Current knowledge on HIV-associated Plasmablastic Lymphoma

HIV-associated PBL is an AIDS-defining cancer, classified by WHO as a distinct entity of aggressive DLBCL. To date less than 250 cases have been published, of them 17 are pediatric. The pathogenesis of this rare disease is related to immunodeficiency, chronic immune stimulation and EBV. Clinically is a rapid growing destructive disease mainly involving the oral cavity even if extraoral and extranodal sites are not infrequent. The diagnosis requires tissue mass or lymph node biopsy and core needle or fine needle biopsy is acceptable only for difficult access sites. Classically immunophenotype is CD45, CD20, CD79a negative and CD38, CD138, MUM1 positive, EBER and KI67 is >80%. Regarding the therapy, standard treatment is, usually, CHOP or CHOP-like regimens while more intensive regimens as CODOX-M/IVAC or DA-EPOCH are possible options. Use of cART is recommended during chemotherapy, keeping in mind the possible overlapping toxicities. Rituximab is not useful for this CD20 negative disease and CNS prophylaxis is mandatory. Intensification with ABMT in CR1 may be considered for fit patients. For refractory/relapsed patients, therapy is, usually, considered palliative, however, in chemo-sensitive disease, intensification + ABMT or new drugs as Bortezomib may be considered. Factors affecting outcome are achieving complete remission, PS, clinical stage, MYC, IPI score. Reported median PFS ranges between 6–7 months and median OS ranges between 11–13 months. Long term survivors are reported but mostly in pediatric patients. Finally, due to the scarcity of data on this subtype of NHL we suggest that the diagnosis and the management of HIV-positive PBL patients should be performed in specialized centers

Immunophenotypical Switch versus Tumor Heterogeneity in a Patient with HIV-Associated Diffuse Large B-Cell Lymphoma

Patients with HIV/AIDS have a higher risk of developing aggressive B-cell lymphomas, such as diffuse large B-cell lymphoma (DLBCL). Lymphomas are rather heterogeneous in nature and in a few cases can switch their genetic or immunohistochemical phenotype, transform into other lymphomas or carry more than one malignant clone. In this report, we present the case of a 47-year-old man with HIV infection who was diagnosed with an apparent low-risk, early-stage DLBCL, but became refractory to therapy while undergoing treatment with rituximab-containing chemotherapy. We postulate that the development of his refractory disease occurred in the context of an immunohistochemical switch or the surge of a clone refractory to therapy. This phenomenon was not associated with a superinfection with EBV or HHV-8

The biology and treatment of plasmablastic lymphoma

AbstractPlasmablastic lymphoma (PBL) is an aggressive lymphoma commonly associated with HIV infection. However, PBL can also be seen in patients with other immunodeficiencies as well as in immunocompetent individuals. Because of its distinct clinical and pathological features, such as lack of expression of CD20, plasmablastic morphology, and clinical course characterized by early relapses and subsequent chemotherapy resistance, PBL can represent a diagnostic and therapeutic challenge for pathologists and clinicians alike. Despite the recent advances in the therapy of HIV-associated and aggressive lymphomas, patients with PBL for the most part have poor outcomes. The objectives of this review are to summarize the current knowledge on the epidemiology, biology, clinical and pathological characteristics, differential diagnosis, therapy, prognostic factors, outcomes, and potential novel therapeutic approaches in patients with PBL and also to increase the awareness toward PBL in the medical community

LINFOMA DE CÉLULAS GRANDES B DIFUSO, EPSTEIN-BARR VIRUS POSITIVO

El Linfoma de células grandes B difuso EBV positivo es una entidad descrita el 2008 en la clasificación de la WHO y que ha sufrido cambios hasta la fecha. Corresponde del 2 al 15% de todos los Linfomas de células grandes B difuso, con variación de acuerdo a la localización geográfica. Afecta a jóvenes y adultos. Inicialmente reconocida como una entidad agresiva con pobre sobrevida, hoy se conoce su alta sensibilidad al rituximab. El pronóstico mejora notablemente cuando el tratamiento está basado en inmunoquimioterapia. DOI: https://doi.org/10.25176/RFMH.v17.n3.119

HIGHLAND AVIAN SURVEYS IN CERRO HOYA NATIONAL PARK (AZUERO, PANAMÁ) REVEAL NEW RANGE EXTENSIONS, INCLUDING A RARE HUMMINGBIRD (SELASPHORUS SP.)

Abstract ∙ Cerro Hoya National Park in Panama (CHNP) is one of the least explored protected areas in southern Mesoamerica. It houses the greatest ecosystem diversity in the degraded Azuero Peninsula, but its inaccessibility hinders expeditions and scientific research. Avian richness in CHNP was previously estimated by combining data from five ornithological expeditions at ca. 225 species. However, the highest altitude areas remained relatively unexplored and some historical records were not verified. We intensively surveyed highlands and foothills of CHNP and identified five new range extensions. One of these included a Selasphorus sp., of which we collected three male (two adults, one immature) and one female specimens. We compared morphology and plumage color patterns of these with closely related species in detail and confirm their likely classification with the endangered Glow-throated Hummingbird (Selasphorus ardens). This species is rare, with few documented sightings, confirmed specimens, and no unambiguous female museum specimen until now. We also confirmed historical records and provide 26 unlisted species (one by an external source) to CHNP. Our results confirm that Cerro Hoya is a highly biodiverse skyisland of Mesoamerica. We urge improved enforcement of its protection in combination with further studies of the ecology and evolutionary processes in this unique region. Resumen · Censos ornitológicos en ambientes de altura en el Parque Nacional Cerro Hoya (Azuero, Panamá) revelan nuevas extensiones de distribución, incluyendo un inusual colibrí (Selasphorus sp.) El Parque Nacional Cerro Hoya (PNCH) es una de las áreas protegidas menos exploradas de Panamá y el sur de Mesoamérica. Este alberga la mayor diversidad de ecosistemas en la degradada península de Azuero, pero su inaccesibilidad dificulta la investigación científica. Combinando datos de cinco expediciones ornitológicas, se estimó una riqueza aviar de alrededor de 225 especies. No obstante, las áreas de mayor altitud permanecieron relativamente inexploradas y algunos registros históricos no fueron verificados. Exploramos intensivamente las tierras altas y estribaciones del PNCH e identificamos cinco nuevas extensiones de distribución. Uno de estos incluye Selasphorus sp., del cual colectamos tres especímenes machos (dos adultos, un juvenil) y una hembra. Comparamos detalladamente la morfología y los patrones de coloración del plumaje con especímenes de especies relacionadas estrechamente. Confirmamos que estos individuos podrían pertenecer al amenazado Colibrí Ardiente (Selasphorus ardens). Esta especie es rara, con pocos avistamientos y especímenes de museo con información inconsistente, principalmente en hembras. También confirmamos los registros históricos y agregamos 26 especies no listadas para el PNCH (una obtenida por fuente externa). Nuestros resultados confirman que Cerro Hoya es una isla-cielo de gran biodiversidad en Mesoamérica. Es urgente mejorar la aplicación de su protección, así como estudiar la ecología y los procesos evolutivos en esta región única

Plasmablastic Lymphoma: A Systematic Review

Plasmablastic lymphoma (PBL) is a very aggressive variant of diffuse large B-cell lymphoma initially described in the oral cavity of HIV-infected individuals. PBL represents a diagnostic challenge given its characteristic morphology and lack of CD20 expression, and also a therapeutic challenge, with early responses to therapy, but with high relapse rates and poor prognosis. In recent years, our understanding and clinical experience with PBL has increased in both HIV-positive and -negative settings. However, given its rarity, most of the data available rely on case reports and case series. The main goal of this article is to systematically review the most recent advances in epidemiology; pathophysiology; clinical, pathologic, and molecular characteristics; therapy; and prognosis in patients with PBL. Specific covered topics include new pathological markers for diagnosis, its association with Epstein-Barr virus, and the need of more intensive therapies

Malignant pheochromocytoma treated with minimally invasive surgery

Indexación: Web of Science; Scielo.Introducción: El Feocromocitoma es un tumor de células cromafines productor de catecolaminas. Su variedad maligna es muy poco frecuente. Objetivo: Describir 2 casos de Feocromocitoma maligno tratados con cirugía mínimamente invasiva. Casos clínicos: Presentamos 2 pacientes de sexo femenino, de 31 y 51 años respectivamente. La primera con historia de hipertensión arterial de larga data y uso de múltiples fármacos. La segunda paciente debuta con dolor abdominal. En ambos casos los estudios radiológicos y metabólicos confirmaron el diagnóstico de Feocromocitoma. A la primera paciente se le realizó un procedimiento laparoscópico puro, mientras que a la segunda paciente se le realizó un procedimiento laparoscópico asistido por robot. En ambos casos fue necesario realizar adrenalectomía en bloque con el riñón, por compromiso del hilio renal. No hubo complicaciones severas intra ni post-operatorias, como tampoco conversión a cirugía abierta. La histopatología confirmó el diagnóstico de Feocromocitoma maligno con metástasis ganglionares regionales y márgenes quirúrgicos negativos. Ambas se mantienen en control, sin evidencia de recurrencia. Conclusión: El Feocromocitoma maligno puede ser manejado de forma mínimamente invasiva, con morbilidad y resultados oncológicos comparables a cirugía abierta.Introduction: Pheochromocytoma is a tumor derived from chromaffin cells that produces cathecolamines. Malignant pheochromocytomas are extremely rare. Aim: To describe 2 cases of malignant Pheochromocytoma treated with pure laparoscopic and robot assisted surgery. Clinical cases: Two female patients, 31 and 51 years of age, presented with a history of high blood pressure and abdominal pain. Radiologic and metabolic studies confirmed the diagnosis of Pheochromocytoma in both patients. A pure laparoscopic approach was performed in the first patient while a robotic assisted laparoscopy was done in the second patient. In both cases, adrenalectomy with in bloc nephrectomy was performed due to compromise of the renal sinus. There were no complications or conversion to open surgery. Histopathology confirmed the diagnosis of malignant Pheochromocytoma with metastasis to regional nodes and negative surgical margins. At follow-up, there is no evidence of recurrence. Conclusion: Malignant Pheochromocytoma can be treated with minimally invasive surgery, with morbidity and oncological outcomes similar to open approach.http://ref.scielo.org/ksq86

Nutritional status and clinical outcome of children on continuous renal replacement therapy: a prospective observational study

BACKGROUND: No studies on continuous renal replacement therapy (CRRT) have analyzed nutritional status in children. The objective of this study was to assess the association between mortality and nutritional status of children receiving CRRT. METHODS: Prospective observational study to analyze the nutritional status of children receiving CRRT and its association with mortality. The variables recorded were age, weight, sex, diagnosis, albumin, creatinine, urea, uric acid, severity of illness scores, CRRT-related complications, duration of admission to the pediatric intensive care unit, and mortality. RESULTS: The sample comprised 174 critically ill children on CRRT. The median weight of the patients was 10 kg, 35% were under percentile (P) 3, and 56% had a weight/P50 ratio of less than 0.85. Only two patients were above P95. The mean age for patients under P3 was significantly lower than that of the other patients (p = 0.03). The incidence of weight under P3 was greater in younger children (p = 0.007) and in cardiac patients and in those who had previous chronic renal insufficiency (p = 0.047). The mortality analysis did not include patients with pre-existing renal disease. Mortality was 38.9%. Mortality for patients with weight < P3 was greater than that of children with weight > P3 (51% vs 33%; p = 0.037). In the univariate and multivariate logistic regression analyses, the only factor associated with mortality was protein-energy wasting (malnutrition) (OR, 2.11; 95% CI, 1.067-4.173; p = 0.032). CONCLUSIONS: The frequency of protein-energy wasting in children who require CRRT is high, and the frequency of obesity is low. Protein-energy wasting is more frequent in children with previous end-stage renal disease and heart disease. Underweight children present a higher mortality rate than patients with normal body weight