Enfermedad de Castleman multicéntrica en pacientes HIV positivos. Reporte de dos casos y revisión de la literatura / Multicentric Astleman disease in HIV positive patients. Two cases. Report and literature review

La enfermedad de Castleman (EC) es un proceso linfoproliferativo poco frecuente que se caracteriza por hiperplasia de los ganglios linfáticos. Existen dos variedades histológicas bien diferenciadas la hialino-vascular y la plasmocelular, que a su vez pueden ser localizadas o multicéntricas. La forma hialino-vascular suele ser asintomática y localizada en mediastino mientras que la plasmocelular se presenta frecuentemente con signo-sintomatología sistémica y suele ser difusa o multicéntrica. En el contexto de la enfermedad debida al virus de la inmunodeficiencia humana (VIH), la EC se asocia en su patogenia a la infección por el herpes virus humano tipo-8 (HHV-8). La mayoría de los casos corresponden a la variante hialino-vascular (80/90%) en tanto un pequeño porcentaje (10/20%) son de la variante plasmocelular. En algunos pacientes, el patrón histopatológico puede ser mixto. Se describen dos casos de enfermedad de Castleman multicéntrica HHV8- positiva en pacientes con enfermedad HIV/SIDA.

Castleman's disease (CD), is a rare hematological condition of uncertain etiology, involves a massive proliferation of lymphoid tissues and typically presents as mediastinal masses. This is considered as a distinct type of lymphoproliferative disorder associated with inflammatory symptoms. In the context of human immunodeficiency virus (HIV) infection, CD is associated with human herpesvirus-8 (HHV8) infection. Most cases of CD represent either the hyaline vascular variant (80­90% of cases) or the plasma cell variant (10­20%); a small percentage present with a mixed histologic appearance. Two cases of Castleman's disease associated with HHV-8 and HIV/AIDS infection are described

Sarcoma de Kaposi en pacientes que viven con el VIH en Guinea Ecuatorial / Kaposi sarcoma in patients living with HIV in Equatorial Guinea

Introducción: El sarcoma de Kaposi es una neoplasia oportunista asociada a la inmunodepresión causada por VIH, que se relaciona con la infección por VHH tipo 8. Objetivo: Describir la presentación del sarcoma de Kaposi en personas que viven con VIH en Guinea Ecuatorial. Métodos: Se realizó un estudio descriptivo de carácter retrospectivo para identificar la prevalencia y las características epidemiológicas y clínicas del sarcoma de Kaposi en las personas que viven con VIH que acuden a las unidades de referencia para el manejo de casos en Guinea Ecuatorial. Se revisaron las historias clínicas de una muestra aleatoria y representativa de 338 pacientes del grupo que ha recibido tratamiento en las unidades de referencia para enfermedades infecciosas de Bata, desde enero de 2007 a febrero de 2012. Resultados: Se identificaron 40 pacientes diagnosticados de sarcoma de Kaposi (prevalencia del 11, 83 por ciento). La mediana de la edad al diagnóstico de sarcoma de Kaposi fue de 43 años, siendo la ratio del sexo de 1/1. La media de linfocitos CD4 al diagnóstico fue de 166 (rango 21-375) y la frecuencia de afectación oral fue de 45 por ciento. En la mayoría de los pacientes (94,6 por ciento) la observación del sarcoma de Kaposi fue anterior al inicio del tratamiento antirretroviral. Las cifras de linfocitos T CD4/mm3 inferiores a 100 aparecían sobre todo en pacientes menores de 30 años, y esto era especialmente frecuente en el grupo de mujeres (OR 11, p <0,04, Ic 95 por ciento 0,8-148). Conclusiones: El sarcoma de Kaposi es una neoplasia prevalente en personas que viven con VIH seguidas en las unidades de referencia en Guinea Ecuatorial. En mujeres menores de 30 años podría existir un diagnóstico tardío(AU)

Introduction: Kaposi sarcoma is an opportunistic neoplasm associated to the immunosuppression caused by HIV and related to infection by HHV-8. Objective: Describe the presentation of Kaposi sarcoma in people living with HIV in Equatorial Guinea. Methods: A retrospective descriptive study was conducted to identify the prevalence and the clinical and epidemiological characteristics of Kaposi sarcoma in people living with HIV attending reference units for the management of cases in Equatorial Guinea. A review was carried out of the medical records of a random sample representative of 338 patients from the group receiving treatment at Bata reference unit for infectious diseases from January 2007 to February 2012. Results: A total 40 patients diagnosed with Kaposi sarcoma were identified (prevalence of 11,83 percent). Mean age at Kaposi sarcoma diagnosis was 43 years, with a 1/1 sex ratio. The mean CD4 lymphocyte count at diagnosis was 166 (range 21-375), whereas the frequency of oral damage was 45 percent. In most patients (94.6 percent) detection of Kaposi sarcoma was prior to the start of antiretroviral therapy. CD4 T lymphocyte levels / mm3 below 100 were mainly found in patients aged under 30 years, a fact particularly frequent among women (OR 11, p< 0.04, CI 95% 0.8-148). Conclusions: Kaposi sarcoma is a neoplasm prevailing in people living with HIV who attend reference units in Equatorial Guinea. Late diagnosis could exist among women aged under 30 years(AU)

Sarcoma de Kaposi ganglionar postrasplante renal: reporte de dos casos en un centro de trasplante colombiano Kaposi sarcoma in lymph nodes after kidney transplantation: report of two cases in a Colombian transplant center / Sarcoma de Kaposi ganglionar postrasplante renal: reporte de dos casos en un centro de trasplante colombiano Kaposi sarcoma in lymph nodes after kidney transplantation: report of two cases in a Colombian transplant center

El cáncer es una causa importante de morbilidad y mortalidad en los receptores de trasplante. La combinación de infecciones virales, terapia de inmunosupresión y la alteración en el sistema inmune en los pacientes trasplantados, contribuyen al desarrollo de cáncer. El sarcoma de Kaposi es causado por el virus herpes humano 8 (VHH-8), y aunque es raro en la población general, puede ser hasta 300 veces más frecuente en los pacientes con trasplante renal. El diagnóstico de la enfermedad se realiza a menudo con base en las características de las lesiones, pero debe ser confirmado por histología. En años recientes, los inhibidores de mTOR han mostrado ser efectivos para el control del sarcoma de Kaposi en los pacientes trasplantados, ya que se interrumpe el efecto antiapoptótico y la angiogénesis dependientes de la proteína mTOR, los cuales son esenciales para el desarrollo y la propagación de células malignas. Se presentan dos casos de pacientes con sarcoma de Kaposi ganglionar, sin lesiones en piel, en nuestro centro de trasplante, quienes respondieron de manera positiva al cambio del esquema inmunosupresor con inhibidores de mTOR

Cancer is a major cause of morbidity and mortality in transplant recipients. The combination of viral infections, immunosuppression therapy and immune system dysfunction in transplant patients contribute to the development of cancer. Kaposi sarcoma is caused by human herpes virus 8 (HHV-8) and although rare in the general population, it is reported to be up to 300 times more common in kidney transplant patients. Diagnosis of the disease is often made on the basis of the characteristic appearance of lesions, but must be confirmed by histology. In recent years, mTOR inhibitors have been shown to be effective in controlling Kaposi sarcoma in transplant patients, due to disruption of the antiapoptotic effect and angiogenesis dependent on the mTOR protein, which are essential for development and propagation of malignant cells. We present two case reports of patients with Kaposi sarcoma in lymph nodes and no skin lesions, who responded well to the immunosuppressive therapy switch with mTOR inhibitors

Case for diagnosis. Penile lesion in HIV-negative patient

Abstract We present the case of an HIV-negative man with asymptomatic penile erythematoviolaceous papules associated with similar slightly verrucous papules in the interdigital space of the right foot. A biopsy of the penile lesion confirmed Kaposi's sarcoma. No other causes of immunosuppression were observed. Penile lesions of KS are rare in HIV-negative individuals but it should also be considered in the differential diagnosis. Careful follow-up is recommended.

Two cases of rare HHV8-driven intravascular lymphoma with synchronous Kaposi sarcoma, both diagnosed at autopsy in renal transplant recipients

We present the first report of two rare yet remarkably similar autopsy cases of Kaposi sarcoma (KS) and intravascular human herpesvirus 8 (HHV8) positive lymphoproliferative disorder in renal transplant patients. It is well established that HHV8 infection causes Kaposi sarcoma (KS). More recently, it is recognized that HHV8 is also related to several lymphoproliferative conditions. These are poorly characterized and often difficult to diagnose. In both cases described herein, the diagnoses of multifocal hepatic KS and intravascular HHV8 positive (EBV negative) systemic diffuse large B-cell lymphoma, NOS were made at autopsy. Given the findings we describe in cases with fatal outcomes, we discuss the implications of HHV8 screening in solid allograft recipients.

Distribution of dendritic cells in aids-associated oral kaposi's sarcoma

Abstract The aim of this study was to determine if the distribution of Langerhans cells (LC) and interstitial dendritic cells (IDC) is altered in AIDS-associated oral Kaposi's sarcoma when compared to HIV-negative highly vascular oral lesions. Fifty-one cases of AIDS-associated oral Kaposi's sarcoma and 20 of highly vascular oral lesions were retrospectively retrieved. All cases of Kaposi's sarcoma were confirmed with immunoreactions against CD34 and HHV-8. Clinical data regarding sex, age and lesions location were obtained from pathology reports. Immunohistochemistry against CD207 (immature dendritic cells) and CD83 (mature dendritic cells) were done. LC were in the epithelium and IDC in the stroma. CD207+ cells predominated in the epithelium of the lesions, whereas CD83+ cells predominated in their stromal compartment. Kaposi's sarcoma had a lower CD207+ immature LC count (p=0.02) and an increased CD207+ IDC than highly vascular oral lesions (p<0.001). Moreover, Kaposi's sarcoma also showed an increased number of mature CD83+ IDC than highly vascular oral lesions (p<0.001). There were significant alterations in the distribution of LC and IDC in AIDS-associated Kaposi's sarcoma when compared to HIV-negative vascular oral lesions, suggesting that changes in their concentrations may play a role in the pathogenesis of Kaposi's sarcoma.

Resumo O objetivo deste estudo foi determinar se a distribuição das células de Langerhans (CL) e das células dendríticas intersticiais (CDI) está alterada no sarcoma de Kaposi oral associado à AIDS quando comparado às lesões orais altamente vasculares HIV-negativas. 51 casos de sarcoma de Kaposi oral associado à AIDS e 20 de lesões orais altamente vasculares foram recuperados retrospectivamente. Todos os casos de sarcoma de Kaposi foram confirmados pela positividade para os anticorpos CD34 e HHV-8. Dados clínicos sobre sexo, idade e localização das lesões foram obtidos dos laudos histopatológicos. Foram realizadas imunoistoquímica contra CD207 (células dendríticas imaturas) e CD83 (células dendríticas maduras). As CL estavam presentes no epitélio enquanto as CDI estavam presentes no estroma. As células CD207+ predominaram no epitélio das lesões, enquanto as células CD83+ predominaram no estroma. O sarcoma de Kaposi teve uma contagem mais baixa de CD imaturas CD207+ (p = 0,02) e número aumentado de CDC CD207+ do que lesões orais altamente vasculares (p<0,001). Além disso, o sarcoma de Kaposi também mostrou um número aumentado de CDI CD83+ maduras do que lesões orais altamente vasculares (p<0,001). Houve alterações significativas na distribuição de CL e CDI no sarcoma de Kaposi associado à AIDS quando comparado às lesões orais vasculares HIV-negativas, sugerindo que alterações na distribuição das mesmas podem desempenhar um papel na patogênese do sarcoma de Kaposi.

Sarcoma de Kaposi epidémico en África Subsahariana / Epidemic Kaposi sarcoma in Sub-Saharan Africa

El sarcoma de Kaposi se ha convertido en uno de los tumores más prevalentes en África tras la epidemia de VIH, que afecta de una manera similar a hombres y mujeres. El retraso diagnóstico y el limitado acceso a tratamiento antirretroviral o quimioterapia condicionan el pronóstico de los pacientes que lo padecen. En este artículo se realiza una revisión sobre la referida enfermedad, con el objetivo de describir sus aspectos más relevantes en los últimos años en África, como son su epidemiología, caractéristicas clínicas y opciones terapéuticas existentes. Este tumor es provocado por la infección por virus herpes humano tipo 8, que resulta más prevalente en las zonas rurales del continente africano. Se postula la transmisión a través de la saliva como la vía más importante de contagio en África. La inmunodepresión que causa el VIH favorece el efecto oncogénico del virus. La forma epidémica de SK se manifiesta inicialmente como lesiones hiperpigmentadas o violáceas en la piel, que pueden extenderse a linfáticos o mucosas y a nivel sistémico, principalmente a pulmón o aparato digestivo. El síndrome de reconstitución inmune sistémica puede complicar la evolución del paciente. El inicio temprano de la terapia antirretroviral resulta imprescindible. Además, el pronóstico de los pacientes mejora con la suma de tratamiento quimioterápico con doxorrubicina, vincristina, etopóxido o bleomicina principalmente(AU)

Kaposi sarcoma (KS) has become one of the most prevalent tumors in Africa after the HIV epidemic. KS affects both men and women. Diagnostic delay and limited access to antiretroviral treatment or chemotherapy have an impact on the prognosis of KS patients. A review was conducted about KS with the purpose of describing its most outstanding characteristics in recent years in Africa, such as its epidemiology, clinical features, and existing therapeutic options. This tumor is caused by infection with human herpesvirus 8, which is more prevalent in rural areas of the African continent. Transmission via saliva was found to be the most important transmission route in Africa. HIV-related immunosuppression fosters the oncogenic effect of the virus. The epidemic form of KS initially presents as hyperpigmented or violet-colored skin lesions which may extend to lymph nodes or mucosae, or systemically, mainly to the lungs or the digestive tract. Systemic immune reconstitution syndrome may complicate the patient's evolution. Early start of antiretroviral therapy is indispensable. Additionally, prognosis improves with chemotherapy with doxorubicin, vincristine, etoposide or bleomycin, mainly(AU)

Kaposi sarcoma of th e penis in anHIV-negative patient / Sarcoma de Kaposi de pênis em paciente HIV negativo

ABSTRACT Kaposi sarcoma is an angioproliferative disorder that ranges from a single indolent skin lesion to respiratory and gastrointestinal/visceral involvement. Kaposi sarcoma is rare in non-immunosuppressed patients. Nineteen cases of penile Kaposi sarcoma in HIV-negative patients were reported in 2012. We present the case report of a 48-year-old male patient with no previous medical history, who came to our urology clinic presenting a purple-color papule on the penis glans. Lab tests revealed negative serology for HIV, but tissue PCR was positive for human herpesvirus 8. Histopathology examination after lesion excision was compatible with Kaposi sarcoma. No other cutaneous or mucosal lesions were present. Primary Kaposi sarcoma of the penis is rare, but may occur in non-immunosuppressed patients.

RESUMO O sarcoma de Kaposi é uma doença angioproliferativa que varia de uma lesão cutânea indolente isolada ao envolvimento visceral respiratório e gastrintestinal. É raro em pacientes não imunossuprimidos. Dezenove casos de sarcoma de Kaposi de pênis em pacientes HIV negativos foram relatados em 2012. Descrevemos o caso de um paciente do sexo masculino, 48 anos, sem história pregressa, que se apresentou em nossa clínica urológica com pápula violeta na glande. Os testes de laboratório revelaram sorologia negativa para HIV, mas o PCR em tecido foi positivo para o herpesvírus humano 8. A histopatologia após a excisão da lesão foi compatível com sarcoma de Kaposi. Não existia outra lesão cutânea ou de mucosa. O sarcoma de Kaposi primário de pênis é raro, mas pode ocorrer em pacientes não imunossuprimidos.

Sarcoma de Kaposi em paciente portador de HIV: relato de caso / Kaposi sarcoma in a patient with HIV: case report

Sarcoma de Kaposi é um tumor maligno originado do endotélio vascular que acomete principalmente pele e mucosas. Geralmente, é associado à síndrome da imunodeficiência adquirida ­ aids, apresentando lesões vinhosas, arredondadas que, com o passar dos dias, tornam-se purpúricas, elevadas e com distribuição multifocal. Nesse estudo é relatado o caso de um paciente do sexo masculino, de 42 anos de idade, de fototipo IV, com emagrecimento, diarreia, pápulas e placas eritemato-violáceas nos membros inferiores. Durante a internação, encontrou-se sorologia positiva para HIV e ao realizar histopatológico das lesões cutâneas, confirmou-se o diagnóstico de sarcoma de Kaposi. O objetivo do presente estudo é ressaltar que quando presente infecção pelo citomegalovírus em paciente com aids há maior predisposição para o desenvolvimento de tal neoplasia. (AU)

Kaposi's sarcoma is a malignant tumor originating from the vascular endothelium, which mainly affects the skin and mucous membranes. Generally, it is associated with acquired immunodeficiency syndrome - AIDS, presenting rounded, wine-like lesions that become purpuric, elevated, and multifocal in the course of days. In this study the case of a 42-year-old male phototype IV with weight loss, diarrhea, papules and erythematous-purple plaques in the lower limbs was reported. During the hospitalization, positive serology for HIV was found and the diagnosis of Kaposi's sarcoma was confirmed in the histopathological examination of cutaneous lesions. The objective of the present study is to highlight that when present with cytomegalovirus in a patient with AIDS there is a greater predisposition for the development of such neoplasia. (AU)

Sarcoma de Kaposi em Paciente Jovem Imunocompetente: uma Quinta Variante Epidemiológica? ­ Relato de Caso / Kaposi's Sarcoma in Young Immunocompetent Patient: a Fifth Epidemiological Variant? ­ Case Report / Sarcoma de Kaposi em Paciente Joven Inmunocompetente: ¿una Quinta Variante Epidemiológica? ­ Informe de Caso

Introdução: O sarcoma de Kaposi apresenta, classicamente, quatro tipos de variantes: clássico, endêmico, associado à imunossupressão (ou iatrogênico) e epidêmico (ou relacionado à Aids). Todos esses subtipos estão relacionados ao herpes-vírus humano 8. Uma quinta variante clínico-epidemiológica vem sendo proposta na literatura, que inclui uma apresentação visceral da doença no grupo de homens que fazem sexo com homens sem fatores de imunossupressão identificados. Relato de caso: Descreve-se o caso de um paciente masculino de 24 anos de idade, de orientação homossexual, sem fatores de imunossupressão, com apresentação linfonodal de sarcoma de Kaposi, e sem outros fatores que o incluam dentro das classificações da doença atualmente conhecidas. O paciente recebeu tratamento quimioterápico com paclitaxel, atingindo resposta completa e mantida até o momento, 42 meses após o término do tratamento. Conclusão: Esse caso reforça que a patogênese do sarcoma de Kaposi ainda é pouco clara, e que provavelmente múltiplos fatores, tanto do vírus como do hospedeiro, interajam entre si para desencadear a carcinogênese. É possível que o hábito sexual não encerre relação com essa patogênese, comportando-se apenas como fator confundidor. O paciente apresentou toxicidade mínima durante o tratamento com paclitaxel e atingiu resposta completa e mantida.

Introduction: Kaposi sarcoma classically presents four types of variants: classic, endemic, immunosuppression-associated (or iatrogenic) and epidemic (or AIDS-associated). All subtypes are invariably linked to human herpesvirus-8. A fifth clinical-epidemiological variant has been proposed in the literature, which includes a visceral presentation of the disease in the group of men who have sex with men without detected immunosuppressive factors. Case Report: We report the case of a 24-year-old male patient with a homosexual orientation without immunosuppressive factors, diagnosed with KS, with lymph node involvement, and without other disease characteristics that could include him within the currently known four types of Kaposi sarcoma classification. The patient received chemotherapy with paclitaxel, evolving with complete and sustained reponse until now, 42 months after the ending of treatment. Conclusion: This case reinforces that the pathogenesis of KS is still unclear, and that probably multiple factors, both virus and host, interact with each other to trigger carcinogenesis. It is possible that the sexual habit does not influence this pathogenesis, behaving only as a confounding factor. The patient had minimal toxicity during treatment with paclitaxel and achieved a complete and sustained response.

Introducción: El sarcoma de Kaposi presenta, clásicamente, cuatro tipos de variantes: clásico, endémico, asociado a inmunosupresión (o iatrogénico) y epidémico (o relacionado a SIDA). Todos estos subtipos están relacionados con el virus del herpes humano tipo 8 (HHV-8). Una quinta variante clínica-epidemiológica está en estudio, incluye una presentación visceral de la enfermedad en un grupo de hombres que tienen sexo con hombres sin factores de inmunosupresión de causa detectada. Informe de Caso: Se desarrolló un estudio sobre un paciente masculino de 24 años de edad, de orientación homosexual, sin factores de inmunosupresión, presentando diagnóstico de sarcoma de Kaposi con característica linfonodal de la enfermedad, y sin otros factores que incluyan dentro de las clasificaciones de sarcoma de Kaposi actualmente conocidas. El paciente recibió tratamiento quimioterápico con paclitaxel, alcanzando respuesta completa y sostenida hasta el momento, 42 meses después del término del tratamiento. Conclusión: Este caso refuerza que la patogénesis del sarcoma de Kaposi es poco clara, y que probablemente múltiples factores, tanto del virus y del hospedador, interactúan entre sí para desencadenar La carcinogénesis. Es posible que el hábito sexual no encierre relación con esa patogénesis, comportándose apenas como factor confundidor. El paciente presentó toxicidad mínima durante el tratamiento y alcanzó una respuesta completa y sostenida hasta el momento, pudiendo, el paclitaxel, ser considerado una opción sólida para el tratamiento del sarcoma de Kaposi en ese grupo de pacientes con esa variante de presentación.

Sarcoma de Kaposi clásico en paciente fototipo VI. Primer reporte en Colombia / Classic Kaposi's sarcoma in phototype VI patient. First report in Colombia

Resumen El sarcoma de Kaposi es un tumor vascular de bajo grado, que afecta piel y mucosas, pudiendo comprometer ganglios linfáticos y órganos internos. Generalmente está asociado a infección por virus herpes humano 8, se clasifica en cuatro subtipos clínico-epidemiológicos, entre ellos el tipo clásico, que se manifiesta habitualmente en ancianos blancos, inmunocompetentes, de origen judío o mediterráneo. Se presenta el caso de un varón de 69 años, con fototipo VI, que desarrolló inicialmente máculas violáceas, eritematosas, de borde definido en región externa del pie izquierdo, que progresivamente se tornaron a placas y nódulos violáceos, acompañado de edema. Se realizó biopsia que mostró lesión vascular, cuya inmunohistoquímica presentó reactividad para CD30, CD34 y virus herpes humano 8, confirmando un sarcoma de Kaposi en fase de nódulo. Este caso resulta excepcional y se debe tener presente en el diagnóstico diferencial; los reportes en Suramérica son escasos, siendo este el primero en Colombia. MÉD.UIS. 2017;30(3):129-33.

Abstract Kaposi's sarcoma is a low-grade vascular tumor that affects the skin and mucous membranes and may compromise lymph nodes and internal organs. It is usually associated with human herpes virus infection 8, classified into four clinical-epidemiological subtypes, including the classical type, which is usually manifested in elderly, immunocompetent white Jews or of Mediterranean origin. We present the case of a 69-year-old male, with phototype VI, who initially developed violaceous macules, erythematous, with defined border in the external region of the left foot, which progressively turned to violet plaques and nodules, accompanied by edema. A biopsy was performed showing vascular lesion, whose immunohistochemistry presented reactivity for CD30, CD34 and human herpesvirus 8, confirming a Kaposi's sarcoma in the nodule phase. This case is exceptional and must be kept in mind in the differential diagnosis; the reports in South America are scarce, being the first in Colombia. MÉD.UIS. 2017;30(3):129-33.

Enfermedad de Castleman multicéntrica e infección por virus Epstein Barr en paciente con infección por vih / Castleman disease and infection of Epstein Barr virus in a patient with HIV infection

La enfermedad de Castleman (ECM) es un desorden linfoproliferativo y con un pronóstico desfavorable, que se lo ha asociado a la infección por el virus herpes humano tipo 8 (HHV-8). El mecanismo supuesto de ac-ción del HHV-8 sería la secreción de interleuquinas (IL) virales, homólo-gas a IL humanas tales como IL-6 e IL-10. La coinfección por HHV-8 y vi-rus Epstein Barr (EBV) es bien conocida en pacientes con infección por el virus de la inmunodeficiencia humana tipo 1 (HIV-1). Estos gamma-herpesvirus se asocian a trastornos linfoproliferativos que son favore-cidos por la inmunosupresión. Puede presentarse en el contexto de un buen estado inmunológico y su tratamiento se basa en el uso de quimioterapia. La reactivación del EBV latente es favorecida por la infección por HIV-1 y el uso de drogas inmunosupresoras, determinando un potencial riesgo para el desarrollo de nuevos desór-denes linfoproliferativos. Se presenta el caso de un paciente con enferme-dad de Castleman asociado a la infección por HIV-1 y HHV-8, que presentó como complicación post-qui-mioterapia reactivación de infección EBV. Se realizó una revisión del concepto de ECM y la relación pato-génica entre los virus HIV-1, HHV-8 y EBV

Castleman's disease (CD) is a lymphoproliferative disorder with an unfavorable prognosis, which has been associated with human herpes virus 8 (HHV-8) infection. The presumed mechanism of action of HHV-8 would be the secretion of viral interleukin (IL), homologous to human IL such as IL-6 and IL-10. The co-HHV8 infection and Epstein Barr virus (EBV) is well known in patients infected with the HIV type 1 (HIV-1). These gamma-herpesvirus were associated with lymphoproliferative disorders that are favored by immunosuppression. It can occur in the context of a patient's good immune system and its treatment is based on the use of chemotherapy. Reactivation of latent EBV is favored by HIV-1 and the use of immunosuppressive drugs, determining a potential risk for the development of new lymphoproliferative disorders.We report here a patient with Castleman's disease associated with HIV-1 and HHV-8, who presented, as a complication of the chemotherapy, the reactivation of EBV infection. A review of the concept of CD and the pathogenic relationship between HIV-1, HHV-8 and EBV virus was performed

Sarcoma de Kaposi no trato gastrintestinal alto em paciente infectado por HIV / Upper gastrointestinal Kaposi's sarcoma in HIV-infected patient

Introdução: o Sarcoma de Kaposi (SK) é a neoplasia mais comum em portadores da Síndrome da Imunodeficiência Adquirida (SIDA). É raro o acometimento exclusivo do trato gastrointestinal por essa afecção, ocorrendo em 3,5% dos casos. O objetivo desse estudo é relatar o caso de um paciente portador de SIDA com anemia e vômitos, diagnosticado com SK gastrointestinal. Relato de caso: paciente do gênero masculino, 29 anos, foi admitido no Hospital Universitário (HU/UFPI) devido a sintomas inespecíficos com 1 mês de evolução. Paciente tinha antecedente pessoal de retrovirose. Para investigação dos sintomas, o paciente realizou endoscopia digestiva alta que revelou lesões violáceas sésseis de antro gástrico e duodeno. A análise histopatológica constatou fragmentos de mucosa gástrica e entérica exibindo, em lâmina própria, proliferação de capilares sanguíneos em fenda, células fusiformes atípicas com núcleos hipercromáticos e extravasamento de hemácias. O estudo imuno-histoquímico mostrou que as células neoplásicas expressaram o antígeno nuclear latente-1 do herpesvírus humano 8 (HHV8 LNA1; clone LN53, diluição 1:25, Diagnostic Biosystems, Pleasanton, CA) em padrão nuclear, confirmando o diagnóstico de SK. Em decorrência de múltiplas complicações e agravamento clínico, o paciente foi a óbito após 15 dias da admissão. Discussão e Conclusão: incidência de SK gastrointestinal é subestimada. Estômago, duodeno e o trato biliar são os locais mais comumente acometidos. A detecção do HHV-8 por imuno-histoquímica é uma ferramenta útil, especialmente na lesão inicial em que o caráter neoplásico não é evidente. A mortalidade dos pacientes com SK gastrointestinal exclusivo é significantemente maior em relação aos que não têm esse tipo de acometimento. Tal fato pode explicar o prognóstico reservado do paciente relatado no presente caso.

Introduction: Kaposi sarcoma (KS) is the most common malignancy in patients with Acquired Immunodeficiency Syndrome (AIDS). It is rare the exclusive involvement of the gastrointestinal tract by this condition, occurring in 3.5% of cases. The aim of this study is to report the case of an HIV-infected patient with anemia and vomiting diagnosed with gastrointestinal KS. Case report: a 29-year-old male patient admitted to the University Hospital (HU/UFPI) due to non-specific symptoms with one month of evolution. He had personal history of retrovirus. To research the symptoms, the patient underwent upper endoscopy that revealed sessile purplish lesions of gastric antrum and duodenum. Histopathological analysis found gastric and enteric mucosa fragments exhibiting in lamina propria proliferation of blood capillaries with formation of slit like spaces, atypical spindle cells with hyperchromatic nuclei and extravasation of red blood cells. Immunohistochemical study showed that tumor cells expressed human herpesvirus 8 latent nuclear antigen-1 (HHV-8 LNA1; clone LN53, dilution 1:25, Diagnostic Biosystems, Pleasanton, CA) in nuclear pattern, confirming the diagnosis of KS. Because of multiple complications and clinical worsening, the patient died after 15 days of admission. Discussion and Conclusion: the incidence of gastrointestinal KS is underestimated. Stomach, duodenum and biliary tract are the most commonly affected sites. The detection of HHV-8 for immunohistochemistry is a useful tool, particularly in the initial lesion in which the neoplastic character is not evident. The mortality of patients with exclusive gastrointestinal KS is significantly higher than those who do not have that involvement. This may explain the poor prognosis of the patient reported in this case.

Atypical Kaposi's sarcoma in young inmunocompetent patient

Abstract: Kaposi´s sarcoma is a rare tumor associated with human herpes virus 8 (HHV-8) infection. Four main clinical subtypes have been described. This study reports on a form of KS in an HIV negative and immunocompetent middle-aged man. The only remarkable factor is that he has sex with other men. This form of Kaposi´s sarcoma is rare. It occurs more in younger patients than in the classic form, is limited to the skin, and is associated with a good prognosis. The means of transmission of the virus is through saliva in oroanal or orogenital sexual practices. Mechanisms of tumor development are still not well known. Given the possible increased number of this variant, it would be interesting to extend this study.

Angiosarcoma in HIV-negative patients is not associated with HHV-8

Abstract: BACKGROUND: Angiosarcoma is an aggressive, malignant neoplasm of vascular or lymphatic origin. Herpes virus 8 (HHV-8) is a member of the herpes family with a tropism for endothelial cells and it has been proven to induce vascular neoplasms, such as Kaposi's sarcoma. The role of HHV-8 in the pathogenesis of angiosarcoma has not been well defined. OBJECTIVE: To investigate the relationship between the presence of HHV-8 and angiosarcoma. METHODS: In this study, the team investigated the relationship between the presence of HHV-8, as determined by polymerase chain reaction, and angiosarcoma, using samples from patients with epidemic Kaposi's sarcoma as controls. RESULTS: While all control cases with epidemic Kaposi's sarcoma were positive for HHV-8, none of the angiosarcoma cases was. CONCLUSION: These findings support most previous studies that found no association between HHV-8 and angiosarcoma.

Mycosis fungoides and Kaposi's sarcoma association in an HIV-negative patient

Abstract The association of mycosis fungoides and kaposi’s sarcoma in HIV-negative patients is a rare phenomenon. The presence of human herpesvirus 8 (HHV-8) – associated with all forms of Kaposi’s sarcoma – has also been recently identified in mycosis fungoides lesions. However, a causal association between HHV-8 and the onset of mycosis fungoides has not been established yet. The present case reports a patient who developed Kaposi’s sarcoma lesions after a two-year UVB phototherapy to treat a mycosis fungoides. Negative immunohistochemistry staining for Kaposi’s sarcoma-associated herpesvirus in the initial mycosis fungoides lesions strengthens the absence of a link between Kaposi’s sarcoma-associated herpesvirus and mycosis fungoides. Immunosuppression caused by the lymphoma and prolonged phototherapy were probably the contribut ing factors for the onset of Kaposi’s sarcoma.

Human herpesvirus 8 (HHV-8) detected by nested polymerase chain reaction (PCR) in HIV patients with or without Kaposi's sarcoma. An analytic cross-sectional study / Herpesvírus humano 8 (HHV-8) detectado por reação da polimerase em cadeia do tipo nested PCR em pacientes HIV-positivos com ou sem sarcoma de Kaposi. Um estudo transversal analítico

CONTEXT AND OBJECTIVE: Kaposi's sarcoma (KS) is a common neoplastic disease in AIDS patients. The aim of this study was to evaluate the frequency of human herpesvirus 8 (HHV-8) infection in human immunodeficiency virus (HIV)-infected patients, with or without KS manifestations and correlate HHV-8 detection with KS staging. DESIGN AND SETTING: Analytic cross-sectional study conducted in a public tertiary-level university hospital in Ribeirão Preto, São Paulo, Brazil. METHODS: Antibodies against HHV-8 lytic-phase antigens were detected by means of the immunofluorescence assay. HHV-8 DNA was detected in the patient samples through a nested polymerase chain reaction (nested PCR) that amplified a region of open reading frame (ORF)-26 of HHV-8. RESULTS: Anti-HHV-8 antibodies were detected in 30% of non-KS patients and 100% of patients with KS. Furthermore, the HHV-8 DNA detection rates observed in HIV-positive patients with KS were 42.8% in serum, 95.4% in blood samples and 100% in skin biopsies; and in patients without KS, the detection rate was 4% in serum. Out of the 16 serum samples from patients with KS-AIDS who were classified as stage II, two were positive (12.5%); and out of the 33 samples from patients in stage IV, 19 (57.6%) were positive. CONCLUSION: We observed an association between HHV-8 detection and disease staging, which was higher in the serum of patients in stage IV. This suggests that detection of HHV-8 DNA in serum could be very useful for clinical assessment of patients with KS and for monitoring disease progression.

CONTEXTO E OBJETIVO: Sarcoma de Kaposi (SK) é uma doença neoplásica comum em pacientes com aids. O objetivo deste estudo foi avaliar a frequência da infecção por herpesvírus humano 8 (HHV-8) em pacientes infectados por HIV, com ou sem SK e associar a detecção do HHV-8 com o estadiamento do SK. TIPO DE ESTUDO E LOCAL: Estudo transversal analítico realizado em hospital universitário público terciário de Ribeirão Preto, São Paulo, Brasil. MÉTODOS: Anticorpos contra antígenos de fase lítica do HHV-8 foram detectados por imunofluorescência. O DNA viral de HHV-8 foi detectado em amostras de pacientes pela reação em cadeia da polimerase do tipo nested (nested PCR), que amplificou uma região do fragmento de leitura aberta (ORF)-26 do HHV-8. RESULTADOS: Anticorpos anti-HHV-8 foram detectados em 30% dos pacientes sem SK e 100% dos com SK. Além disso, a detecção de HHV-8 DNA observada em pacientes HIV-positivos com SK foi de 42,8% no soro, 95,4% em amostras de sangue e 100% em biópsias de pele, e em pacientes sem SK foi de 4% no soro. Das 16 amostras de soro de pacientes com SK-AIDS classificados como estádio II, duas foram positivas (12,5%) e, das 33 amostras de pacientes no estádio IV, 19 (57,6%) foram positivas. CONCLUSÃO: Observamos associação entre a detecção do HHV-8 e o estadiamento da doença, que foi maior no soro de pacientes no estágio IV. Isso sugere que a detecção do HHV-8 no soro poderia ser muito útil para a avaliação clínica de pacientes com SK e para o monitoramento da progressão da doença.

Enfermedad de Castleman e infección por HIV. Presentación de dos casos y revisión de la literatura / Castleman disease and HIV infection. Presentation of two cases and revision of the literature

La enfermedad de Castleman es un desorden linfoprolifera-tivo de origen aún incierto pero, en principio, relacionado con una dis-función de las células dendríticas foliculares y con una producción al-terada de distintas citoquinas, la mayor parte de ellas con actividad proinflamatoria y responsable de la sintomatología que presentan los pacientes.La relación con la presencia del HHV8, especialmente de las formas graves, ha sido ampliamente documentada en los últimos años y su desarrollo en el marco de la infección por el HIV permite una evolu-ción desafortunada de esta asociación morbosa presentando una ten-dencia importante hacia el desarrollo de patologías neoplásicas tales como la enfermedad de Kaposi y distintos tipos de linfomas.Se presentan dos casos de enfermedad de Castleman asociados a in-fección por HIV y HHV8 y se describe el contexto patogénico donde se desarrollan

Castleman ́s disease is a lymphoproliferative disorder of uncertain origin but, principally, related to dysfunction of follicular dendritic cells and impaired production of various cytokines, most of which have proinflammatory activity and are responsible for the symptoms that patients present.The relationship between Castleman ́s disease and HHV8, especially in severe forms, has been well documented in the last years. This morbid association is related to an unfortunate evolution in the context of HIV infection, presenting an increased risk of neoplastic disorders such as Kaposi ́s disease and various types of lymphomas.Two cases of Castleman ́s disease associated with HHV8 and HIV infection, and the pathogenic context in which they developed, are presented and described

Doença de Castleman multicêntrica não associada aos vírus HHV-8 e HIV / Multicentric Castleman disease not associated with HHV-8 and HIV viruses

A doença de Castleman (DC) é uma desordem linfoproliferativa policlonal, também conhecida como hiperplasia nodular gigante ou hiperplasia angiofolicular linfoide. Esta é uma doença rara que está frequentemente associada ao vírus da imunodeficiência humana (HIV) e ao herpes vírus 8 (HHV-8). Os achados histopatológicos encontrados na DC sugerem uma intensa resposta aos estímulos antigênicos observada em várias doenças associadas com ativação imune, como a artrite reumatoide. Um fator importante implicado na patogênese da DC é a produção autônoma da interleucina-6 (IL-6). Nessa doença, as manifestações clínicas estão relacionadas aos níveis de IL-6, e a remoção cirúrgica dos linfonodos acometidos ou a utilização de anticorpos anti-IL-6 fazem regredir os sintomas. Descrevemos um caso da DC multicêntrica em uma mulher jovem, não associada à infecção pelo vírus HHV-8 ou à imunossupressão. Uma breve revisão da literatura se segue à descrição do caso clínico.

Castleman's disease (CD) is a polyclonal lymphoproliferative disorder also known as giant nodular hyperplasia or angiofollicular lymph node hyperplasia. It is a rare disease often associated to human immunodeficiency virus (HIV) and human herpes virus 8 (HHV-8). Histopathological findings in Castleman's disease suggest an exaggerated response to antigenic stimuli seen in other diseases associated with immune activation, such as rheumatoid arthritis. An important aspect of its pathogenesis is the autonomous production of interleukin-6 (IL-6). In this disease, the clinical manifestations are associated to IL-6 serum levels, and surgical removal of the compromised lymph nodes or use of anti-IL-6 antibodies can slow down the symptoms. We describe a multicentric Castleman's disease in a young woman not associated to HHV-8 virus infection or immunosuppression. A short review of the literature follows the description of this clinical case.