Evaluation of secondary malignancies in a large series of mycosis fungoides.

BACKGROUND: An increased risk of Secondary Malignancies (SMs) in Mycosis Fungoides (MF) has been suggested previously. However, the relationship between this risk and the features of MF is not well-known. OBJECTIVE: To investigate the rate and types of SMs in a large cohort of MF patients focusing on the associated features of these patients. METHODS: The demographic features, subtype, and stage of MF, as well as the temporal relationship between the diagnosis of MF and the development of SMs were determined. Major clinical features of MF in this group were compared with MF patients without association of SMs. RESULTS: Among 730 MF patients with a mean follow-up period of 67.9 ± 52.4 months, 56 SMs were identified in a total of 52 (7.1%) patients. While 28.8% of patients were previously diagnosed with other malignancies, then subsequently had a diagnosis of MF, it was vice versa in 53.8% of patients. Most of the SM-associated MF patients had early-stage (80.7%) and classical type of MF (86.5%) without a significant difference from MF patients without association of SMs; 85.5% and 72.5%, respectively. The most commonly identified SMs were hematologic malignancies (64.3%) including lymphomatoid papulosis (n = 22), Hodgkin's lymphoma (n = 4), non-Hodgkin's lymphoma (n = 5), polycythemia vera (n = 2). Other most commonly associated malignancies were breast cancer (n = 4), prostate cancer (n = 3), renal cell carcinoma (n = 2), melanoma (n = 2), and Kaposi's sarcoma (n = 2). STUDY LIMITATIONS: A single tertiary dermatology center study with a retrospective design. CONCLUSION: Apart from the well-known lymphomatoid papulosis association, systemic hematological malignancies were also quite common in the large cohort of MF patients.

Characteristics and outcomes of 727 patients with mycosis fungoides and Sézary syndrome from a Brazilian cohort.

BACKGROUND: Mycosis fungoides (MF) and Sézary syndrome (SS) are the most prevalent cutaneous lymphomas. They were not described in a large Brazilian cohort yet. We aimed, with this single-center, retrospective cohort analysis, to describe the characteristics and outcomes of MF/SS in a tertiary public health service in Brazil. METHODS: MF/SS patients evaluated at the University of São Paulo Medical School between 1989 and 2018 were included. Data were collected at diagnosis. Demographic, clinical, histopathological, immunopathological, molecular, laboratory, and follow-up data were analyzed. RESULTS: Among 727 patients, 92.6% (673) were diagnosed with MF, 7.4% (54) with SS. There were 51.2% (372) of males, 48.8% (355) of females. The median age was 51.8 years; it was higher in erythrodermic MF (60.2) and SS (60.9). Among MF, 41.8% (281) had classic MF, 4.9% (33) folliculotropic MF, 1.8% (12) granulomatous slack skin, and 0.3% (2) pagetoid reticulosis. Common subtypes included erythrodermic (14.1%, 95), hypopigmented (10.8%, 73), and poikilodermatous MF (10.8%, 73). Extracutaneous involvement was rare. Five, 10, 20, and 30-year overall survival rates were 97.3%, 92.4%, 82.6%, and 82.6% for early-stage, and 58.6%, 42.7%, 20.8%, and 15.4% for advanced-stage disease, respectively. After multivariate analysis, SS diagnosis, folliculotropic MF, erythrodermic MF, clinical stage, age (≥60 years), increased lactate dehydrogenase, and large cell transformation conferred poorer prognosis. CONCLUSIONS: We observed a higher percentage of hypopigmented MF compared to the literature, and demographic (older age) and prognostic (poorer prognosis) similarities between erythrodermic MF and SS, suggesting a possible relationship between these erythrodermic lymphomas. Factors associated with a poorer prognosis were compatible with the literature.

Mycosis Fungoides in Puerto Rico: A 15-year Follow-up Retrospective Study.

OBJECTIVE: To describe the patient population with mycosis fungoides (MF) in Puerto Rico in terms of demographics, disease course, and management. METHODS: We performed a retrospective chart review of patients with MF who were being followed at the University of Puerto Rico dermatology clinics from 1999 through 2016. Collected data included demographics, age at diagnosis, stage at diagnosis, follow-up time, treatment, and stage at the time of the study. RESULTS: A total of 53 patients were diagnosed with MF from 1999 through 2016, with a mean follow-up period of 89 months. Of those, 45% were male. At the time of diagnosis, 40% were at stage 1A, 53% were at stage 1B, and 7% were at stages 2 to 4. During data collection, 74% of the patients remained stable, 18% improved clinically, and 8% progressed in disease stage. The treatment modalities used included phototherapy, topical steroids, topical and systemic retinoids, methotrexate, topical and systemic chemotherapy, and interferon. CONCLUSION: Our study reflects the chronic and indolent course of MF, which has an overall good prognosis if diagnosed at an early stage, as has been demonstrated in the recent literature. The information contained within this manuscript should contribute to the understanding and characterization of MF in patients in Puerto Rico.

Primary cutaneous lymphoma in Argentina: a report of a nationwide study of 416 patients.

BACKGROUND: The aim of this study was to determine the relative frequency of primary cutaneous lymphoma (PCL) in Argentina according to the new World Health Organization (WHO)-European Organization for the Research and Treatment of Cancer (EORTC) classification system. METHODS: A total of 416 patients from 21 dermatology services were included during a 5-year period (2010-2015); these patients were classified using WHO-EORTC criteria. RESULTS: There were 231 (55.2%) males and 185 (44.8%) females; the male-to-female ratio was 1.35. The median age of the patients was 57 years (range, 0-90 years). Most patients were Caucasian (79%), and only 16% of patients were registered as Amerindian. Most patients (387/416, 93%) had cutaneous T-cell lymphoma (CTCL); 28 patients (6.7%) were diagnosed with cutaneous B-cell lymphoma (CBCL). The most frequent CTCL subtypes, in decreasing order of prevalence, were mycosis fungoides (MF), including its variants (75.7%); CD30+ primary cutaneous lymphoproliferative disorders (7.2%); and Sézary syndrome (SS) (3.1%). Cutaneous follicle center lymphoma was the most common CBCL subtype (2.9%). In the subset of patients ≤20 years of age, the most common condition was MF (57%), followed by extranodal NK-T nasal-type lymphoma (14%). CONCLUSIONS: This study revealed relatively higher rates of MF and lower rates of CBCL in Argentinean patients that have been reported in American and European countries.

Clinical and epidemiological profile of patients with early stage mycosis fungoides.

BACKGROUND: Mycosis fungoides is the most common form of primary cutaneous lymphoma, with an indolent, slowly progressive course and 88% five-year survival rate. The diagnosis is challenging, especially in the early stages, and usually relies on a good clinical-histopathological correlation. OBJECTIVE: The aim was to establish the clinical and epidemiological profile of patients with early-stage mycosis fungoides. METHODS: This was a retrospective cross-sectional observational study with an exploratory analysis. Outcome variables were disease progression and mycosis fungoides-related death. RESULTS: One hundred and two patients were included. The majority were white males, with a mean age of 55.6 years. Mean time from onset of lesions to diagnosis was 51.08 months. The majority of patients were classified as IB stage according to TNMB. Mean follow-up time was 7.85 years. Disease progression was seen in 29.4% of the patients. Death related to the disease occurred in 7.9% of patients. Plaque lesions, involvement of more than 10% of the body surface, altered lactate dehydrogenase and beta-2-microglobulin, and stage IB were significantly associated with disease progression, and altered lactate dehydrogenase and beta-2-microglobulin also correlated with higher frequency of deaths. STUDY LIMITATIONS: Small sample and retrospective design. CONCLUSIONS: The clinical and epidemiological profile of patients with early-stage mycosis fungoides in our sample corroborates reports in the literature. Diagnostic delay in our series is also consistent with previous findings, but the rate of disease progression, despite treatment, was higher than reported in the literature.

Clinical and epidemiological profile of patients with early stage mycosis fungoides

Abstract: Background: Mycosis fungoides is the most common form of primary cutaneous lymphoma, with an indolent, slowly progressive course and 88% five-year survival rate. The diagnosis is challenging, especially in the early stages, and usually relies on a good clinical-histopathological correlation. Objective: The aim was to establish the clinical and epidemiological profile of patients with early-stage mycosis fungoides. Methods: This was a retrospective cross-sectional observational study with an exploratory analysis. Outcome variables were disease progression and mycosis fungoides-related death. Results: One hundred and two patients were included. The majority were white males, with a mean age of 55.6 years. Mean time from onset of lesions to diagnosis was 51.08 months. The majority of patients were classified as IB stage according to TNMB. Mean follow-up time was 7.85 years. Disease progression was seen in 29.4% of the patients. Death related to the disease occurred in 7.9% of patients. Plaque lesions, involvement of more than 10% of the body surface, altered lactate dehydrogenase and beta-2-microglobulin, and stage IB were significantly associated with disease progression, and altered lactate dehydrogenase and beta-2-microglobulin also correlated with higher frequency of deaths. Study limitations: Small sample and retrospective design. Conclusions: The clinical and epidemiological profile of patients with early-stage mycosis fungoides in our sample corroborates reports in the literature. Diagnostic delay in our series is also consistent with previous findings, but the rate of disease progression, despite treatment, was higher than reported in the literature.

Immune privilege disruption in folliculotropic mycosis fungoides: investigation of major histocompatibility complex antigen expression.

BACKGROUND: Folliculotropic mycosis fungoides (FMF) is a cutaneous T-cell lymphoma mainly affecting the hair follicle, which seems to represent a place of immune privilege phenomenon. OBJECTIVES: To explore a possible role of immune privilege (IP) in FMF analyzing the major histocompatibility complex (MHC) expression. METHODS: Immunohistochemistry for HLA-G and MHC-II was performed to formalin-fixed paraffin-embedded cutaneous skin biopsies of FMF patients (n = 43), conventional mycosis fungoides (CMF; n = 13), alopecia areata (AA; n = 13), and normal scalp skin (NS; n = 12). RESULTS: HLA-G expression was lower in FMF (34%: 14/41) and CMF (18%: 2/11) groups compared to alopecia areata (92%:11/12) and normal scalp skin group (100%: 12/12). MHC-II expression in hair follicle was greater in the FMF group (18/42: 43%) compared to AA (0%) and NS (0%). HLA-G and MHC-II expression in cellular infiltrate had no difference among FMF and CMF groups and was different compared to the AA group. CONCLUSIONS: Our data support the hypothesis of disruption of immune privilege based on the lower expression of HLA-G and higher expression of MHC-II in the follicular epithelium in mycosis fungoides compared to alopecia areata and normal scalp skin. The lack of difference between FMF and CMF groups did not support the role of these molecules as a driver of folliculotropism. The expression of MHC molecules seems to be different between neoplastic and inflammatory infiltrates. The definitive significance of expression of the MHC molecules remains unclear, and more studies are necessary to fully understand the role of these molecules in cutaneous lymphomas.

Síndrome de Sézary. Presentación de un caso / Sézary síndrome. Case presentation

RESUMEN El síndrome de Sézary constituye la fase leucémica de la micosis fungoide caracterizado por eritrodermia, adenopatías superficiales y células atípicas en sangre. Predomina en los hombres con una proporción 2/1 respecto a las mujeres, y en las edades entre los 60 y 70 años de edad. La enfermedad es de difícil tratamiento, con un pronóstico reservado por su baja supervivencia. Por ser infrecuente y su posible similitud con otras dermatosis, se presenta un caso con antecedentes de psoriasis vulgar con 5 años de evolución, que hacía aproximadamente 6 meses, se encontraba sin mejoría en brote de agudización a pesar de los tratamientos indicados (AU).

ABSTRACT Sezary syndrome is the leukemic part of the fungoid mycosis, characterized by erythroderma, surface adenopathies and atypical cells in blood. It predominates in men with a 2/1 proportion in respect to women, and in ages ranging from 60 to 70 years. It is a difficult treated disease, with a reserved prognosis because of the low survival. Due to its infrequency and possible similarity to other dermatosis, it is presented a case with antecedents of vulgar psoriasis of 5 years evolution, without improvement for around 6 months, in acute outbreak in spite of the indicated treatments (AU).

Hypopigmented mycosis fungoides: a 20-case retrospective series.

BACKGROUND: Hypopigmented mycosis fungoides (hMF) is a rare subtype of mycosis fungoides. The aim of this study was to identify the clinical-epidemiological profile of our patient group and also to provide additional information about treatment responses and prognosis. METHODS: This is a cross-sectional retrospective observational study, with exploratory analysis. The outcome variables were disease progression and related death. RESULTS: Twenty patients with hMF were selected from a group of 102 patients diagnosed with MF. There was no gender difference (10 females and 10 males). Mean age at diagnosis was 43.85 years, and most patients had mixed or black skin color. The mean time between the onset of the lesions and the diagnosis was 66.75 months. Patients were equally distributed in stages IA (50%) and IB (50%). Photochemotherapy (psoralen and ultraviolet A) was the predominant therapeutic modality. The mean follow-up time was 7.25 years. In 10%, disease progression was observed. Death related to the disease occurred in one patient. CONCLUSIONS: The clinical and epidemiological profile of patients with hypopigmented MF found in our sample is in agreement with what is described in the literature, with the exception of the age at diagnosis, higher than expected. Diagnostic delay time, despite long, is also consistent with the medical literature; however, in this sample, we had two cases of disease progression, with death of one patient, despite the treatment, which is extremely important since hypopigmented MF is usually associated with good prognosis.

Mycosis Fungoides: Experience in a Pediatric Hospital. / Micosis fungoide. Experiencia en un hospital pediátrico.

Mycosis fungoides (MF), the most common primary cutaneous T-cell lymphoma, is unusual in children. OBJECTIVES: We aimed to describe the epidemiologic, clinical, histopathologic, and immunophenotypic characteristics of MF as well as treatments and course of disease in a pediatric case series. MATERIAL AND METHOD: Data for all patients admitted to our pediatric hospital (Hospital Dr. J. P. Garrahan) in Argentina with a clinical and histopathologic diagnosis of MF between August 1988 and July 2014 were included. RESULTS: A total of 14 patients were diagnosed with MF. The ratio of boys to girls was 1:1.33. The mean age at diagnosis was 11.23 years (range, 8-15 years). The mean time between onset and diagnosis was 3.5 years (range, 4 months-7 years). All patients had hypopigmented MF and 42% also presented the features of classic MF. Seven (50%) had the CD8+ immunophenotype exclusively. Seventy-eight percent were in stage IB at presentation. Phototherapy was the treatment of choice. Four patients relapsed at least once and skin lesions progressed in 3 patients. All patients improved. CONCLUSIONS: MF is unusual in children. The hypopigmented form is the most common. Diagnosis is delayed because the condition is similar to other hypopigmented diseases seen more often in childhood. Although prognosis is good, the rate of recurrence is high, so long-term follow-up is necessary.

Folliculotropic mycosis fungoides: clinical and epidemiological evaluation in a single center in Brazil.

BACKGROUND: Folliculotropic mycosis fungoides (FMF) is a rare variant of mycosis fungoides with clinical peculiarities, refractoriness to conventional treatments, and worse prognosis when compared to classic mycosis fungoides. OBJECTIVE: To evaluate the clinical and epidemiological characteristics of FMF patients in a single center in Brazil. METHODS: Data were retrospectively collected from patients with FMF who attended the Cutaneous Lymphoma Clinic, University of São Paulo Medical School, between 1987 and 2013. RESULTS: Thirty-three patients were included (median age 46 years old at diagnosis; 20 male; 27 white). The median disease duration before diagnosis was 3 years. Regarding stage at diagnosis, 61% had advanced stage disease (≥IIb). Follicular papules were reported in 66% and alopecia in 59% of the cases. The most involved regions were limbs, followed by trunk and head. Pruritus was present in 81% of the patients. The median time of patients' follow-up was 38 months. At the last follow-up visit, 67% of the patients were alive with active disease, 27% deceased, and 6% were in complete remission. Four patients had large cell transformation. At the time of diagnosis, 25% of the patients showed eosinophilia. LIMITATIONS: Retrospective study with partial unavailable data. CONCLUSIONS: The characteristics of our patients with FMF correlated with aspects previously described in the literature, which were at a more advanced stage at diagnosis and had a less favorable outcome. Pruritus is a very common complaint. Large cell transformation should be monitored as it is implicated in poor prognosis.

Evaluación de los criterios diagnósticos-clínicos y anatomo patológicos en pacientes con micosis / Fungoides diagnostic criteria evaluation of the clinic anda anatomo pathology in patients with mycosis fungoides

Se realizó un estudio retrospectivo para evaluar cuáles eran las características clínicas y anatomo patológicas para el diagnóstico de los pacientes con micosis fungoides (MF) tratados en el servicio de dermatología del CES Sabaneta entre abril de 1997 y mayo de 2004. Además se buscaba describir las características sociodemográficas locales y contrastarlas con el perfil epidemiológico descrito en la literatura. Materiales y métodos: la muestra estuvo conformada por 43 casos con diagnóstico de MF a los que se les revisó la historia clínica y las placas histológicas fueron evaluadas por dos patólogos separadamente. Resultados: la edad media de los pacientes fue de 57,7 años, la mayoría fueron mujeres de fototipo II. El 58,1% presentó compromiso de la superficie corporal mayor al 11% y la lesión semiológica mas frecuente fue la placa. Las áreas mas afectadas fueron las extremidades inferiores luego la espalda, extremidades superiores y por último abdomen. Sólo 12 pacientes tenían un diagnóstico previo, el más frecuente fue la parapsoriasis de grandes placas. El 71% de los casos se diagnosticó en la primera biopsia. Los hallazgos histológicos más frecuentes entre los dos patólogos fueron: la presencia de infiltrado inflamatorio a nivel papilar y perivascular; epidermotropismo lineal y pagetoide; las microabscesos de Pautrier y la fibrosis de la dermis papilar. Al comparar los diagnósticos entre los dos patólogos, sólo los microabscesos de Pautrier mostraron concordancia.

A retrospective study was made to evaluate clinical characteristics and anatomic pathological for the diagnosis of patients with mycosis fungoides (MF) treated in the dermatology service of the CES Sabaneta from April 1997 until May 2004. In addition, a description of the local socio demographics characteristics were looked for and contrast them with the epidemiologist profile described in literature. Materials and methods: The sample was conformed by 43 cases with diagnose of MF, in which were review their clinical history and the histological plates were evaluated by two pathologists separately. Results: The average age of the patients was of 57, 7 years; the majority was women of phototype II. 58,1% had affected a greater corporal surface above 11% and the frequents semiologic injury was the plate. The more affected areas were the inferior extremities then the back, superior extremities and finally the abdomen. Just 12 patients had previous diagnosis; the most frequent was the parapsoriasis of great plates. In this study 71% of the cases were diagnosed in the first biopsy, the most frequent histologists findings made by the two pathologists were: the presence of infiltrated inflammatory at papillary level and perivascular, linear and pagetoid epidermotropism, the microabscesses of Pautrier and the fibrosis of the papillar dermis. When those findings were compared by the two pathologists through the index of Kappa with a confidence level of 95%, just the microabscesses of Pautrier showed agreement.

Micosis fungoide en estadio de placas / Mycosis fungoide in stage of plates

La micosis fungoide es una neoplasia maligna originada en los linfocitos T, usualmente inicia con la aparición de manchas planas o estadio de mancha, con el correr de los años las lesiones van siendo infiltradas apareciendo las placas o estadio de placas. Presentamos a una paciente de 62 años de edad con hallazgos clínicos e histopatológicos de micosis fungoide en estadio de placas que cursa con compromiso ganglionar. Se hace una breve revisión de la presentación clínica de la enfermedad, su histopatología y tratamiento.

Mycosis fungoides is a malignant neoplasm originated in T lymphocytes, usually it initiates with the appearance of flat spots or stage of spot and as time goes by the injuries are infiltrated appearing the plates or stage of plates. Let's sense beforehand a 62-year-old patient of age with clinical finds and histopathologic of mycosis fungoide in stage of plates that it deals with commitment ganglionar. There is a brief review of the clinical presentation, histopathology and treatment of the disease.

Linfomas cutáneos: experiencia clínica a diez años del servicio de dermatología del Hospital Clínico Regional de Valdivia / Cutaneous lymphomas: clinical experience in 10 years of dermatology service at Hospital Clínico Regional, Valdivia

Los linfomas son transformaciones neoplásicas que residen sobre todo en el tejido linfoide. Dentro de éstos se encuentran los linfomas cutáneos de células T (LCCT), variedad que incluye micosis fungoide (MF) y síndrome de Sézary (SS). Si bien estas dos entidades difieren entre sí por sus manifestaciones clínicas, se cree que son variantes de un mismo trastorno linfoproliferativo de linfocito T CD4+, con tropismo hacia piel. Entre 1988 y 1998, en el Servicio de Dermatología del Hospital Regional de Valdivia se realizó un estudio de biopsias efectuadas en pacientes con sospecha clínica de linfoma cutáneo. De un total de 11 biopsias con diagnóstico histopatológico de LCCT, diez casos resultaron ser MF (90,9 por ciento), y uno SS (9,1 por ciento). Los casos se analizaron según distribución por sexo, edad, procedencia, fecha de aparición de las lesiones, fecha de la primera consulta, antecedentes mórbidos y neoplásicos, características clínicas y distribución anatómica de las lesiones, diagnósticos diferenciales postulados, número de biopsias efectuadas y su informe histopatológico, estadio al momento del diagnóstico y tratamiento efectuado. Se revisó la literatura, con especial énfasis en la etiología, histopatología, clínica y métodos diagnóticos actuales de LCCT

Determinação da viabilidade do Paracoccidioides loboi em biopsias de pacientes portadores de doença de Jorge Lobo / Determination of the viability of the Paracoccidioides loboi in biopsias of carrying patients of illness of Jorge Wolf

Fundamentos- Na doença de Jorge Lobo, devido ao não cultivo do P.loboi, utiliza-se material obtido de biopsias de pacientes, sendo a determinação da viabiliadade imprescindivel para os estudo experimentais da doença. Objetivos- Determinar a viabilidade do P. loboi, por meio de diferentes metodos de coloração, e investigar a influencia da temperatura e de diferentes soluções na viabilidade do fungo. Casuistica e Metodos- Biopsias de 15 pacientes foram processadas em solução salina 0,85%(SS), solução salina de Hank´s(SSH) e solução salina glicerinada a 30% com vitamina B(SSG). A viabilidade foi determinada no momento da coleta da biopsia (tempo 0), empregando-se os corantes diacetato de fluoresceina-brometo de etideo (DF-BE), lactofenol azul-algodão, azul-tripan e verde-janus. As suspensões foram mantidas a 4°C, 23°C e 37°C, e a viabilidade foi determinada a cada tres dias, utilizando-se a coloração DF-BE. Resultados - A viabilidade do P. loboi no tempo 0 variou de 21 a 46%, utilizando-se a coloração de DF-BE. A viabilidade com os demais corantes não foi satisfatoria, devido a dificuldades em separar os fungos viaveis dos não viaveis. A temperatura de 4°C foi mais eficaz em manter a viabilidade do fungo (ate 15 dias). Por outro lado, nenhuma das soluções empregadas conseguiu manter a viabilidade do fungo ou favorecer sua multiplicação. Conclusões - A determinação da viabilidade do P. loboi com DFG-BE podera auxiliar nas tentativas de cultura do fungo, nas inoculações em animais e no controle terapeutico da doença. Alem disso, a temperatura parece ser fator importante no crescimento do P. loboi.

Aspectos clínicos-epidemiológicos de la paracoccidioidomicosis en Venezuela: revision de 268 casos / Aspect clinical-epidemical of the paracoccidioidomicosis in Venezuela: review of 268 cases

Analizamos algunas variables clínico-epidemiológicas de paracoccidioidomicosis en Venezuela como: sexo, edad, procedencia por estado, ocupación, hábitos psicobiológicos, clínica, diagnóstico y evolución de la enfermedad. Se realizó un estudio retrospectivo y multicéntrico revisando las historias clínicas de 311 casos con diagnóstico de paracoccioidomicosis, archivadas en los hospitales de las zonas endémicas del país, durante el decenio 1979-1989. Del total de historias evaluadas, 43 fueron descartadas debido a que no hubo un diagnóstico de certeza de la enfermedad. Se encontró una relación hombre/mujer de 11:1. El pico de mayor prevalencia fue entre el quinto y sexto decenio de la vida. La forma mixta fue la más frecuente en 147 casos. El largo período de incubación de la enfermedad explica el pico de prevalencia por edad. Se han implicado a los estrógenos como factor de resistencia en las mujeres en desarrollar la enfermedad junto con su menor exposición al hongo. Queda la interrogante de si la enfermedad se adquiere en el campo ya que más de la mitad de los casos tenía ocupaciones citadinas. Es frecuente en adultos y rara en los niños, sindo crónica (pulmonar y/o muco-cutánea u otras localizaciones) y aguda/diseminada (fiebre, hepato-esplenomegalia, síndrome adenomegálico), respectivamente

Zigomicose granulomatosa visceral / Zigomycosis visceral granulomatous

Neste trabalho såo revistos os principais aspectos clínicos e patológicos da Zigomicose. Uma ampla revisåo foi feita da sua forma granulomatosa, que redundou na discussåo da nomenclatura adotada para as formas clínicas da doença, e que deu como resultante uma nova classificaçåo clínica, onde ficou estabelecida a forma visceral exclusiva. Comentários sobre a epidemiologia revelaram a prevalência da forma visceral em indivíduos jovens do sexo masculino, predominantemente da zona rural, da regiåo intertropical. O diagnóstico da micose se realiza pelo histopatológico, por patologista experimentado. O cultivo do agente pode nåo ser de sucesso, mas o encontro de Conidiobolus ou Basidiobolus sela o diagnóstico, pois estes fungos nåo såo contaminantes. A açåo patogênica dos fungos tem mecanismo ainda nåo muito claro, mas os aspectos histopatológicos clássicos com a presença de fenômeno de Splendore-Hoeppli associado a eosinofilia tissular e ausência de invasåo vascular, sugerem um mecanismo imunológico com hipersensibilildade. Quanto à terapêutica, ficou estabelecido que apesar da sensibilidade do agente a mais de um quimioterápico, na forma granulomatosa visceral é muito importante o diagnóstico precoce e a terapêutica imediata, condiçoes capazes de impedir a disseminaçåo por contiguidade da doença. A Zigomicose granulomatosa, conhecida há vários anos, só recentemente foi vista no Brasil (1967), e sua forma granulomatosa visceral exclusiva, apenas em 1979. A conceituaçåo da forma visceral exclusiva se encontra em andamento, até agora na literatura mundial existe 7 casos, aos quais acrescentamos 5 casos, sendo que um deles no final da doença ocorreu comprometimento da pele.

Paracoccidioidomicose: inquérito com paracoccidiodina em zonas rurais do estado da Bahia / Paracoccidioidomycosis: surveys with paraccidioidin in rural zone of state of Bahia

Foram realizados inquéritos com a intra-dermo-reaçäo à paracoccidiooidina-polissacáride em 5 pequenas comunidades rurais do interior da Bahia, com o objetivo de determinar a prevalência da paracoccidioidomicose -infecçäo na área e a associaçäo entre sensibilidade à paracoccidioidina e ocupaçäo de lavrador. A positividade na populaçäo geral foi de 21,8 por cento, sendo a mais alta (31,5 por cento) e a mais baixa (9,5 por cento). A positividade à paracoccidioidina aumentou significativamente com a idade, mas, ao contrário, o sexo, o local de nascimento, a condiçäo de migrante e a procedência dos migrantes näo influenciaram na positividade a este antígeno. Nem a ocupaçäo de lavrador nem o tempo de exercício nesta atividade alteraram a positividade. Observou-se que o intervalo de 24 horas foi o mais indicado para a leitura, após a aplicaçäo do antígeno. A intradermoreaçäo á histoplasmina introduzida com a finalidade de revelar possíveis reaçöes inespecíficas (cruzadas) à paracoccidioidina, e realizada em todos os indivíduos, revelou taxa de infecçäo geral de 31,9 por cento a mais alta, com 49,1 por cento e a mais baixa 15,0 por cento

Incidence of Kaposi's sarcoma and mycosis fungoides in the United States including Puerto Rico, 1973-81.

The incidence of Kaposi's sarcoma (KS) was examined with the use of data from the Surveillance, Epidemiology, and End Results (SEER) Program of the National Cancer Institute. KS is a manifestation of the recent epidemic of acquired immunodeficiency syndrome (AIDS) that has occurred particularly among homosexual men. The incidence of KS in 1973-79 was found to be higher (0.29 male and 0.07 female cases/100,000/yr) than is usually cited for the pre-AIDS KS incidence rates. Collectively, the 9 SEER registries in the United States showed only a slight increase in the incidence of KS between 1973-79 and 1980-81. However, the SEER registry covering San Francisco, which is a high-risk area for AIDS, showed a marked excess of KS in 1981. The KS case rate among never-married men younger than 50 years old, a surrogate index of homosexuality, was found to be markedly elevated in the post-AIDS period, compared with the case rate of a reference disease, mycosis fungoides. Never-married men younger than 50 years old, therefore, constitute a SEER-identifiable population who can be monitored for risk of KS and other neoplasms that might be related to AIDS. In addition, the incidence rate of KS in the SEER registry of Puerto Rico was generally higher than that in the U.S. SEER registries, despite data that suggested that KS may be underreported. The demographic characteristics of patients diagnosed as having KS in Puerto Rico suggested the classical rather than the AIDS-related form of KS.