Precordial pain, leukocytosis and bicytopenia in a teenager with systemic juvenile idiopathic arthritis under immunosuppressive therapy / Dor precordial, leucocitose e bicitopenia em adolescente com artrite idiopática juvenil sistêmica em terapia imunossupressora

ABSTRACT Objective: To highlight the importance of the new classification criteria for the macrophage activation syndrome (MAS) in systemic juvenile idiopathic arthritis in order to reduce morbidity and mortality outcome related to this disease. Case description: A 12-year-old female patient with diagnosis of systemic juvenile idiopathic arthritis under immunosuppression therapy for two years developed cough, acute precordial chest pain, tachypnea, tachycardia and hypoxemia for two days. Chest tomography showed bilateral laminar pleural effusion with bibasilar consolidation. The electrocardiogram was consistent with acute pericarditis and the echocardiogram showed no abnormalities. Laboratory exams revealed anemia, leukocytosis and increased erythrocyte sedimentation rate, as well as C-reactive protein rate and serum biomarkers indicative of myocardial injury. Systemic infection and/or active systemic juvenile idiopathic arthritis were considered. She was treated with antibiotics and glucocorticoids. However, 10 days later she developed active systemic disease (fever, evanescent rash and myopericarditis with signs of heart failure) associated with macrophage activation syndrome, according to the 2016 Classification Criteria for Macrophage Activation Syndrome in Systemic Juvenile Idiopathic Arthritis. She was treated for five days with pulse therapy, using glucocorticoids, immunoglobulin and cyclosporine A, with improvement of all clinical signs and laboratory tests. Comments: Myopericarditis with signs of heart failure associated with MAS is a rare clinical presentation of systemic juvenile idiopathic arthritis. Macrophage activation syndrome occurs mainly during periods of active systemic juvenile idiopathic arthritis and may be triggered by infection. Knowledge about this syndrome is crucial to reduce morbidity and mortality.

RESUMO Objetivo: Destacar a importância do conhecimento sobre os novos critérios de classificação para síndrome de ativação macrofágica (SAM) na artrite idiopática juvenil sistêmica para reduzir a morbidade e mortalidade desse desfecho. Descrição do caso: Adolescente do sexo feminino de 12 anos de idade, em terapia imunossupressora por diagnóstico de artrite idiopática juvenil sistêmica há 2 anos, com quadro de tosse, dor precordial aguda, taquipneia, taquicardia e hipoxemia há 2 dias. A tomografia de tórax evidenciou efusão pleural laminar bilateral com consolidação bibasal. O eletrocardiograma foi compatível com pericardite aguda, e o ecocardiograma foi normal. Os exames laboratoriais revelaram anemia, leucocitose e aumento da velocidade de hemossedimentação, proteína C-reativa e marcadores séricos de lesão miocárdica. Infecção sistêmica e/ou doença sistêmica em atividade foram consideradas. A paciente foi tratada com antibióticos e glicocorticoide. Entretanto, dez dias depois, evoluiu com doença sistêmica em atividade (febre, exantema e miopericardite com insuficiência cardíaca) associada à SAM, de acordo com o 2016 Classification Criteria for Macrophage Activation Syndrome in Systemic Juvenile Idiopathic Arthritis, e necessitou de cinco dias de pulsoterapia com glicocorticoide, imunoglobulina e ciclosporina A, com melhora de todos os parâmetros clínicos e laboratoriais. Comentários: A miopericardite com sinais de insuficiência cardíaca associada à SAM é uma apresentação clínica rara da artrite idiopática juvenil sistêmica, que ocorre principalmente em períodos de atividade sistêmica da doença e pode ser deflagrada por infecções. O conhecimento sobre essa síndrome é fundamental para reduzir morbidade e mortalidade desse grave desfecho.

Variables condicionantes del tratamiento en pacientes añosos con leucemia mieloide aguda: Experiencia institucional / Treatment conditioning variables in elderly patients with acute myeloid leukemia. An institutional experience

Los mayores de 60 años con leucemia mieloide aguda (LMA) tienen peor pronóstico que el resto de los pacientes. En la literatura se expresan diferentes factores que podrían condicionar su supervivencia. Se propuso determinar cuáles fueron los principales determinantes de supervivencia global en nuestra población; y como objetivo secundario cuáles estaban vinculados con mortalidad temprana, entendida la misma como muerte dentro de las ocho semanas de iniciado el tratamiento. Para ello se diseñó un estudio de cohorte retrospectivo que incluyó 133 pacientes no tratados previamente. El análisis ajustado a covariables demostró que las variables de mayor peso para determinar supervivencia global fueron el recuento leucocitario ≥ 30 000 al diagnóstico [HR ajustado 2.19 (1.06-4.53), p = 0.03)] y el estado general (ECOG) 3 o 4 [HRa 4.63 (1.69-12.68), p < 0.001)]. En cuanto a mortalidad relacionada al tratamiento, el estado general (ECOG) 3-4 mostró ser la única variable que mantuvo su poder estadístico en el análisis multivariado con OR ajustado (ORa) 12.40 (IC 1.12-137.17, p = 0.04). El mal resultado inherente a los pacientes añosos con diagnóstico de LMA no se entiende por completo aún. Probablemente la mejor forma de evaluarlos debería tener en cuenta no solo la edad, sino también resultados de laboratorio, de estudios genéticos y moleculares, utilizando índices específicos de comorbilidad, estado general y alguna evaluación geriátrica de fragilidad. Este estudio identificó que la leucocitosis y el mal estado general fueron los factores que mostraron un mayor poder en la predicción de la mortalidad.

Patients over 60 years old with acute myeloid leukemia (AML) have a worse prognosis due to several factors that determine the therapeutic outcome. The main predictors of mortality in patients with AML reported in the literature were analyzed in our population. The primary objective was to analyze overall survival. The secondary objective was to determine treatment-related mortality, defined as death within eight weeks of starting treatment. It was designed as a retrospective study. A total of 133 treatment naive patients were included, from January 1991 to August 2014. The adjusted analysis showed that the most important variables to determine overall survival were the WBC count ≥ 30 000 at diagnosis [adjusted HR 2.19 (1.06-4.53), p = 0.03)] and the Performance Status (ECOG) 3 or 4 [aHR 4.63 (1.69-12.68), p < 0.001)]. Performance Status 3-4 was the only variable that conditioned treatment related mortality, showing in the univariate analysis an OR 5.44 (CI 1.93-15.28, p < 0.001). It was also the only variable that kept its statistical power in the multivariate analysis adjusted OR (aOR) 12.40 (IC 1.12-137.17, p = 0.04). The inherent poor outcome in elderly patients diagnosed with AML is not fully understood. The best way of assessing these elderly patients should probably include not only age but the best way of assessing these elderly patients should probably include not only age but laboratory, genetic and molecular studies. Especially designed comorbidity and fragility indices should be included, along with functional status. Leukocytosis and poor quality of life were identified as the most powerfull factors for predicting mortality in our study.

Cholangiocarcinoma with a paraneoplastic leukemoid reaction mimicking a pyogenic liver abscess

No abstract available.

Synchronous Malignant Intraductal Papillary Mucinous Neoplasms of the Bile Duct and Pancreas Requiring Left Hepatectomy and Total Pancreatectomy / 대한소화기학회지

Intraductal papillary mucinous neoplasm of the bile duct (IPMN-B) and intraductal papillary mucinous neoplasm of the pancreas (IPMN-P) have striking similarities and are recognized as counterparts. However, simultaneous occurrence of IPMN-B and IPMN-P is extremely rare. A 66 year-old female presented with recurrent epigastric pain and fever. During the past 9 years, she had three clinical episodes related to intrahepatic duct stones and IPMN-P in the pancreas head and was managed by medical treatment. Laboratory test results at admission revealed leukocytosis (12,600/mm3) and elevated CA 19-9 level (1,200 U/mL). Imaging study demonstrated liver abscess in the Couinaud's segment 4, IPMN-B in the left lobe, and IPMN-P in the whole pancreas with suspicious malignant change. Liver abscess was drained preoperatively, followed by left lobectomy with bile duct resection and total pancreatectomy with splenectomy. On histologic examination, non-invasive intraductal papillary mucinous carcinoma arising from various degree of dysplastic mucosa of the liver and pancreas could be observed. However, there was no continuity between the hepatic and pancreatic lesions. This finding in our case supports the theory that double primary lesions are more likely explained by a diffuse IPMN leading to synchronous tumors arising from both biliary and pancreatic ducts rather than by a metastatic process. Herein we present a case of simultaneous IPMN of the bile duct and pancreas which was successfully treated by surgical management.

Prediction of bacterial meningitis based on cerebrospinal fluid pleocytosis in children

Children with cerebrospinal fluid pleocytosis are frequently treated with parenteral antibiotics, but only a few have bacterial meningitis. Although some clinical prediction rules, such as bacterial meningitis score, are of well-known value, the cerebrospinal fluid white blood cells count can be the initial available information. Our aim was to establish a cutoff point of cerebrospinal fluid white blood cell count that could distinguish bacterial from viral and aseptic meningitis. A retrospective study of children aged 29 days to 17 years who were admitted between January 1st and December 31th, 2009, with cerebrospinal fluid pleocytosis (white blood cell > 7 µL-1) was conducted. The cases of traumatic lumbar puncture and of antibiotic treatment before lumbar puncture were excluded. There were 295 patients with cerebrospinal fluid pleocytosis, 60.3% females, medium age 5.0 ± 4.3 years distributed as: 12.2% 1-3 months; 10.5% 3-12 months; 29.8% 12 months to 5 years; 47.5% >5 years. Thirty one children (10.5%) were diagnosed with bacterial meningitis, 156 (52.9%) viral meningitis and 108 (36.6%) aseptic meningitis. Bacterial meningitis was caused by Neisseria meningi tidis (48.4%), Streptococcus pneumoniae (32.3%), other Streptococcus species (9.7%), and other agents (9.7%). cerebrospinal fluid white blood cell count was significantly higher in patients with bacterial meningitis (mean, 4839 cells/µL) compared to patients with aseptic meningitis (mean, 159 cells/µL, p < 0.001), with those with aseptic meningitis (mean, 577 cells/µL, p < 0.001) and with all non-bacterial meningitis cases together (p < 0.001). A cutoff value of 321 white blood cell/µL showed the best combination of sensitivity (80.6%) and specificity (81.4%) for the diagnosis of bacterial meningitis (area under receiver operating characteristic curve 0.837). Therefore, the value of cerebrospinal fluid white blood cell count was found to be a useful and rapid diagnostic test to distinguish between bacterial and nonbacterial meningitis in children.

Two Cases of Myeloproliferative Neoplasm with a Concurrent JAK2V617F Mutation and BCR/ABL Translocation without Chronic Myelogenous Leukemia Phenotype Acquisition during Hydroxyurea Treatment

No abstract available.

Infección urinaria en pediatría: relación entre clínica, paraclínica y gamagrafía renal / Urinary tract infection in children: relation between symptoms, lab work-up and renal scan

Objetivo: describir la relación entre hallazgos clínicos, laboratorio y gamagrafía renal estática (GR-DMSA) en pacientes pediátricos hospitalizados por infección del tracto urinario (ITU) en el Hospital de San José. Materiales y métodos: estudio descriptivo, de corte transversal. Se incluyeron 130 pacientes entre tres meses y 14 años de edad. Se tomó información de las historias clínicas revisando las variables: edad, género, fiebre, estado general, leucocitosis en hemograma, velocidad de sedimentación globular (VSG), proteína C reactiva, hipostenuria, nitritos y estearasas leucocitarias (EL) en uroanálisis, comparando cada una de ellas con el resultado de la GR-DMSA. Resultados: el 76% de los pacientes mostró pielonefritis con GR-DMSA. El 87,7% de éstos eran lactantes y preescolares y el 72% niñas. Se realizaron análisis bivariados utilizando como medida de asociación el riesgo relativo (RR) y como medida de la precisión el intervalo de confianza (IC) del 95%. Se encontró que los pacientes que presentan leucocitosis, aumento de la VSG y EL positivas tienen probabilidad 1,81 veces mayor de presentar pielonefritis. Conclusiones: si un paciente presenta leucocitosis, VSG elevada y EL positiva, no se recomienda realizar GR-DMSA, dado que la probabilidad que curse con pielonefritis es 81%. La sensibilidad de estos laboratorios es del 76% y la especificidad del 58%, comparada con la GR-DMSA.

Objective: to describe the relation between clinical symptoms, lab work-up and static renal scan (DMSA) in pediatric in-patients with urinary tract infection (UTI) at the San José Hospital. Materials and methods: a descriptive, cross-sectional study was undertaken. A total of 130 children aged 0.3 months to 14 years were enrolled in the study. Their clinical histories were reviewed considering the following variables: age, gender, fever, general status, leukocytosis (on CBC), erythrocyte sedimentation rate (ESR), C-reactive protein level, hypostenuria, nitrates and leukocyte stearates (LS) in urinalysis, comparing each of them with the DMSA renal scan results. Results: the DMSA revealed pyelonephritis in 76%. Infants and pre-school children accounted for 87.7% of the latter and 72% were females. A bivariable analysis was performed using relative risk (RR) as the measure of association and a confidence interval (CI) of 95% as the accuracy measure. It was found that patients with leukocytosis, increased ESR and positive LS had 1.81-fold greater odds of pyelonephritis. Conclusions: DMSA is not recommended in patients with leukocytosis, increased ESR and positive LS, for the odds of being associated with pyelonephritis is 81%. The sensitivity of lab work-ups was 76% and specificity was 58%, compared to the DMSA renal scan.

Prenatal Diagnosis of Transient Abnormal Myelopoiesis in a Down Syndrome Fetus

We report a case of transient abnormal myelopoiesis in a Down syndrome fetus diagnosed at 28(+3) weeks of gestation that rapidly progressed to intrauterine death 10 days later. Fetal hepatosplenomegaly with cerebral ventriculomegaly, although not specific, may be a suggestive finding of Down syndrome with transient abnormal myelopoiesis. Prompt fetal blood sampling for liver function test and chromosomal analysis are mandatory for early detection and management.

Routine laboratory investigations in infants and children presenting with fever and seizures at the University Hospital of the West Indies / Investigaciones de rutina en el laboratorio en relación con infantes y niños que se presentan con fiebre y convulsiones en el Hospital Universitario de West Indies

A retrospective chart review of the case notes of all children aged 6 months to 8 years presenting with fever and seizures to the University Hospital of the West Indies (UHWI) between January 2000 and December 2004 was conducted. Descriptive analyses were performed. Fifty-nine children (median age 1.58 years, range 0.58 to 6.83 years) were entered into the study. The main laboratory abnormalities were metabolic acidosis (23%), anaemia (10%), leukocytosis (35%) and hypomagnesaemia (3%). These were not significantly associated with meningitis or an underlying bacterial infection. There were no significant episodes of hyponatraemia, hypocalcaemia or hypoglycaemia. Meningitis was uncommon and occurred in only two (3.4%) children both younger than 16 months of age and who had other abnormal clinical signs. This study demonstrated that routine performance of haematological and biochemical investigations in children presenting with seizures and fever were of limited value. Lumbar punctures in children older than age 18 months with no other abnormal clinical signs were also found to be of low yield. Current American Academy of Paediatrics (AAP) recommendations that serum electrolytes, calcium, phosphate, magnesium, complete blood count and blood glucose should not be performed routinely in a child with a first simple febrile seizure can be safely applied to this study population.

Se llevó a cabo un estudio retrospectivo de las historias clínicas en busca de notas sobre los casos de todos los niños de 6 meses a 8 años de edad que se presentaron con fiebre y convulsiones en el Hospital Universitario de West Indies (HUWI) entre enero de 2000 y diciembre de 2004. Se realizaron análisis descriptivos. Cincuenta y nueve niños (edad mediana (1.58 años, rango 0.58 a 6.83 años) formaron parte de este estudio. Las principales anormalidades halladas mediante el laboratorio fueron: acidosis metabólica (23%), anemia (10%), leucocitosis (35%), e hipomagnesemia (3%). Éstas no estuvieron significativamente asociadas con meningitis o alguna infección bacteriana subyacente. No hubo episodios significativos de hiponatremia, hipocalcemia o hipoglicemia. La meningitis fue poco común, ocurriendo sólo en dos niños (3.4%), ambos con menos de 16 meses de edad y con otros signos clínicos anormales. Este estudio demostró que el trabajo de rutina realizado en las investigaciones hematológicas y bioquímicas en los niños que se presentaron con fiebre y convulsiones, tuvo un valor limitado. También se halló que las punciones lumbares realizadas a niños de más de 18 meses sin ningún otro signo clínico anormal, tuvieron poco valor. Las recomendaciones actuales de la Academia Americana de Pediatría (AAP) en cuanto a que las pruebas de electrolitos en suero, las mediciones de calcio, fosfato, magnesio, el conteo sanguíneo completo, y la prueba de glucosa en sangre, no deben ser realizadas rutinariamente en un niño con una primera simple convulsión febril, pueden ser aplicadas con seguridad a esta población bajo estudio.

An observational study to know the association of leukocytosis and fever with infection in post cardiac surgery patients.

BACKGROUND: Leukocytosis and fever in the absence of infection are common phenomena in post surgical period. The systemic inflammatory response syndrome induced by surgical trauma is a well-known entity, with resultant release of variety of inflammatory cytokines leading to fever and/or leukocytosis in spite of the absence of infection. AIMS AND OBJECTIVE: To know the association of leukocytosis and fever with infection in immediate post cardiac surgical period. METHODS: It was a retrospective, observational study including 569 patients, who underwent cardiac surgeries in our institute. All demographic data, comorbidities and other factors associated with fever and/or leukocytosis were analyzed. The sensitivity and specificity of fever and/or leukocytosis were analyzed for the diagnosis of infection. RESULTS: Out of 569 patients; 49 patients (9%) had evidence of infection on microbiological culture. Mean total leukocyte counts (TLC) on zero and 1st postoperative days were associated with infection with a sensitivity (75.5% and 63.3%) and specificity (15.8% and 24.0%) respectively. Mean maximum temperature (Tmax) on zero and 1st postoperative days were associated with infection with a sensitivity (20.4% and 24.5%) and specificity of (82.5% and 83.3%) respectively. The combined sensitivity and specificity of leukocytosis and fever for the diagnosis of infection on 2nd postoperative day was 14.3% and 91.5% respectively. CONCLUSION: Fever and leukocytosis are poor predictors of diagnosing infection on the first two postoperative days. However, fever and leukocytosis combined together have low sensitivity (14.3%) with high specificity (91.5%) for the diagnosis of infection on the 2nd and subsequent postoperative days.

Rare presentation of leucocytosis.

We present the case of a 44 years man who presented to us with persistent leucocytosis. Following relevant investigations, we diagnosed him to have Chronic Neutrophilic Leukaemia (CNL); a rare haematological disorder. Ten months later, he remains non-responsive to standard line of treatment.

Prevalence, hematology and serum biochemistry in stray dogs naturally infected by Hepatozoon canis in São Paulo / Prevalência, hematologia e bioquímica sérica em cães de rua naturalmente infectados por Hepatozoon canis, em São Paulo

Cães capturados nas ruas de quatro municípios do estado de São Paulo foram examinados para estudar a prevalência de Hepatozoon canis. Dos 222 animais, 13 (5.9 por cento) encontravam-se infectados. Exames hematológicos realizados em nove animais evidenciaram três com anemia e um com leucocitose. A única alteração encontrada nos exames bioquímicos foi hiperglobulinemia.

Urgencias oncológicas / Oncology urgency

Las urgencias oncológicas son un capítulo importante dentro de la oncología pediátrica y de especial interés, tanto para los médicos generales como pediatras, ya que éstas son causa de morbimortalidad en pacientes con cáncer y pueden estar presentes desde el momento del diagnóstico. Aunque existe una gran variedad de ellas, en este capítulo sólo abordaremos las de mayor frecuencia como son la lisis tumoral, muchas veces presente al momento del diagnóstico y que puede conducir a una insuficiencia renal; la hiperleucocitosis, que es capaz de producir enfermedad por lisis tumoral o por un síndrome de hiperviscosidad, que se manifiesta por complicaciones pulmonares y neurológicas, siendo la más grave de ellas la hemorragia intracraneala; finalmente revisaremos la urgencia oncológica más frecuente que es la neutropenia febril que es secundaria a la toxicidad por quimioterapia y que puede causar la muerte en estos pacientes.

Estudo do leucograma em pacientes com infecçäo do trato urinário / Study of the leucocytes in patients with tract urinary infection

Foram estudados os leucogramas de 78 pacientes atendidos no Laboratório Médico Ciência no Hospital Infantil Joana de Gusmäo, de Florianópolis, no período de 01/01/1997 até 01/09/1997 com diagnóstico de Infecçäo do Trato Urinário (ITU), apresentando o estudo, uma correlaçäo entre a alteraçäo no leucograma com infecçäo urinária. Todos os pacientes foram submetidos a exames de urina, urocultura e leucograma considerando apenas os que apresentaram cultura positiva. Observou-se que a maioria dos pacientes acometidos por ITU apresentavam leucocitose, neutrofilia e desvio à esquerda, já as granulaçöes tóxicas näo foram normalmente encontradas.Os resultados ainda demonstraram que há relaçäo entre o aumento da leucocitúria e o aumento na leucometria, porém isso näo ocorre de maneira proporcional. Também näo houve relaçäo proporcional entre o aumento do número de Unidades Formadoras de Colônias (UFC) e da leucocitose. Os agentes mais frequentemente encontrados foram a E. coli e o Proteus sp porém näo se pode afirmar quais os que causam maiores atençöoes na leucometria

Manifestaciones clínicas de la neumonía tuberculosa / Clinical manifestations of tuberculous pheumonia

La neumonía tuberculosa, una de las formas de presentación de la TBC pulmonar, es una entidad poco estudiada en nuestro medio. realizamos un estudio descriptivo de 23 asos de neumonía tuberculosa en el Hospital Nacional Dos de Mayo durante el período 1980-1993. Los objetivos fueron: indicar las manifestaciones clínicas presentadas y obtener parámetros que ayuden a diferenciarla de la neumonía neumocócuca. Encontramos: un tiempo de enfermedad mayor de 15 días en el 78.2 por ciento, como síntomas principales, fiebre (91.3 por ciento), dolor torácico (65.2 por ciento), tos con expectoración no hemoptoica (65.2 por ciento), disnea (47.8 por ciento) y disminución de peso (47.8 por ciento); como signos, síndrome de condensación (86.6 por ciento) y crepitantes bilaterales (13.4 por ciento-casos de BNM tuberculosa-). El diagnóstico bacteriológico llegó al 56.5 por ciento; el 64.7 por ciento de 17 pacientes entre 15-60 años presentó leucocitosis. El diagnóstico presuntivo más frecuente al ingreso fue neumopatía aguda 52.2 por ciento), mientras que el de neumonía tuberculosa llegó sólo al 13 por ciento. una vez establecido el tratamiento específico, la evolución fue favorable en el 95.6 por ciento. En comparación con las otras formas de TBC pulmonar, la presentación promedio anual de la neumonía tuberculosa fue nemor al 1 por ciento. Concluimos que la neumonía neumocócica y que los parámetros de diferencicación con la misma fueron el tiempo de enfermedad mayor de 15 días y la disminución de peso.

Características clínicas y de laboratorio en pacientes con shock séptico / Laboratory and clinical characteristics in patients with septic shock

El shock séptico (SS) es la quinta causa de muerte y el 10 por ciento del total de admisiones a la Unidad de Cuidados Intensivos Generales (UCIG) del Hospital Arzobispo Loayza (HAL). Se realizó un estudio descriptivo para determinar las características clínicas y de laboratorio en pacientes con diagnóstico de SS que son admitidos a la UCIG del HAL. Se incluyó en el análisis a 53 pacientes, los cuales fueron agrupados de acuerdo a su evolución. La edad promedio fue de 54.7a ñ 21.4. La mayoría procedía del servicio de Cirugía. El foco séptico más frecuente fue el abdominal. Se encontró diferencia estadística en los niveles promedio de frecuencia cardiaca, frecuencia respiratoria y niveles de hematocrito en los pacientes que se recuperaron del cuadro respecto a los que fallecieron. La leucocitosis estuvo presente en mayor proporción entre los pacientes que fallecieron. No hubo diferecia en la evaluación del estado hemodinámico al ingreso entre vivos y muertos. El score APACHE II fue significativamente mayor entre los que fallecieron (24 ñ 6 muertos versus 17 ñ 7 en fallecidos). No hubo diferencia en la presencia ni el número de órganos en disfunción orgánica aguda a la admisión a la UCIG entre vivos y fallecidos.

Sinus histiocytosis with massive lymphadenopathy: the first three cases reported in Thailand.

Three cases of sinus histiocytosis with massive lymphadenopathy are reported in Thai children and is probably a first report in Thailand. They were clinically characterized by bilateral massive cervical lymphadenopathy. Other lymph node groups were also involved but no extranodal manifestations could be detected. Leukocytosis with neutrophilia, hypergammaglobulinemia and polyclonal gammopathy were common features. The characteristic histopathologic findings in the involved lymph nodes included pericapsular fibrosis, markedly dilatation of subcapsular and medullary sinuses which were filled with numerous histiocytes showing active phagocytosis of lymphocytes and lymphoplasmacytoid proliferation in paracortical strands and medullary cord. The disease characteristically has a benign protracted clinical course, and does not respond to either irradiation or chemotherapy. The etiology and pathogenesis of this disorder are unknown. The possible pathologic mechanisms include an abnormal response to a specific infectious process and an immune deficiency status.

Síndrome de Sweet: relato de um caso / Sweet's syndrome: a case report

Os autores relatam um caso de síndrome de Sweet, dermatose neutrofílica febril aguda, mostrando a dificuldade do diagnóstico diferencial com o eritema polimorfo e a sua pronta resposta terapêutica näo só aos corticosteróides orais, como também ta indometacina