Severe Multi-Resistant Pemphigus vulgaris: prolonged remission with a single cycle of Rituximab / Penfigo vulgar grave Multirresistente: controle prolongado com um unico ciclo de Rituximabe

Pemphigus vulgaris is an autoimmune bullous disease whose therapy is based on systemic corticosteroids, with or without immunosuppressants. Rituximab is a chimeric monoclonal antibody of the IgG class, directed at a specific CD20 B cell surface antigen, used in pemphigus vulgaris empirically since 2002, with success in 90% of the cases and long periods of remission. Male patient, 33 years old, diagnosed with pemphigus vulgaris, confirmed by histopathology and direct immunofluorescence. He was treated for seven months with numerous treatments, including immunosuppressive drugs, with an unsatisfactory response, until he had complete remission with the use of rituximab. During a 34-month follow-up period, the patient presented a slight clinical relapse, which was successfully controlled with prednisone in a daily dose of 120mg, soon reduced to 20mg.

Pênfigo Vulgar é uma doença bolhosa auto-imune, cuja terapêutica é baseada em corticoesteróides sistêmicos, associados ou não a imunossupressores. Rituximabe é um anticorpo monoclonal quimérico da classe IgG direcionado a um antígeno CD20 de superfície celular específico da célula B, usado em pênfigo vulgar desde 2002, com sucesso em 90% e longos períodos de remissão. Paciente masculino, 33 anos, diagnóstico de pênfigo vulgar, confirmado por histopatologia e imunofluorescência direta. Durante 7 meses, recebeu inúmeros tratamentos com imunossupressores, apresentando resposta insatisfatória e progressão da doença, até que logo após a introdução de rituximabe teve completa remissão. Durante um acompanhamento de 34 meses, apresentou leve recidiva clínica, controlada com prednisona 120mg/dia, rapidamente reduzida e em uso atual de Prednisona 20mg/dia.

Mucosa-associated lymphoid tissue lymphoma of the trachea: case report / Linfoma do tecido linfoide associado à mucosa de traqueia: relato de caso

CONTEXT: Mucosa-associated lymphoid tissue (MALT) lymphomas are most commonly found in the stomach, lungs, orbital soft tissue, salivary glands and thyroid. Involvement of the trachea is extremely rare. CASE REPORT: This report describes a rare case of MALT lymphoma of the trachea in a 71-year-old woman who presented with a one-year history of coughing, dyspnea, hoarseness and weight loss. There was an infiltrative lesion in the mid-trachea. The anatomopathological diagnosis was only made from the fifth endoscopic biopsy attempt. Immunochemotherapy consisting of rituximab, cyclophosphamide, vincristine and prednisone (R-COP) induced complete remission of the symptoms and endoscopic lesion. CONCLUSIONS: MALT lymphoma of the trachea is extremely rare and indolent disease. It has to be considered in the differential diagnosis of airway lesions. It is crucial to obtain an anatomopathological diagnosis from a specialized pathologist. Immunochemotherapy with R-COP induced complete remission of the disease.

CONTEXTO: Linfomas de tecido linfoide associado à mucosa (TLAM) são mais comumente encontrados no estômago, pulmões, órbita, glandulas salivares e tireoide. O envolvimento da traqueia é extremamente raro. RELATO DE CASO: Relato de caso raro de linfoma TLAM de traqueia, em mulher de 71 anos com tosse, dispneia, rouquidão e emagrecimento há um ano. Detectou-se lesão infiltrativa na porção média da traquéia. O diagnóstico anatomopatológico só foi obtido na biópsia do quinto procedimento endoscópico. A imunoquimioterapia R-COP (rituximabe, ciclofosfamida, vincristina e prednisona) induziu remissão completa dos sintomas e da lesão endoscópica. CONCLUSÕES: Linfoma TLAM de traqueia é uma doença indolente e extremamente rara, que deve ser considerada no diagnóstico diferencial das lesões de vias aéreas. O diagnóstico anatomopatológico por um patologista experimentado é fundamental. Imunoquimioterapia com R-COP levou à remissão completa da doença.

Sulfasalazine-induced DRESS syndrome (Drug Rash with Eosinophilia and Systemic Symptoms) / Síndrome DRESS (Drug Rash with Eosinophilia and Systemic Symptoms) induzida por sulfasalazina

CONTEXT: DRESS syndrome (Drug Rash with Eosinophilia and Systemic Symptoms) is a type of drug reaction commonly mistaken for a viral infection. It must be recognized promptly due to its high morbidity and 10 percent mortality rate. Few cases of DRESS syndrome induced by sulfasalazine have been reported in the literature. CASE REPORT: The case of a 47-year-old white Brazilian woman who developed DRESS syndrome eight weeks after starting a course of sulfasalazine for treatment of seronegative arthritis is reported. She presented a skin rash, fever, hepatitis, lymphadenopathy, eosinophilia and atypical lymphocytes. The causative drug was discontinued immediately, but she only improved after treatment with prednisone.

CONTEXTO: A síndrome DRESS (Drug Rash with Eosinophilia and Systemic Symptoms) é um tipo de reação a drogas comumente confundida com uma infecção viral. Deve ser prontamente reconhecida devido a sua alta morbidade e taxa de mortalidade de 10 por cento. Poucos casos de síndrome DRESS induzida por sulfasalazina foram relatados na literatura. RELATO DE CASO: É relatado o caso de uma mulher branca, brasileira, de 47 anos, que desenvolveu a síndrome DRESS oito semanas após iniciar tratamento com sulfasalazina para artrite soronegativa. A paciente apresentou rash maculopapular, febre, hepatite, linfadenomegalia, eosinofilia e linfocitose atípica. A medicação foi suspensa imediatamente no início dos sintomas, mas só houve melhora clínica após tratamento com prednisona.

Investigação da trombose venosa na gravidez / Deep vein thrombosis during pregnancy work up

CONTEXTO: A trombose venosa profunda (TVP) na gravidez é fator determinante no aumento da morbidade e da mortalidade maternofetal. Pode ocorrer na presença de trombofilias, por compressão da veia cava inferior, estase venosa ou alterações hormonais. OBJETIVOS: Analisar pacientes grávidas e no pós-parto imediato portadoras de TVP em membros inferiores, pesquisar as possíveis causas de trombofilia e realizar revisão de literatura. MÉTODOS: Foram analisadas gestantes e puérperas encaminhadas por ginecologistas e obstetras com quadro clínico suspeito de TVP, de janeiro de 2004 a novembro de 2006, período em que foram realizados 24.437 partos no Hospital e Maternidade São Luiz (HMSL), sendo 89 por cento cesarianas, 7,5 por cento partos normais e 3,5 por cento fórceps. Do total de pacientes encaminhadas com quadro clínico sugestivo, foram realizados 42 diagnósticos clínicos de TVP em gestantes com idade entre 21 e 39 anos, confirmados por duplex scan venoso. Imediatamente antes da introdução da terapia anticoagulante, foram colhidos exames para pesquisa de trombofilia, os quais foram repetidos após o período de tratamento. RESULTADOS: Das 42 pacientes portadoras de TVP, 32 eram primigestas (três gemelares sem alterações trombofílicas, duas por fecundação in vitro), oito secundigestas e duas tercigestas. Em quatro pacientes, a TVP ocorreu no primeiro trimestre da gestação (9,5 por cento); em 11, no segundo trimestre (26,2 por cento); em 27, no terceiro trimestre (64,3 por cento). Dos 42 casos de diagnóstico de TVP, 18 (42,8 por cento) ocorreram nas veias infrapatelares. Houve um caso de tromboembolismo pulmonar (TEP) em paciente de 37 anos que havia realizado fecundação in vitro, com gestação gemelar, e TVP (ausência de trombofilia) diagnosticada após a cesariana. Das 42 pacientes, 16 (38,1 por cento) tiveram a causa da TVP estabelecida, com prevalência de mutação heterozigótica do fator V de Leiden (FVL) em seis pacientes...

BACKGROUND: Deep venous thrombosis (DVT) during pregnancy is a determining factor that contributes to increased maternal-fetal morbidity and mortality. It may occur when there is thrombophilia, due to compression of the inferior vena cava, venous stasis or hormonal changes. OBJECTIVES: To assess patients who are pregnant or have just given birth and who have a DVT condition in the lower limbs, to search for possible causes of thrombophilia and to perform a review of the literature. METHODS: Pregnant and puerperal patients were assessed by gynecologists and obstetricians when there was suspicion of DVT, from January 2004 through November 2006, during which time there were 24,437 childbirths at Hospital e Maternidade São Luiz; of these, 89 percent were cesarean, 7.5 percent were normal births and 3.5 percent were forceps deliveries. Of the total number of patients referred with a clinical status suggesting DVT, 42 cases were clinically diagnosed as DVT, in pregnant women aged between 21-39 years, confirmed by venous duplex scan. Right before the introduction of anticoagulant therapy, samples were collected to investigate thrombophilia, which were repeated after the treatment. RESULTS: Of the 42 patients with DVT, 32 were primigravid (three twin pregnancies with no thrombophilic changes, two resulting from in vitro fecundation), eight were mothers at second birth and two were at third birth. In four patients, DVT occurred in the first trimester of pregnancy (9.5 percent), in 11 patients DVT was present in the second trimester (26.2 percent) and in 27 patients the disease developed in the third trimester of pregnancy (64.3 percent). Of the 42 patients diagnosed with DVT, 18 (42.8 percent) occurred in infrapatellar veins. There was a case of pulmonary thromboembolism in a 37-year-old patient, who had been submitted to in vitro fecundation, with twin pregnancy and a diagnostic of DVT (no thrombophilia) after a cesarean...

Mediastinal diseases: clinical and therapeutic aspects

Background: Mediastinal affections are common and encompass a great number of different diagnoses. Objective: To analyze the clinical aspects and the therapeutic response of 114 patients with mediastinal diseases treated at the Thoracic Surgery Department of Santa Casa de São Paulo Hospital, from 1979 and 1997. Method: The patients were grouped according to the benign or malignant nature of the disease, and the two groups were compared regarding gender, age bracket, symptomatology, topography of the lesion, mortality, and response to treatment. Results: Sixty-three patients had neoplasia: 31 benign and 32 malignant. Fifty-one cases were not neoplastic. No difference was found between the groups regarding gender or age bracket. Half of the patients were between 20 and 49 years of age. The anterior mediastinum was the most frequently affected compartment (66 patients), followed by the upper mediastinum (18 patients), the posterior mediastinum (16 patients), and the middle mediastinum (14 patients). The most frequent histological types were: benign thymus diseases (N = 40), mesenchymal tumors (N = 17), lymphomas (N = 15), neural tumors (N = 9), and germ cell tumors (N = 8). Malignant tumors were more frequently symptomatic (91 percent), and benign tumors were more frequent in asymptomatic patients (92 percent). The most frequent symptoms were related to myastenia gravis, followed by dyspnea and chest pain. Weight loss, anorexia and fever were significantly more frequent in patients with malignant neoplasias. Conclusions: Regarding the clinical aspects we can state that benign lesions were predominant, that mediastinal diseases were more prevalent in young adults, and that benign lesions were more frequent in asymptomatic patients. Treatment (clinical/surgical) was effective in most patients, benefiting approximately 90 percent of the patients with benign affections and 45 percent of the patients with malignant tumors. In 73 percent of the benign affections, surgical treatment was capable of achieving the cure. Mortality resulting from complications was 1.75 percent.

Bronchial oncocytoma

CONTEXT: Oncocytomas are generally small and present slow growth. Finding of the tumor usually occurs incidentally. Their incidence is higher among male patients. Oncocytomas in mucous bronchial glands are extremely rare. CASE REPORT: A 35-year-old male who presented bronchial oncocytoma. The tumor was found after bronchoscopy that investigated an atelectasis of the upper left lobe. Histological examination with optical microscopy revealed a mature neoplasm formed by ovoid cells with thin, granular, eosinophilic cytoplasm and small nuclei similar to oncocytes. Electron microscopy showed mitochondrial hyperplasia. A three-year follow-up after thoracotomy followed by lobectomy and removal of the bronchial tumor was uneventful