Clinical and histopathology characteristics of Castleman disease: a multicenter study of 51 Brazilian patients.

Castleman's disease (CD) is a rare and heterogeneous lymphoproliferative disorder, with limited available clinical information in Brazil. A retrospective study was carried out through information contained in the medical records of 51 patients, between July 1999 and June 2020. Seven patients were excluded, and 44 were analyzed in total. The average age of unicentric CD (UCD) patients was 35 years old and of multicentric CD (MCD) patients was 49 years old (p = 0.013). Regarding gender, there was a predominance of females among patients with UCD (68.4%) and males in patients with MCD (57.9%) (p = 0.103). The most common site of involvement in UCD was the cervical region (36.8%). A total of 73.7% of patients with UCD and 68.4% of patients with MCD presented the histological form hialyne-vascular (HV) (p = 0.499). Most patients with laboratory abnormalities had MCD. A total of 78% of the patients were asymptomatic, with the majority of symptomatic patients with MCD (p = 0.042). Only two of the 27 patients evaluated for the presence of human immunodeficiency virus (HIV) had positive serology. HHV-8 was evaluated in 14 cases, being positive in two. Of the patients with UCD, 94.7% underwent excisional biopsy, against only 41.2% of patients with MCD (p = 0.01). The mean follow-up was 61 months. We observed similarities in the clinical profile between patients in our study and patients described in the literature, such as gender, mean age, B symptoms, visceromegaly, fluid accumulation, and treatment. Unlike the literature, the cervical region was the most affected site, besides the greater association of the HV histological subtype among patients with MCD.

Angiogênese e prognóstico em linfoma de células do manto / Angiogenesis and prognosis in mantle cell lymphoma

INTRODUÇÃO: Fatores prognósticos em Linfoma de Células do Manto (LCM) foram definidos em época sem padronização de tratamento e menos conhecimento de patogenia. Entre estes conhecimentos a participação da angiogênese ainda tem sido pouco estudada. Assim, a identificação de novos fatores prognósticos e melhor definição do impacto da angiogênese no LCM podem auxiliar na terapêutica e caracterizar grupos com maior risco de óbito. OBJETIVO: Avaliar a microdensidade vascular (MDV) em pacientes com LCM tratados no A.C.Camargo Cancer Center e relacioná-la a fatores que impactem na sobrevida global(SG). MATERIAL E MÉTODOS: Estudo retrospectivo, unicêntrico, transversal em portadores de LCM não indolente em cujas amostras histológicas foi quantificada MDV através de CD34 e avaliada angiogênese através de HIF-∝ e VEGF. Também foram consideradas variáveis demográficas, clínicas, laboratoriais, índices prognósticos e dados de tratamento e resposta ao tratamento. Modelo de riscos proporcionais de Cox simples foi ajustado para descrever a relação entre variáveis independentes e tempo até o óbito. Seleção das variáveis para modelo de regressão de Cox múltiplo foi considerada a partir dos resultados do modelo de Cox simples. RESULTADOS: No período de 2006 a 2014 analisamos 63 pacientes. A maioria foi do sexo masculino (68.3%) com estádio IV (81%). A mediana de idade foi de 64 anos e mediana de MIPI e MIPI-b de 5,7 e 6,2 respectivamente. Citarabina em alta dose foi utilizada em 44,4% dos pacientes e 36,5 % dos pacientes realizaram TACTH. O tempo mediano de seguimento foi 72,34 meses e a mediana de sobrevida global (SG) foi de 93,91 meses. Dos 63 pacientes iniciais, 34 dispunham de amostras para análise de MDV. A média e mediana de MDV foi de 14,88 e 3,5 vasos/mm2 respectivamente e 64,71% dos pacientes apresentaram VEGF e HIF-∝ ≥10%. Em análise univariada nenhum parâmetro de angiogenese apresentou significância estatística. Idade, hemoglobina , albumina, número de leucócitos, linfócitos, plaquetas, valores de MIPI e Ki-67 apresentaram significância estatística para SG. Em análise multivariada idade, concentração de albumina e plaquetas apresentaram significância permitindo estabelecer que pacientes com idade superior a 70 anos possuíram risco 8 vezes maior (HR=8,025; valor p=0,0101) de ir à óbito em relação aqueles com idade menor ou igual a 70 anos. Para cada incremento de unidade de albumina houve redução de 79,9% (HR=0,201; valor p=0,005) no risco de óbito mantido a idade constante enquanto pacientes com contagem de plaquetas acima de 146x109/l tiveram redução de risco de óbito em 78% (HR=0,231; valor p=0,003). CONCLUSÃO: Os resultados do presente estudo permitem concluir que a angiogênese não demonstrou ser um valor preditivo de óbito e que parâmetros diferentes dos habitualmente utilizados associam-se a maior risco de óbito. Tais achados devem ser validados em uma coorte mais ampla de pacientes.

INTRODUCTION: Mantle Cell Lymphoma (MCL) prognostic factors were defined in a no standardized treatment and less well-known pathogenesis time. Among them, angiogenesis has been little studied. Therefore, angiogenesis impact better definition and new prognostic factors can define higher death-risk groups and help MCL patients' management. PURPOSE: To evaluate microvascular density (MDV) in MCL patients treated at the AC Camargo Cancer Center as well as identify demographic, clinical and laboratory factors that impact on overall survival (OS). MATERIAL AND METHODS: We conducted a retrospective, single-center and cross-sectional study. We included all non-indolent MCL patients who underwent immuno-chemotherapy. MDV was quantified by CD34 analysis and angiogenesis was determined by HIF-∝ and VEGF markers. We took into account demographic, clinical, laboratory, prognostic indexes, treatment and response to treatment variables. Cox's proportional hazards model was adjusted to data to describe variables and time until death relationship. Variables selection for multiple Cox regression analysis model was considered from simple Cox model results. RESULTS: From 2006 to 2014, we analyzed 63 patients. The majority were male (68.3%) and Ann Arbor stage IV (81%) and they were 64 years old median age with median MIPI= 5.7 and MIPI-b= 6.2. High dose cytarabine was used in 44.4% of patients and 36.5% underwent TACTH. Median follow-up was 72.34 months and median overall OS was 93.91 months. Of 63 patients, 34 had samples for MDV analysis. The mean and median MDV was 14.88 and 3.5 vessels / mm2, respectively and 64.71% of patients had VEGF and HIF-∝ ≥10%. In univariate analysis, no angiogenesis parameter showed statistical significance. Age, hemoglobin, albumin, leukocytes, lymphocytes, platelets, MIPI and Ki-67 values were statistically significant for overall survival. In multivariate analysis, age, albumin concentration and platelet count were statistically significant, allowing the establishment of a predictive model. Patients over 70 years old had more than 8 times increased death-risk (HR = 8.025; p = 0.0101) in relation to those with less than or equal to 70 years old. For each albumin unit increment a reduction of 79.9% (HR = 0.201; p-value = 0.005) is expected in death-risk while patients with platelet count above 146 x 109/l had a 78% reduction in death-risk in relation to those with platelet count equal to or less than this value (HR = 0.231; p-value = 0.003). CONCLUSION: Angiogenesis has not been shown to have death predictive value. Diferent parameters from those commonly used are associated with higher death-risk. Such findings should be validated in a broader cohort of patients.

O uso intravenoso de sacarato de hidróxido de ferro III em pacientes com anemia ferropriva / Evaluation of the efficacy of intravenous iron III-hydroxide saccharate for treating adult patients with iron deficiency anemia

OBJETIVO: Avaliar a eficácia do uso intravenoso de sacarato de hidróxido de ferro III no tratamento de pacientes adultos com anemia ferropriva que não obtiveram resposta satisfatória à terapia com ferro oral. MÉTODOS: No período de janeiro de 2003 a janeiro de 2004, estudamos 25 pacientes com anemia ferropriva que apresentaram intolerância e/ou resposta inadequada ao tratamento com ferro por via oral e/ou valor de hemoglobina < 7 g/dl. Os principais exames laboratoriais realizados foram: hemograma completo, contagem de reticulócitos, ferro sérico, capacidade total de ligação de ferro e ferritina sérica. Os pacientes receberam uma dose semanal de 200 mg de sacarato de hidróxido de ferro III diluído em 250 ml de soro fisiológico 0,9 por cento administrado por via intravenosa em 30 minutos. O tratamento foi realizado até a obtenção dos valores de hemoglobina =12 g/dl para mulheres e =13 g/dl para homens, ou até a administração da dose total de ferro parenteral recomendada para cada paciente. RESULTADOS: A idade mediana dos 25 pacientes estudados foi de 45 anos, variando entre 31 e 70 anos; 19 (76 por cento) eram do sexo feminino. A causa mais comum de anemia ferropriva no sexo feminino foi sangramento uterino anormal observado em 13/19 pacientes (68 por cento) e, no sexo masculino, gastrectomia parcial observada em 4/6 (67 por cento). Dezessete (68 por cento) pacientes foram incluídos neste estudo por falta de resposta à terapia com ferro oral, 6/25 (24 por cento) por intolerância ao ferro oral e 2/25 (8 por cento) por hemoglobina < 7 g/dL. Correção da anemia foi obtida em 12/19 (63 por cento) dos pacientes do sexo feminino e em 5/6 (83 por cento) dos pacientes do sexo masculino. Os valores médios da hemoglobina e da ferritina eram de 8,09 g/dl e 4,20 ng/ml (pré-tratamento) e 12,42 g/dl e 87,78 ng/ml (pós-tratamento) (p<0,001), respectivamente. O aumento médio de hemoglobina foi de 3,74 g/dl, variando entre 1,30 g/dl e 7,60 g/dl. Nenhum paciente recebeu transfusão de sangue durante ou após o tratamento com ferro intravenoso. CONCLUSÃO: O uso intravenoso de sacarato de hidróxido de ferro III é uma opção eficaz e segura no tratamento de pacientes adultos com anemia ferropriva que não obtiveram resposta satisfatória com a utilização do ferro oral. Esta opção terapêutica deve ser levada em consideração sobretudo nos pacientes com intensa anemia a fim de se obter rápido aumento dos valores da hemoglobina e se evitar transfusão de sangue.

[Evaluation of the efficacy of intravenous iron III-hydroxide saccharate for treating adult patients with iron deficiency anemia]. / O uso intravenoso de sacarato de hidróxido de ferro III em pacientes com anemia ferropriva.

OBJECTIVE: To evaluate the efficacy of intravenous iron III-hydroxide saccharate for treating adult patients with iron deficiency anemia lacking satisfactory response to oral iron therapy. METHODS: Between January 2003 and January 2004, 25 patients with iron deficiency anemia who presented intolerance or inadequate response to iron oral therapy, or hemoglobin level < 7 g/dl were studied. The main laboratory tests performed were: complete blood cell count, reticulocyte count, serum iron, total iron-binding capacity, serum ferritin. Patients received a weekly dose of 200 mg of iron diluted in 250 mL of 0.9% sodium chloride solution administered intravenously for 30 minutes. Treatment continued until a hemoglobin level = 12 g/dL for women and = 13 g/dL for men were obtained or until full administration of the total dose of parenteral iron recommended for each patient. RESULTS: Median age of the patients studied was 45 years (ages ranging from 31 to 70). Nineteen out of 25 patients (76%) were women. The most common cause of iron deficiency anemia was abnormal uterine bleeding observed in 68% of the female patients (13 out of 19) and partial gastrectomy observed in 67% of the male patients (4 out of 6). Seventeen (68%) patients were included in this study because they did not respond to oral iron therapy, 24% (6 out of 25) showed intolerance to oral iron and 8% (2 out of 25) presented hemoglobin level < 7 g/dl. Correction of anemia was obtained in 12 out of 19 female patients (63%) and in 5 out of 6 male patients (83%). The mean hemoglobin and ferritin values were 8.09 g/dl and 4.20 ng/ml (pre-treatment) and 12.42 g/dl and 87.78 ng/ml (post-treatment) (p < 0.001), respectively. The average increase of hemoglobin was 3.74 g/dl, ranging from 1.30 to 7.60 g/dl. None of the patients received blood transfusion during or after the intravenous iron treatment. CONCLUSION: The use of intravenous iron III-hydroxide saccharate is an efficacious and safe option in the treatment of adult patients with iron deficiency anemia lacking satisfactory response to oral iron therapy. This option of treatment should be considered mainly for patients with severe anemia in order to obtain rapid increase of the hemoglobin level and avoid blood transfusion.

Endocardite infecciosa: análise de 20 casos de óbito na Santa Casa de Säo Paulo / Infectious endocarditis: analysis of 20 death cases in Santa Casa de Säo Paulo

PURPOSE--To analyze the clinical, laboratory and pathological aspects of 20 cases of infectious endocarditis (IE) who died. The authors compared patients with diagnosis before death of IE and those with diagnosis was made after autopsy. METHODS--Twenty patients who died with IE between April 1982 and November 1991 were studied. We looked for the clinical aspects (fever, cardiac murmurs, anemia, splenomegaly, embolic events and skin manifestation), laboratory aspects (hemocultures), echocardiographic and anatomopathologic features (valvar vegetations events and embolic accidents founded at autopsy). The sample was divided in two sub-groups: A--with clinical diagnosis of IE before and B--without diagnosis before death. RESULTS--Group A--9 patients aged 8-58 years, 3 men, all them with cardiac murmurs, fever and anemia, 5 with splenomegaly. Hemocultures were done in 7 patients and positive in 1. Echocardiogram with valvar vegetation were found in 4 out of 5 patients (80 per cent positive). At autopsy mitral valve vegetation were present in 7, aortic 3, tricuspid 3. One patient showed the exposure of three valves and two of 2 valves. Embolic events were found in 4. Group B--11 patients most of them older then 50 years (54.5 per cent) (p < 0.05) 5 men, all them presented fever and anemia. Cardiac murmurs in 6 (54 per cent) and none with splenomegaly. In one case hemoculture and echocardiogram, were done and were negative. Anatopathologic study showed compromise of mitral valve in 5, aortic 4, tricuspid 2, pulmonary 1. Two patients had 2 valves compromised. In one case a mural vegetation (right atrium) was found. Embolic events were present in 2 cases. CONCLUSION--In group B a significant number of patients (p < 0.05) were older than 50 years and presented his symptoms as an acute illness. We concluded that older patients with compromised general state and fever with or without embolic events IE must be remember