Brazilian workshop model to train investigators in chronic graft-versus-host disease clinical trials according to the 2005-2006 National Institutes of Health recommendations

BACKGROUND: The lack of standardization of clinical diagnostic criteria, classification and severity scores of chronic graft-versus-host disease led the National Institutes of Health to propose consensus criteria for the purpose of clinical trials. METHODS: Here we describe a one-day workshop model conducted by the Chronic Graft-versus-Host Disease Brazil-Seattle Consortium Study Group to train investigators interested in participating in multicenter clinical trials in Brazil. Workshop participants included eight transplant physicians, one dermatologist, two dentists, three physical therapists and one psychologist from five institutions. Workshop participants evaluated nine patients with varying degrees of severity of mucocutaneous lesions and other manifestations of the disease followed by a training session to review and discuss the issues encountered with the evaluation and scoring of patients and in the methods used to evaluate grip strength and the 2-minute walk test. RESULTS: Most participants had difficulties in rating the percentage of each type of mucocutaneous lesion and thought 20 minutes was insufficient to evaluate and record the scores of each patient using the National Institutes of Health criteria and other cutaneous assessments. Several specific areas of difficulties encountered by the evaluators were: 1) determining the percentage of erythema in movable and non-movable sclerosis, 2) whether to score all cutaneous findings in a particular area or just the dominant lesion; 3) clarification of the definition of poikiloderma in chronic graft-versus-host disease; 4) discrepant interpretation of the mouth score and 5) clarification on the methodology used for the evaluation of grip strength and the 2-minute walk tests. CONCLUSIONS: Results of this workshop support the need to train investigators participating in clinical trials on chronic graft-versus-host disease.

A multicenter feasibility study of chronic graft-versus-host disease according to the National Institute of Health criteria: efforts to establish a Brazil-Seattle consortium as a platform for future collaboration in clinical trials

BACKGROUND: New criteria for the diagnosis and classification of chronic graft-versus-host disease were developed in 2005 for the purpose of clinical trials with a consensus sponsored by the National Institute of Health. OBJECTIVES: The aim of this study is to present the results of a multicenter pilot study performed by the Brazil-Seattle chronic graft-versus-host disease consortium to determine the feasibility of using these criteria in five Brazilian centers. METHODS: The study was performed after translation of the consensus criteria into Portuguese and training. A total of 34 patients with National Institute of Health chronic graft-versus-host disease were enrolled in the pilot study between June 2006 and May 2009. RESULTS: Of the 34 patients, 26 (76 percent) met the criteria of overlap syndrome and eight (24 percent) the classic subcategory. The overall severity of disease was moderate in 21 (62 percent) and severe in 13 (38 percent) patients. The median time from transplant to onset of chronic graft-versus-host disease was 5.9 months (Range: 3 - 16 months); the median time for the overlap syndrome subcategory was 5.9 months (Range: 3 - 10 months) and for the classic subcategory, it was 7.3 months (Range: 3 - 16 months). At a median follow up of 16.5 months (Range: 4 - 39 months), overall survival was 75 percent. CONCLUSIONS: It was feasible to use the National Institute of Health consensus criteria for the diagnosis and scoring of chronic graft-versus-host disease in a Brazilian prospective multicenter study. More importantly, a collaborative hematopoietic cell transplantation network was established in Brazil offering new opportunities for future clinical trials in chronic graft-versus-host disease and in other areas of research involving hematopoietic stem cell transplantation.

Manifestações tegumentares da doença enxerto contra hospedeiro em pacientes transplantados de medula óssea / Tegumentary manifestations of graft-versus-host disease in bone marrow transplantation recipients

A doença enxerto contra hospedeiro (DECH) é uma síndrome sistêmica que ocorre em pacientes que recebem linfócitos imunocompetentes. A fisiopatologia envolve uma reação imunológica entre linfócitos transplantados e tecidos do hospedeiro, e ocorre por ataque imune das células T do doador às células do hospedeiro, as quais diferem daquelas pelos antígenos de histocompatibilidade. É, assim, uma complicação primária do transplante de medula óssea (TMO) alogênico. O envolvimento cutâneo é freqüente na DECH e contribui para a morbidade e mortalidade do TMO. O dermatologista tem papel importante na avaliação dos pacientes auxiliando no reconhecimento precoce da DECH e suas complicações e no acompanhamento clínico desses pacientes. Nesta revisão os autores enfatizam as manifestações cutâneas da DECH, tendo como base sua experiência pessoal no acompanhamento de pacientes portadores de DECH transplantados de medula óssea no Centro Nacional de Transplante de Medula Ossea/Inca/MS, no Rio de Janeiro, nos últimos 14 anos.

Porfiria cutânea tardia sintomática: relato de um caso / Late symptomatic porphyria cutanea: report of a case

Os autores apresentam um caso de Porfiria cutânea tardia sintomática, associada a alcoolismo e descrevem métodos diagnósticos práticos para identificaçäo das porfirinas na urina

Tratamento de pênfigos: revisäo de 31 casos / Treatment of penphigus: review of 31 cases

A revisäo do tratamento de 16 pacientes com pênfigo foliáceo no período de 1978-1988 no Hospital Universitário Clementino Fraga Filho é apresentada. O diagnóstico foi confirmado pelo exame hsitopatológico e foi usado um curso inicial de doses diárias de prednisona oral, 80 a 120 mg no pênfifo vulgar e 40 a 120 mg no pênfigo foliáceo. A terapêutica mostrou-se eficaz ao final do primeiro mês de tratamento, independentemente das doses empregadas em ambos os quadross. Ao final do sexto mês, um terço dos casos de pênfigo vulgar e metade dos casos de pênfigo foliáceo alcançaram a dose de manutençäo de 10mg. O período de acompanhamento que variou de um a mais de cinco anos mostoru que o percentual de óbitos foi semelhante em ambos os quadros. Os efeitos colaterais, principalmente infecçäo bacterianas e fúngicas, foram mais freqüentes nos casos de pênfigo vulgar