Abstract Background: Adult T-cell leukemia/lymphoma is a peripheral disease associated with human T-cell lymphotropic virus type 1. Treatment is carried out according to clinical type with watchful waiting being recommended for less aggressive types. Aggressive adult T-cell leukemia/lymphoma is generally treated with chemotherapy and/or antivirals. The objective of this study was to correlate the survival of patients diagnosed in Bahia, Brazil, with the therapeutic approaches employed and to evaluate what issues existed in their treatment processes. Methods: Eighty-three adult T-cell leukemia/lymphoma patients (26 smoldering, 23 chronic, 16 acute, 13 lymphoma and five primary cutaneous tumoral) with available data were included in this study. Results: Complete response was achieved in seven smoldering patients with symptomatic treatment, in two with chronic disease using antivirals/chemotherapy, in one with acute disease using antivirals and in one lymphoma using the LSG15 regimen [vincristine, cyclophosphamide, doxorubicin, and prednisolone (VCAP); doxorubicin, ranimustine, and prednisolone (AMP); and vindesine, etoposide, carboplatin, and prednisolone (VECP)]. Smoldering patients who received symptomatic treatment presented longer survival. Favorable chronic patients treated with antivirals presented longer survival compared to the unfavorable subtype. However, for the acute form, first-line chemotherapy was better, albeit without significance, than antivirals. Only one of the patients with lymphoma and primary cutaneous tumors responded. Conclusions: Watchful waiting associated with phototherapy represents the best option for smoldering adult T-cell leukemia/lymphoma with survival in Bahia being superior to that described in Japan. There was a trend of better results with zidovudine/interferon-alpha in favorable chronic disease. Excellent results were achieved in the lymphoma type treated with the LSG15 protocol. Patients are diagnosed late probably due to lack of knowledge of adult T-cell leukemia/lymphoma by primary healthcare doctors and a Brazilian treatment protocol needs to be established.

Abstract Hodgkin-like ATLL is a rare variant of adult T-cell leukemia/lymphoma (ATLL), a disease caused by human T-cell lymphotropic virus type-1 (HTLV-1). At admission, a 46-year-old female presented with lymphadenomegaly, lymphocytosis, slight elevation of LDH blood level, and acid-alcohol resistant bacilli in sputum and was being treated for pulmonary tuberculosis (Tb). She had lymphocytosis in the previous 20 months. Serology for HTLV-1 was positive. Lymph node was infiltrated by medium-sized lymphocytes with scattered Hodgkin and Reed-Sternberg-like cells CD30+, CS1-4+, and CD79a+. Background cells were CD4+ and CD25+. A clinical diagnosis of favorable chronic ATLL was given. She was treated with chemotherapy but later progressed to acute ATLL and ultimately died. Hodgkin-like ATLL should be considered in the histological differential diagnosis with Hodgkin lymphoma since treatment and prognosis of these diseases are distinct. It is also important to search for HTLV-1 infection in patients with unexplained prolonged lymphocytosis.

Resumo A leucemia/linfoma de células T do adulto (LLcTA) é uma neoplasia de células T maduras CD4+ causada pelo vírus linfotrópico para células T humanas tipo 1 (HTLV-1). Acredita-se que existem cerca de 20 milhões de pessoas infectadas em todo o mundo, principalmente no Japão, na África, no Caribe e na América do Sul, particularmen te no Brasil e no Peru. A LLcTA acomete cerca de 5% dos indivíduos infectados e classifica-se nas seguintes formas clínicas: aguda, linfomatosa, tumoral primária de pele, crônica (favorável e desfavorável) e indolente (leucêmica e não leucêmica). Embora seja considerada uma doença agressiva, há casos com longa evolução. Salientamos a importância da classificação clínica como elemento im prescindível para avaliação do prognóstico e conduta terapêutica adequada. Como já foram publicados vários casos no Brasil e essa doença ainda é pouco conhecida, decidimos fazer um trabalho de revisão para divulgar os seus aspectos clínicos, hematológicos, anatomopatológi cos, diagnósticos e terapêuticos. O melhor meio de redu zir a ocorrência de LLcTA seria sustando a transmissão vertical do vírus pela amamentação.

Summary Adult T-cell leukemia/lymphoma (ATL) is a malignancy of mature CD4+ T-cells caused by human T-cell lymphotropic virus type 1 (HTLV-1). Twenty million people are believed to be infected throughout the world, mostly in Japan, Africa, the Caribbean, and South America, particularly in Brazil and Peru. ATL affects about 5% of infected individuals and is classified in the following clinical forms: acute, lymphoma, primary cutaneous tumoral, chronic (favorable and unfavorable), and smoldering (leukemic and non-leukemic). Although it is considered an aggressive disease, there are cases with a long progression. We emphasize the importance of clinical classification as an indispensable element for evaluating prognosis and appropriate therapeutic approach. Since several cases have been published in Brazil and this disease is still poorly known, we decided to make a review paper for dissemination of clinical, hematological and pathological aspects, diagnosis, and therapy. The best way to reduce the occurrence of ATL would be halting the transmission of the virus through breastfeeding.

A leucemia/linfoma de células T do adulto (ATL) é tipo agressivo de doença linfoproliferativa causada pelo vírus linfotrópico para células T humanas (HTLV-I), geralmente fatal e que não responde a quimioterapia. Classifica-se em formas aguda, crônica, linfomatosa e indolente (smoldering). Outra forma clínica, a tumoral primária de pele, com características diferentes, foi sugerida recentemente. As formas aguda, linfomatosa e tumoral primária de pele são as de pior prognóstico. Os critérios diagnósticos de ATL são: sorologia positiva para o HTLV-I; diagnóstico citológico ou histológico de leucemia/linfoma de células T, CD4+/CD25+; presença de linfócitos T anormais em sangue periférico; confirmação de integração monoclonal do DNA proviral do HTLV-I. Há lesões de pele em cerca de 70% dos casos,que podem ser primários (formas indolente e tumoral primária da pele) ou secundários. As lesões cutâneas são múltiplas, sendo as mais freqüentes a eritrodermia, as pápulas e as placas. A ATL não tem aspecto histológico característico, podendo apresentar padrões superponíveis ao linfoma periférico T não especificado, à micose fungóide ou ao linfoma anaplásico de grandes células. O padrão imuno-histoquímico pode também simular o de outros tipos de linfoma T. Por esse motivo, é muito importante que no Brasil seja solicitada sorologia para o HTLV-I em todos os casos de leucemia e/ou linfoma de células T maduras.

Adult T cell leukemia/lymphoma (ATL) is an aggressive type of lymphoproliferative disease associated with the human T-cell lymphotropic virus type I (HTLV-I) that is characterized by a short survival time and absence of response to chemotherapy. ATL is classified into four clinical types: acute, chronic, lymphoma, and smoldering. Another clinical form of ATL, the primary cutaneous tumoral,with diverse characteristics, has been recently suggested. Patients with acute, lymphoma and primary cutaneous tumoral types have a poor prognosis. The diagnostic criteria of ATL consist of: positive serology for HTLV-I; cytologic or histologic confirmation of CD4+/CD25+ T-cell leukemia/lymphoma; abnormal T lymphocytes in peripheral blood; and confirmation of monoclonal integration of HTLV-I proviral DNA. There is skin involvement in around 70% of ATL cases, which could be primary (smoldering and primary cutaneous tumoral) or secondary. The skin lesions are multiple, erythroderma, papules and plaques being the most common. ATL has no characteristic histological pattern, and may present patterns that could superimpose nonspecific peripheral T-cell lymphoma, mycosis fungoides or anaplastic large cell lymphoma. The immunohistochemistry pattern may also be similar to that of other T-cell lymphomas. Thus, it is very important that in Brazil HTLV-I infection be investigated in all mature T-cell leukemias/lymphomas.

OBJETIVOS: Revisão da literatura sobre doenças relacionadas à infecção pelo vírus linfotrópico de células T humanas (HTLV-I) na infância e adolescência, focalizando clínica, diagnóstico, patogênese, evolução e tratamento. FONTES DOS DADOS: Literatura médica dos últimos 20 anos utilizando PubMed e MEDLINE e livros médicos especializados, com ênfase na dermatite infecciosa associada ao HTLV-I (DIH), na forma infanto-juvenil da mielopatia associada ao HTLV/paraparesia espástica tropical (HAM/TSP), na leucemia/linfoma de células T do adulto (ATL) e na uveíte associada ao HTLV-I. Palavras-chave usadas na pesquisa: dermatite infecciosa associada ao HTLV-I, mielopatia associada ao HTLV/paraparesia espástica tropical, leucemia/linfoma de células T do adulto, uveíte associada ao HTLV-I. SÍNTESE DOS ACHADOS: A DIH é uma dermatite crônica, recidivante e infectada da infância que sempre envolve o couro cabeludo e que pode evoluir para HAM/TSP e ATL. A HAM/TSP é uma mielopatia crônica e incapacitante do adulto. Há 17 casos infanto-juvenis de HAM/TSP bem documentados na literatura, 12 dos quais em pacientes com DIH. Ao contrário da doença no adulto, essa forma é rapidamente progressiva. A ATL é uma leucemia/linfoma T do adulto, geralmente fatal. De 24 casos infanto-juvenis de ATL da literatura, 11 foram diagnosticados no Brasil. CONCLUSÕES: Essas doenças devem ser mais freqüentes na infância e adolescência do que indica a literatura. É aconselhável fazer sorologia para o HTLV-I em crianças e adolescentes com eczema crônico e recidivante, com sintomas e sinais de mielopatia ou com diagnóstico de leucemia/linfoma de células T. É importante que os pediatras saibam reconhecer as manifestações pediátricas dessa infecção para diagnosticá-las corretamente, propiciando aos pacientes orientação e tratamento adequados.

OBJECTIVES: To review the literature on diseases linked with infection by human T-cell lymphotropic virus type I (HTLV-I) in childhood and adolescence, with focus on clinical aspects, diagnosis, pathogenesis, progression and treatment. SOURCES: Medical literature published during the last 20 years identified using PubMed and MEDLINE and from specialized medical books, with emphasis on infective dermatitis associated with HTLV-I (IDH), on the juvenile form of HTLV-associated myelopathy/tropical spastic paraparesis (HAM/TSP), on adult T-cell leukemia/lymphoma (ATL) and on HTLV-I-associated uveitis. Keywords used to search databases were: HTLV-I-associated infective dermatitis, HTLV-I-associated myelopathy/tropical spastic paraparesis, adult T-cell leukemia/lymphoma, HTLV-I-associated uveitis. SUMMARY OF THE FINDINGS: IDH is a chronic, relapsing and infected dermatitis of childhood which always involves the scalp and which may progress to HAM/TSP and ATL. HAM/TSP is a chronic and incapacitating myelopathy of adults. There are 17 well-documented cases of HAM/TSP in children and adolescents in the literature, 12 of whom are patients with IDH. In contrast with the adult form of the disease, the juvenile form is rapid and progressive. ATL is a type of T-cell leukemia/lymphoma that affects adults and is generally fatal. Eleven of the 24 published reports of ATL in children and adolescents were diagnosed in Brazil. CONCLUSIONS: These diseases are likely to be more common in childhood and adolescence than the literature would suggest. It is advisable that serological testing be performed for HTLV-I in children and adolescents suffering from chronic and relapsing eczema, with signs and symptoms of myelopathy or with a diagnosis of T-cell leukemia/lymphoma. It is important that pediatricians know how to recognize the pediatric manifestations of this infection in order to correctly diagnose them and offer their patients appropriate guidance and treatment.

A dermatite infecciosa associada ao HTLV-I (DIH) é um tipo de eczema infectado e recidivante que incide em crianças que adquirem verticalmente a infecção pelo HTLV-I. Inicia-se após os 18 meses de idade. No entanto, existe relato recente de início na vida adulta. As lesões são eritemato-descamativas, infectadas e freqüentemente crostosas. Localizam-se, com maior freqüência, no couro cabeludo, regiões retroauriculares, pescoço e região inguinal, mas podem ser generalizadas. Vêem-se também físsuras retroauriculares, pápulas eritêmato-descamativas e foliculares. Prurido discreto a moderado, secreção nasal crônica e blefaroconjutivite podem ser observados. A DIH sempre se associa à infecção por Staphylococcus aureus e/ou Streptococcus beta hemoliticus. O diagnóstico diferencial clínico deve ser feito com as dermatites atópica e seborréica e, do ponto de vista anatomopatológico, com a psoríase e o linfoma cutâneo de células T. São discutidos os aspectos clínico-imunopatológicos, o diagnóstico diferencial e a evolução da DIH. Considerando a freqüência da DIH em Salvador, aconselha-se que seja feita sorologia para o HTLV-I em todos os casos de eczema severo em crianças e que essa possibilidade diagnóstica também seja considerada em adultos.

Infectious dermatitis associated with HTLV-I (IDH) is a form of infected and recurrent dermatitis which affects children vertically infected with HTLV-I. IDH starts after 18 months of age. The lesions are erythematous-scaly and frequently crusty and are more frequently located on the scalp and on the retroauricular regions, but the lesions may be generalized. Pustules, erythematous-scaly and follicular papulae and retroauricular fissures may also be observed. Children present mild to moderate pruritus, lesions in the nostrils, and blepharoconjunctivitis. IDH is always associated with Staphylococcus aureus infection and/or Streptococcus beta haemolyticus infection. The differential diagnosis with atopic and seborrheic dermatitis can be made clinically. Histologically it is important to make a differential diagnosis with psoriasis and cutaneous T-cell lymphoma. We discuss here the clinical-immuno-pathological aspects, the differential diagnosis, and the evolution of IDH. Considering the frequency of IDH in Salvador, Bahia, we suggest that children with severe eczema be submitted to routine serology for HTLV-I and that IDH be included in the differential diagnosis of dermatitis in adulthood.

We report the case of a 44 year-old female, who presented a long-lasting, clinically atypical, secondary syphilis ("malignant syphilis") in the right foot, which started six months before medical evaluation. The patient had a serological diagnosis of HTLV-I infection and syphilis two years before the onset of the skin lesions, following a blood donation. As she believed she was allergic to penicillin, she initially received sulfamethoxazole + trimethoprim, without any improvement of the clinical picture. After failure of this first treatment regimen, she was given penicillin, which promoted complete healing of the lesion. We found evidence that infection by HTLV-I is capable of modifying the clinical course of secondary syphilis.

Post-kala-azar dermal leishmaniasis (PKDL) is rarely reported in South America. In spite of the fact that there are many reports about the association of visceral leishmaniasis and AIDS, PKDL is very uncommon in HIV-positive patients, and so far only four cases have been documented in the literature. We present another case with unusual clinicopathological aspects. The patient, a 28-year-old male, from Salvador, Bahia (an endemic area) presented with clinical manifestations of visceral leishmaniasis three years after the diagnosis of AIDS. During treatment for visceral leishmaniasis he developed disseminated miliary papules. Microscopically, the skin biopsy showed a "saw-tooth" pattern with a lichenoid mononuclear infiltrate simulating lichen planus. The histopathological diagnosis was achieved through the finding of amastigotes. The authors discuss the clinicopathological aspects of this case based on a review of the specific literature.

Post-kala-azar dermal leishmaniasis (PKDL) is rarely reported in South America. In spite of the fact that there are many reports about the association of visceral leishmaniasis and AIDS, PKDL is very uncommon in HIV-positive patients, and so far only four cases have been documented in the literature. We present another case with unusual clinicopathological aspects. The patient, a 28-year-old male, from Salvador, Bahia (an endemic area) presented with clinical manifestations of visceral leishmaniasis three years after the diagnosis of AIDS. During treatment for visceral leishmaniasis he developed disseminated miliary papules. Microscopically, the skin biopsy showed a "saw-tooth" pattern with a lichenoid mononuclear infiltrate simulating lichen planus. The histopathological diagnosis was achieved through the finding of amastigotes. The authors discuss the clinicopathological aspects of this case based on a review of the specific literature.

A amamentação é o meio mais freqüente de transmissão vertical do HTLV-I. No entanto, crianças não amamentadas mostram-se infectadas em freqüências que variam de 4 a 14%. Nestes casos, os meios mais prováveis de infecção devem ser através da placenta ou por contaminação no canal de parto. Quarenta e um filhos de portadoras do HTLV-I aleitados artificialmente foram submetidos a pesquisa do vírus pela reação em cadeia da polimerase. 81,5% destas crianças nasceram através de cesária eletiva. Nenhum caso de infecção pelo HTLV-I foi detectado. Este fato indica que a infecção por via transplacentária é pouco freqüente e que é provável que o parto artificial tenha contribuído para a ausência de transmissão do vírus.

The most frequent pathway of vertical transmission of HTLV-I is breast-feeding, however bottle fed children may also become infected in a frequency varying from 4 to 14%. In these children the most probable routes of infection are transplacental or contamination in the birth canal. Forty-one bottle-fed children of HTLV-I seropositive mothers in ages varying from three to 39 months (average age of 11 months) were submitted to nested polymerase chain reaction analysis (pol and tax genes). 81.5% of the children were born by an elective cesarean section. No case of infection was detected. The absence of HTLV-I infection in these cases indicates that transmission by transplacental route may be very infrequent.

O presente relato descreve um caso de prototecose cutânea causada por Prototheca wickerhamii em uma mulher não imunodeprimida do estado da Bahia, Brasil. Este é o segundo caso descrito no Brasil. O exame dermatológico revelou placas eritematosas infiltradas na superfície flexora do braço e antebraço direitos. Os autores enfatizam os aspectos anatomopatológicos que podem levar a erro diagnóstico. A paciente foi tratada com sucesso com fluconazole.

The present report describes a case of cutaneous protothecosis caused by Prototheca wickerhamii in a non-immunocompromised female from the state of Bahia, Brazil. This is the second case described in Brazil. Dermatological examination revealed diffusely infiltrated erythematous plaques on the flexor aspect of the right arm and forearm. The authors emphasize the pathological aspects that can lead to misdiagnosis this condition. The patient was successfully treated with fluconazole.

O ameloblastoma é um tumor bastante incomum na cavidade nasal. Surge a partir do epitélio odontogênico, podendo estender-se ao seio maxilar, órbitas e base de crânio. Apresentamos dois casos de ameloblastoma em fossa nasal direita, associados a sintomas nasossinusais e seus principais achados, clínicos e cirúrgicos.

Ameloblastoma is a very unusual tumor in the nasal cavity. It arises from the odontogenic epithelium and extends up to the maxillary sinus, orbits and skull base. We have presented two cases of ameloblastoma, both in the right nasal cavity, associated with nasal and sinusal symptoms, and reported the main surgical and clinical findings.

Em 1993, um surto leishmaniose tegumentar americana (LTA) foi detectado no povoado rural de Canoa, município de Santo Amaro, Bahia. Um estudo observacional prospectivo delineou-se, com objetivo de determinar as taxas de freqüência e caracterizar clinicamente a doença. Foram acompanhados 555 indivíduos, registrando-se 29 casos de LTA, 11 casos sugestivos de LTA pregressa e 529 sadios. Desses 529 sadios, 65 apresentaram reação de Montenegro positiva sem qualquer evidência presente ou passada de doença. A prevalência de LTA no período de estudo foi de 5,2% (29/555). A leishmania envolvida foi caracterizada como Leishmania braziliensis e o vetor, Lutzomyia intermedia. Foram detectados cães e equídeos infectados por leishmania O acometimento de crianças menores de 10 anos, o acometimento igual entre os sexos e um componente de agregação familiar sugerem um padrão de transmissão peri ou intradomiciliar.

An outbreak of American cutaneous leishmaniasis (ACL) was detected in the village of Canoa in 1993. A prospective observational study was outlined to determine the frequency rates and to clinically characterize the disease. A total of 555 people were followed up. There were 29 cases of ACL, 11 cases of probably previous ACL (scars) and 529 healthy individuals. Of these 529 individuals, 65 had a positive Montenegro reaction without any present or past evidence of leishmaniasis. The prevalence of ACL during the two years was 5.2% (29/555). The leishmania involved was Leishmania braziliensis and the vector, Lutzomyia intermedia. Evidence of infection was detected in dogs and horses. The high frequency of the disease among children under ten years, the similar sex distribution of cases and a component of familial aggregation suggest a peri- or intra-domiciliary transmission.