Sarcoma de Kaposi oral en paciente seropositivo, fase sintomática avanzada. Presentación de caso / Oral Kaposi Sarcoma, advanced symptomatic phase, in a seropositive patient. Case presentation

RESUMEN Se presentó el caso clínico de un paciente seropositivo, en fase sintomática avanzada. Fue atendido en el Servicio de Medicina Interna del Hospital Clínico Quirúrgico Docente "León Cuervo Rubio", de la ciudad de Pinar del Río, por presentar disnea, astenia, anorexia y pérdida de peso. Al examen oral se constató lesión tumoral de la lengua que dificultaba la masticación y deglución. La biopsia mostró sarcoma de Kaposi asociado al VIH/sida. La evolución tórpida y el estadio tan avanzado de la enfermedad, propiciaron el deceso del paciente (AU).

ABSTRACT The authors presented the clinical case of a seropositive patient, in advanced symptomatic phase. The patient attended the Internal Medicine Service of the Teaching Clinical Surgical Hospital Leon Cuervo Rubio of Pinar del Rio, presenting dyspnea, asthenia, anorexia and weight loss. On the oral examination, a tumor lesion was found making difficult to chew and swallow. A biopsy showed Kaposi sarcoma associated to HIV/AIDS. The torpid evolution and disease's advanced stage propitiated the patient's death (AU).

Clinical and laboratory characteristics, staging, and outcomes of individuals with AIDS-associated Kaposi's sarcoma at an university hospital.

BACKGROUND:: Kaposi's sarcoma continues to be the most common human immunodeficiency virus - associated neoplasm with considerable morbidity and mortality. OBJECTIVE:: To describe the clinical and laboratory characteristics, initial staging, and outcomes of aids patients with Kaposi's sarcoma at an university hospital of Recife, Pernambuco. METHODS:: This is a descriptive study with analytic character, retrospective, of a case series between 2004 and 2014. RESULTS:: Of the 22 patients included in the study, 20 were aged <40 years (72.7%). The majority had CD4+ T lymphocyte counts of <200 cells/mm3 (77.3%) and human immunodeficiency virus loads of <100,000 copies/mL (78.9%). Lesions were most commonly observed on the skin (90%), and internal organs were affected in 11 of the 22 patients. Only 7 (31.8%) of the 22 patients were undergoing antiretroviral therapy (ART) at the time of Kaposis sarcoma diagnosis, and the initial disease staging classification was high risk (Aids Clinical Trials Group Oncology Committee) in 19 of the 22 patients (86.4%). Regarding Kaposi's sarcoma treatment, 17 of 22 patients (77.3%) underwent systemic chemotherapy + ART and 5 were treated exclusively with ART. Eight of the 22 patients died (36.5%); of these, 87.5% had died within one year of Kaposi's sarcoma diagnosis. LIMITATION OF THE STUDY:: Without a control group, this study cannot be used to generate hypotheses. CONCLUSIONS:: Despite the association between aids and late Kaposi's sarcoma diagnosis in the study population, including an unfavorable risk at the time of staging, a lower mortality rate was observed relative to other studies; this might be related to access to a specialized health service.

Clinical and laboratory characteristics, staging, and outcomes of individuals with AIDS-associated Kaposi's sarcoma at an university hospital

Abstract: Background: Kaposi's sarcoma continues to be the most common human immunodeficiency virus - associated neoplasm with considerable morbidity and mortality. Objective: To describe the clinical and laboratory characteristics, initial staging, and outcomes of aids patients with Kaposi's sarcoma at an university hospital of Recife, Pernambuco. Methods: This is a descriptive study with analytic character, retrospective, of a case series between 2004 and 2014. Results: Of the 22 patients included in the study, 20 were aged <40 years (72.7%). The majority had CD4+ T lymphocyte counts of <200 cells/mm3 (77.3%) and human immunodeficiency virus loads of <100,000 copies/mL (78.9%). Lesions were most commonly observed on the skin (90%), and internal organs were affected in 11 of the 22 patients. Only 7 (31.8%) of the 22 patients were undergoing antiretroviral therapy (ART) at the time of Kaposis sarcoma diagnosis, and the initial disease staging classification was high risk (Aids Clinical Trials Group Oncology Committee) in 19 of the 22 patients (86.4%). Regarding Kaposi's sarcoma treatment, 17 of 22 patients (77.3%) underwent systemic chemotherapy + ART and 5 were treated exclusively with ART. Eight of the 22 patients died (36.5%); of these, 87.5% had died within one year of Kaposi's sarcoma diagnosis. Limitation of the study: Without a control group, this study cannot be used to generate hypotheses. Conclusions: Despite the association between aids and late Kaposi's sarcoma diagnosis in the study population, including an unfavorable risk at the time of staging, a lower mortality rate was observed relative to other studies; this might be related to access to a specialized health service.

[Historical Review of Kaposi sarcoma in pre-HAART era: evolution with different chemotherapy schedules and remission with ganciclovir use]. / Revisión histórica del sarcoma de Kaposi en la era pre-TARAA (terapia antirretroviral altamente activa): evolución con diferentes esquemas de quimioterapia y remisión con el uso de ganciclovir.

Ganciclovir has shown in vitro anti-human herpesvirus-8 activity, Kaposi sarcoma agent. We analyzed all Kaposi sarcoma patients from 1985 to 1996 pre-HAART era and identified Kaposi sarcoma/AIDS patients who achieved complete remission prior to HAART use. RESULTS: We saw 155 Kaposi sarcoma patients up to 1996, 150 with enough information, only 12 received ganciclovir, eight of them for ≥ 21 days; four died within 16 weeks of ganciclovir administration. We identified four male patients with extensive Kaposi sarcoma with complete remission achieved after ganciclovir for CMV end-organ disease. Complete remission was achieved (9, 5, 10 and 5 months) after ganciclovir, which persisted even after antiretroviral therapy failure. All received two nucleosides and indinavir was later added with irregular compliance. The CD4 counts when ganciclovir was started: 11 (4%), 60 (5%), 127 (14%), and 38 (3%) and when they achieved complete remission: 37 (4%), 109 (9%), 313 (13%) and 136 (9%), respectively. Two patients died with no Kaposi sarcoma relapse three years later, with wasting syndrome and other pulmonary-embolism seven years later. One was lost to follow-up in complete remission in the year 2000, the other was alive in 2014 with 27% 820 CD4 cells/ml. The use of ganciclovir was statistically significantly associated with Kaposi sarcoma remission p = 0.001. CONCLUSIONS: Ganciclovir use was associated to complete remission of Kaposi sarcoma in the pre-HAART era.

Steroids are a risk factor for Kaposi's sarcoma-immune reconstitution inflammatory syndrome and mortality in HIV infection.

OBJECTIVES: To investigate the association between Kaposi's sarcoma-associated immune reconstitution inflammatory syndrome (KS-IRIS) and mortality, with the use of glucocorticoids in HIV-infected individuals. DESIGN: Case-control study. METHODS: We reviewed the medical records of 145 individuals with HIV-associated Kaposi's sarcoma receiving antiretroviral therapy. The association of different variables with KS-IRIS and Kaposi's sarcoma-related mortality was explored by univariate and multivariate analyses. The main exposure of interest was the use of glucocorticoids. We also compared the time to KS-IRIS and the time to death of individuals treated with glucocorticoids vs. those nontreated with glucocorticoids, and the time to death of individuals with KS-IRIS vs. those without KS-IRIS by hazards regression. RESULTS: Sixty of 145 individuals received glucocorticoids (41.4%) for the management or suspicion of Pneumocystis jirovecii pneumonia. Fifty individuals had KS-IRIS (37%). The use of glucocorticoids was more frequent in individuals with KS-IRIS than in those without KS-IRIS (54.9 vs. 36.47%, P = 0.047). Kaposi's sarcoma-related mortality occurred in 17 cases (11.7%), and glucocorticoid use was more frequent in this group (76.47 vs. 36.7%, P = 0.003). Glucocorticoid use was a risk factor for mortality (adjusted odds ratio = 4.719, 95% confidence interval = 1.383-16.103, P = 0.0132), and was associated with shorter periods to KS-IRIS (P = 0.03) and death (P = 0.0073). KS-IRIS was a risk factor for mortality (P = 0.049). CONCLUSION: In HIV-infected individuals, the use of glucocorticoids is a risk factor for KS-IRIS and Kaposi's sarcoma-associated mortality. In addition, KS-IRIS is a risk factor for mortality. Therefore, glucocorticoid administration in this population requires careful consideration based on individualized risk-benefit analysis.

Intralesional bevacizumab in patients with human immunodeficiency virus-associated Kaposi's sarcoma in the upper airway.

OBJECTIVES/HYPOTHESIS: The aim of this study was to evaluate the efficacy and safety of intralesional bevacizumab, a monoclonal antibody against vascular endothelial growth factor, in patients with human immunodeficiency virus (HIV)-associated Kaposi's sarcoma of the upper airway receiving antiretroviral therapy. STUDY DESIGN: A pilot randomized, open, phase II study. METHODS: HIV-infected patients with Kaposi's sarcoma lesions of the upper airway in the T0 stage were randomized to receive antiretroviral therapy alone or antiretroviral therapy with intralesional bevacizumab. The primary end point was the assessment of changes in tumor size according to the Response Evaluation Criteria In Solid Tumors (RECIST); the secondary end point was safety. RESULTS: Of the 14 patients with Kaposi's sarcoma included in the study, seven were assigned to the bevacizumab group and seven to the control group. The median age was 30.5 years (interquartile range [IQR], 24.7-38.2). Four patients (28.5%) had >150 CD4 T cells/mm(3). Nine patients had lesions in the oral cavity; three patients had pharyngeal disease; one patient had laryngeal involvement; and one patient had oral cavity, pharyngeal, and laryngeal involvement. Four patients had complete response (28.5%), two had partial response, six had stable disease, and two had progressive disease. The median time to complete response was 13 weeks (IQR, 7.5-36.5). No statistical differences between groups were observed (P = .124). In the bevacizumab group, one patient had a grade I adverse event, and another patient had a grade II adverse event. CONCLUSIONS: Intralesional administration of bevacizumab was well tolerated but had no impact on upper respiratory tract Kaposi's sarcoma lesions of HIV-infected patients.

Kaposiform lymphangiomatosis: a distinct aggressive lymphatic anomaly.

OBJECTIVE: To describe the clinical and imaging characteristics of a new lymphatic disorder with a unique histological pattern and poor prognosis. STUDY DESIGN: An observational, retrospective study identified and characterized 20 patients with distinct lymphatic histopathology referred to the Vascular Anomalies Center at Boston Children's Hospital between 1995 and 2011. RESULTS: The median age at onset was 6.5 years (range, birth to 44 years). Clinical and radiologic findings suggested a generalized process. The most common presentations were respiratory symptoms (50%), hemostatic abnormalities (50%), and an enlarging, palpable mass (35%). All patients had mediastinal involvement; 19 patients developed pericardial (70%) and/or pleural effusions (85%). Extrathoracic disease manifested in bone and spleen and less frequently in abdominal viscera, peritoneum, integument, and extremities. Despite aggressive procedural and medical therapies, the 5-year survival was 51% and the overall survival was 34%. Mean interval between diagnosis and death was 2.75 years (range, 1-6.5 years). CONCLUSIONS: We describe a clinicopathologically distinct lymphatic anomaly. We propose the term kaposiform lymphangiomatosis (KLA) because of characteristic clusters or sheets of spindled lymphatic endothelial cells accompanying malformed lymphatic channels. The intrathoracic component is most commonly implicated in morbidity and mortality; however, extrathoracic disease is frequent, indicating that KLA is not restricted to pulmonary lymphatics. The mortality rate of KLA is high despite aggressive multimodal therapy.

AIDS-related Kaposi's sarcoma in Brazil: trends and geopolitical distribution.

BACKGROUND: AIDS-related Kaposi's sarcoma (KS) is a unique model of the relationship between viral infection, immunity, environmental, and genetic factors in viral cancers. The goal was to determine the distribution of KS cases among Brazilian geopolitical regions, looking at the ecological relationship with median CD4 cell count. METHODS: Ecological study using Brazilian National Diseases Reporting Databases: 1982-2009. Subjects ≥ 13 years of age who have KS cited in their AIDS reporting form were selected, and demographic and HIV exposure data were collected. RESULTS: We found 11,731 KS cases in the period, with a prevalence of 2.4% among AIDS cases; 88% were male, and 68% lived in the Southeast region, which accounted for 59% of AIDS cases. The regional and national prevalence trends were similar, although the highest proportion among women was found in the North region, which has the lowest number of both AIDS and KS cases. Heterosexual transmission accounted for 87% of HIV among women compared to 18% among men. Fifty-seven percent of all KS cases were diagnosed before antiretroviral therapy (ART). Injection drug use accounted for 11% of KS cases. Median survival was 472 days before the ART era and 1482 after it (P < 0.001). Median CD4 counts increased in all regions in the period as ART coverage expanded, and a resulting correlating decline in KS cases was observed. CONCLUSIONS: Prevalence of KS declined after the introduction of ART in all regions of Brazil, suggesting individual protection conveyed by ART.

Virus de la Inmunodeficiencia Humana y Linfoma en un Hospital Universitario de Corrientes Argentina / HIV and Lymphoma in a University Hospital in Corrientes Argentina

Los pacientes infectados por el HIV presentan una mayor morbimortalidad por cáncer como Linfoma y Sarcoma de Kaposi, constituyendo en este grupo entre la 2da y 3ra causa de muerte, a pesar de la TARV . Varios estudios han demostrado la disminución de infecciones oportunistas, Sarcoma de Kaposi, Linfoma primario cerebral,en menor proporción de otros Linfomas NoH como el Linfoma Burkit, y un aumento de neoplasias no asociados al SIDA como el Linfoma Hodgkin.Objetivo: Analizar las características clínicas, tratamiento, sobrevida de pacientes HIV seropositivos con linfoma,diagnosticados y tratados en el Servicio de Clínica Médica del Hospital Escuela José F. de San Martín en la ciudad de Corrientes- Argentina. Evaluar la prevalencia se seropositividad en nuestra población de pacientes con Linfoma, y determinar el pronóstico al momento del diagnóstico.Material y Método: Se realizó un estudio descriptivo observacional mediante un análisis retrospectivo de las historias clínicas de siete pacientes con diagnóstico de Linfoma y serología (+) para HIV, tratados en el servicio de Clínica Médica del Hospital Escuela de Corrientes desde Enero 2003 a Enero 2013.Resultados: Entre 76 casos de Linfoma, 18 fueron tipo Hodgkin y 58 tipo NH. Del total 7 tenían serología positiva para HIV (12,5%) ,1 de 18 con enfermedad de Hodgkin y 6 de 58 LNH, es decir 5.5% y 10.34% respectivamente...

Pneumonia and malnutrition are highly predictive of mortality among African children hospitalized with human immunodeficiency virus infection or exposure in the era of antiretroviral therapy.

OBJECTIVE: To identify clinical characteristics predicting death among inpatients who are infected with or exposed to human immunodeficiency virus (HIV) during a period of pediatric antiretroviral therapy scale-up in sub-Saharan Africa. STUDY DESIGN: Retrospective review of medical records from every child with HIV infection (n = 834) or exposure (n = 351) identified by routine inpatient testing in Kamuzu Central Hospital, Lilongwe, Malawi, September 2007 through December 2008. RESULTS: The inpatient mortality rate was high among children with HIV infection (16.6%) and exposure (13.4%). Clinically diagnosed Pneumocystis pneumonia or very severe pneumonia independently predicted death in inpatients with HIV infection (OR 14; 95% CI 8.2 to 23) or exposure (OR 21; CI 8.4 to 50). Severe acute malnutrition independently predicted death in children who are HIV infected (OR 2.2; CI 1.7 to 3.9) or exposed (OR 5.1; CI 2.3 to 11). Other independent predictors of death were septicemia, Kaposi sarcoma, meningitis, and esophageal candidiasis for children infected with HIV, and meningitis and severe anemia for inpatients exposed to HIV. CONCLUSIONS: Severe respiratory tract infections and malnutrition are both highly prevalent and strongly associated with death among hospitalized children who are HIV infected or exposed. Novel programmatic and therapeutic strategies are urgently needed to reduce the high mortality rate among inpatients with HIV infection and HIV exposure in African pediatric hospitals.

Advanced epidemic Kaposi's sarcoma: treatment with bleomycin or combination of doxorubicin, bleomycin, and vincristine.

BACKGROUND: Systemic chemotherapy is a treatment modality in the management of epidemic Kaposi's sarcoma (EKS). We conducted a prospective trial to compare bleomycin as a single agent with a regimen of low-dose doxorubicin, bleomycin, and vincristine (ABV) in patients with advanced EKS. METHODS: Twenty-four homosexual or bisexual patients, between 21 and 40 years old, with positive enzyme-linked immunosorbent assay (ELISA) test for HIV and extensive EKS were included in the study. Half of the patients received bleomycin alone and the other half ABV. RESULTS: A total of seven patients achieved stable disease (SD) and five progressed during bleomycin treatment. A total of four patients achieved partial remission and eight SD during ABV treatment. There was no survival benefit between either arm of treatment. CONCLUSIONS: Bleomycin alone is not a good starting agent for EKS, whereas ABV seems to be a good choice, because it can produce an acceptable palliation of advanced EKS without major toxicity.

Kaposi's sarcoma in Colombia.

Seventy-nine cases of Kaposi's sarcoma (KS), which correspond to one of every 1,000 malignant tumors, were reviewed at the National Institute of Cancer (NIC), Bogotá, Colombia, from 1935 to 1985. Seventy-four percent of the patients were older than 65 years of age. The male to female ratio was 8:1. In all cases, plaques and nodules first appeared in the lower limbs; they were symmetrical in 47% of the cases and ulcerated in 25%. No generalized or epidemic forms were seen. Esophageal squamous cell carcinoma concurrent with KS was observed in one case. KS developed several years after treatment for follicular lymphoma and chronic lymphocytic leukemia in two patients. An angiosarcomatous variant and one fibrosarcomatous change were seen. Ten cases studied for factor VIII expression through the peroxidase-antiperoxidase (PAP) technique were all reactive. We conclude that in Colombia before 1985, KS behaved as a chronic multicentric Stage I disease without a tendency to associate simultaneously with malignant conditions.

Immediate causes of death in acquired immunodeficiency syndrome.

We evaluated the immediate causes of death in 54 adults who underwent an autopsy and were diagnosed as having died of the acquired immunodeficiency syndrome between April 1980 and October 1983. The study group included 25 Haitians, 19 homosexual men, five intravenous drug abusers, two hemophiliacs (type A), and three with no known risk. Fourteen died of central nervous system diseases: 11 of Toxoplasma encephalitis, one of progressive multifocal leukoencephalopathy, one of viral encephalitis, and one of intracerebral hemorrhage. Thirty died of respiratory failure; 16 of Pneumocystis carinii pneumonia, ten of cytomegalovirus pneumonia, one of multiple infections, one of interstitial pneumonia, and two of bacterial pneumonia. Two died of overwhelming generalized infections: one of Mycobacterium avium-intracellulare and one of listeriosis. Six died of disseminated Kaposi's sarcoma, while the remaining two persons died of Toxoplasma myocarditis (one) and one of shock resulting from a percutaneous liver biopsy, respectively. There were differences in the immediate causes of death between Haitians and homosexuals as follows: 63% of homosexual men died of either P carinii pneumonia or Kaposi's sarcoma vs 20% of Haitians. In contrast, 72% of Haitians died of other opportunistic infections as compared with 21% of homosexuals. There has not been an increase in the proportion of cases diagnosed premortem since 1982 and overall, only 32 (58%) were diagnosed premortem; the rest were diagnosed only at autopsy. This study provided evidence that 42% died of currently untreatable diseases.