prognostic Index Score (IPI), 64,3% dos pacientes tinham baixo grau e 25,7% tinham baixo-intermediário. O diagnóstico histológico mais comum foi linfoma difuso de grandes células B (LDGCB), com 85,7% dos casos. A imunofenotipagem foi positiva para CD20 em 95,3%. Onze pacientes tinham fratura patológica. Todos receberam quimioterapia, sendo que 30% dos regimes incluíam rituximabe. A radioterapia foi utilizada em 38% dos pacientes. A porcentagem total de sobrevida foi de 50% e o tempo médio de sobrevida foi de 80 meses. A idade e o regime de quimioterapia juntos influenciaram diretamente a sobrevida dos pacientes. Os pacientes jovens, assim como os que receberam RCHOP, tiveram prognóstico de sobrevida melhor. Conclusão: A escolha do regime de quimioterapia associada à idade do paciente influenciou a sobrevida dos pacientes com LOP. Nível de Evidência IV; Série de casos.

Hipercalcemia, peak monoclonal y leucemización como presentación de un linfoma de células B de alto grado, con reordenamiento de MYC, BCL-2 y BCL-6 (Triple Hit). Caso clínico / Hypercalcemia, monoclonal peak and leukemic presentation of a High-grade B-cell lymphoma with MYC, BCL-2 and BCL-6 translocations (triple hit). Report of one case

High-grade B-cell lymphomas with rearrangement of MYC, BCL-2 and/or BCL-6 were introduced by the update of the WHO classification of lymphoid neoplasms. They usually present unique morphological and molecular characteristics, with an aggressive clinical outcome and worse prognosis. We report a 48 year-old female patient presenting with B symptoms and enlarged lymph nodes. Blood count showed pancytopenia and peripheral blood smears showed large lymphoid cells, some with nuclei and vacuoles. LDH was 3524 g/L and serum calcium was 11.5 mg/dL. Flow cytometry immunophenotyping showed pathological mature B lymphocytes. Protein electrophoresis showed a slight monoclonal peak. The biopsy disclosed a triple expressor diffuse large B-cell lymphoma, arising from germinal center. FISH was positive for MYC, BCL-2 and BCL-6 (triple hit) with a clonal evolution. Conventional cytogenetics showed a complex karyotype. Chemotherapy was started with R-CHOP (Rituximab/cyclophosphamide/doxorubicin/vincristine/prednisone). She developed impaired consciousness; the brain CT scan showed a large brain mass. The patient died within 3 weeks.

Linfoma de células grandes B originado en enfermedad de Castleman: reporte de un caso y revisión de la literatura / Large B-cell lymphoma coexisting with Castleman's disease: report of one case

We report a 73-year-old female patient with Castleman's disease coexistent with large B cell type non-Hodgkin's lymphoma in a right axillary lymphadenopathy. An excisional biopsy was performed: microscopically, the lymph node revealed the presence of numerous plasma cells and small lymphoid cells characteristic of Castleman's disease. An analysis of another portion of the specimen revealed lymphoid cells with large abnormal nuclei gathered locally that were CDD 79+, CD 38+ and MUM-1+ as well as positive for Kaposi sarcoma-associated herpesvirus and negative for Epstein Barr virus encoded RNA-1 (EBER).

Relato de caso: Linfoma difuso de grandes células B no colo uterino / Case report: Diffuse large B cells lymphoma in the cervix

Relatamos o caso de mulher, 32 anos, branca, previamente hígida, atendida com corrimento vaginal e metrorragia há 3 meses. Exame colpocitológico descreveu esfregaço intensamente hemorrágico e lesão intraepitelial de alto grau com displasia acentuada (NIC III). Ultrassonografia transvaginal e ressonância magnética da pelve caracterizaram aumento dimensional do colo uterino, exibindo aspecto em "barril", por processo expansivo difuso de aspecto infiltrativo. Estudo anatomopatológico e imunohistoquímico de biopsia do colo uterino revelou um linfoma não Hodgkin difuso de grandes células B. (AU)

We report the case of a woman, 32 years old, white, previously healthy, presenting vaginal discharge and metrorrhagia for three months. Pap smear described intensely hemorrhagic smear and high-grade squamous intraepithelial lesions with severe dysplasia (CIN III). Transvaginal ultrasound and MRI of the pelvis characterized an increase in the dimensions of the cervix, displaying aspect "barrel" due process of expansive diffuse infiltrative appearance. The histopathological and immunohistochemistry of the cervical biopsy showed a diffuse non-Hodgkin's lymphoma large B cell. (AU)

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.

Early stage primary gastric diffuse large B-cell lymphoma in a young HIV-positive patient.

HIV infection is known to be associated with the development of a wide range of neoplasia. About 25 to 40% of HIV-positive patients will present some kind of malignancy in the course of the disease; among them 10% are non-Hodgkin lymphomas (NHL) and 20% of these are represented by the diffuse large B-cell lymphoma. HIV-positive patients have a relative risk of 110 times higher to develop neoplasia, than the non-infected population. The gastrointestinal (GI) tract is the most frequent extranodal site of involvement. However, the primary GI lymphoma is rare. The authors present a case of a 31-year-old male patient with a 16-year history of HIV infection, who deliberately withdrew the Highly Active Antiretroviral Therapy (HAART) regimen and was hospitalized because of a respiratory infection. Because of a long-term complaint of dyspepsia, an upper gastrointestinal endoscopy was performed disclosing a large elevated and ulcerated gastric lesion, which biopsy revealed a diffuse large B-cell lymphoma. Clinical, imaging and laboratory tests showed an early stage diagnosis: Lugano stage I. Although not frequent, the authors alert to considering this neoplasia in all HIV-positive patients with dyspeptic symptoms.

Secondary infiltration of the central nervous system in patients with diffuse large B-cell lymphoma.

OBJECTIVE: To investigate the incidence and risk factors of infiltration of the central nervous system after the initial treatment of diffuse large B-cell lymphoma in patients treated at Santa Casa de Misericórdia de São Paulo. METHODS: A total of 133 patients treated for diffuse large B-cell lymphoma from January 2001 to April 2008 were retrospectively analyzed in respect to the incidence and risk factors of secondary central nervous system involvement of lymphoma. Intrathecal prophylaxis was not a standard procedure for patients considered to be at risk. This analysis includes patients whether they received rituximab as first-line treatment or not. RESULTS: Nine of 133 (6.7%) patients developed central nervous system disease after a mean observation time of 29 months. The median time to relapse or progression was 7.9 months after diagnosis and all but one patient died despite the treatment administered. Twenty-six (19.5%) patients of this cohort received rituximab as first-line treatment and nine (7.1%) received intrathecal chemoprophylaxis. Of the nine patients that relapsed, seven (77.7%) had parenchymal central nervous system involvement; seven (77.7%) had stage III or IV disease; one (11.1%) had bone marrow involvement; two (22.2%) had received intrathecal chemoprophylaxis; and 3 (33.3%) had taken rituximab. In a multivariate analysis, the risk factors for this infiltration were being male, previous use of intrathecal chemotherapy and patients that were refractory to initial treatment. CONCLUSION: Central nervous system infiltration in this cohort is similar to that of previous reports in the literature. As this was a small cohort with a rare event, only three risk factors were important for this infiltration.

The utility of lactate dehydrogenase in the follow up of patients with diffuse large B-cell lymphoma.

BACKGROUND: Serum lactate dehydrogenase is a non-specific marker for lymphoma whose prognostic significance is well established for both indolent and aggressive lymphomas at the time of diagnosis. The performance characteristics of this enzyme in predicting relapse in patients with diffuse large B-cell lymphoma has not been well studied. METHODS: This study compared serum lactate dehydrogenase levels in 27 patients with diffuse large B-cell lymphoma who relapsed after sustaining a complete response versus 87 patients who did not relapse. For relapsed patients, the serum lactate dehydrogenase level at relapse was compared with the level three months before (considered baseline). For non-relapsed patients, the last two levels during follow-up were compared. For statistical analysis the T-test was used to compare differences in mean values between groups. The sensitivity, specificity, positive and negative predictive values for serum lactate dehydrogenase in detecting relapse compared to confirmatory imaging were calculated. RESULTS: At relapse, only 33% patients had increases in serum lactate dehydrogenase above the upper limit of normal. The mean increase was 1.2-fold above the upper limit of normal for relapsed vs. 0.83 for those who did not relapse (p-value = 0.59). The mean increase in serum lactate dehydrogenase, from baseline, was 1.1-fold in non-relapsed vs. 1.3 in relapsed patients (p-value = 0.3). The likelihood ratio of relapse was 4.65 for patients who had 1.5-fold increases in serum lactate dehydrogenase above baseline (p-value = 0.03). The sensitivity, specificity, positive and negative predictive values of 1.5-fold increases for detecting relapse, compared to clinical and imaging findings were 0.18, 0.95, 0.55, and 0.79, respectively. CONCLUSION: A 1.5-fold increase in serum lactate dehydrogenase, over a period of 3 months, is associated with increased likelihood of relapse from diffuse large B-cell lymphoma.

The utility of lactate dehydrogenase in the follow up of patients with diffuse large B-cell lymphoma

Background: Serum lactate dehydrogenase is a non-specific marker for lymphoma whose prognostic significance is well established for both indolent and aggressive lymphomas at the time of diagnosis. The performance characteristics of this enzyme in predicting relapse in patients with diffuse large B-cell lymphoma has not been well studied. Methods: This study compared serum lactate dehydrogenase levels in 27 patients with diffuse large B-cell lymphoma who relapsed after sustaining a complete response versus 87 patients who did not relapse. For relapsed patients, the serum lactate dehydrogenase level at relapse was compared with the level three months before (considered baseline). For non-relapsed patients, the last two levels during follow-up were compared. For statistical analysis the T-test was used to compare differences in mean values between groups. The sensitivity, specificity, positive and negative predictive values for serum lactate dehydrogenase in detecting relapse compared to confirmatory imaging were calculated. Results: At relapse, only 33% patients had increases in serum lactate dehydrogenase above the upper limit of normal. The mean increase was 1.2-fold above the upper limit of normal for relapsed vs. 0.83 for those who did not relapse (p-value = 0.59). The mean increase in serum lactate dehydrogenase, from baseline, was 1.1-fold in non-relapsed vs. 1.3 in relapsed patients (p-value = 0.3). The likelihood ratio of relapse was 4.65 for patients who had 1.5-fold increases in serum ...