Evaluación imaginológica de un caso de histiocitosis durante tres años / Imaging evaluation of a case of histiocytosis for three years

La histiocitosis de células de Langerhans es un trastorno celular dendrítico, resultado de la proliferación clonal de este tipo de células, morfológicamente redondeadas e inmaduras inmunofenotípica y funcionalmente; asociadas con eosinófilos, macrófagos, linfocitos y células multinucleadas gigantes, en ocasiones. Puede presentarse de forma monosistémica, cuando afecta a un solo sitio u órgan; o multisistémica cuando se presenta en múltiples órganos o sistemas corporales. La literatura médica y las publicaciones en las que se asocia la imaginología al estudio y caracterización de las enfermedades hematológicas son escasas, aun en la era tecnológica. En este trabajo se presenta un caso de histiocitosis de células de Langerhans que fue seguido imaginológicamente durante 3 años con el consiguiente apoyo al diagnostico, tratamiento, seguimiento y valoración; lo que demuestra la utilidad de la Imaginología como herramienta para lograr un mejor manejo del paciente y como principio de la atención medica multidisciplinaria(AU)

Langerhans cells histiocytosis is a dendritic cell disorder, result of clonal proliferation of this type of cells, morphologically rounded and immature immunophenotypically and functionally; associated with eosinophils, macrophages, lymphocytes and giant multinucleated cells at times. It can present monosystemic, affecting a single site or organ; or multisystemic disease when present in multiple organs or body systems. Medical literature and publications in which imaging is associated with the study and characterization of hematological diseases are scarce, even in the technological era. This paper presents a case of Langerhans cells histiocytosis that was followed imaging during 3 years with the subsequent support to diagnosis, treatment, monitoring and evaluation; demonstrating the usefulness of imaging as tool to achieve better patient management and as a principle of multidisciplinary medical care(AU)

Langerhans' cell histiocytosis: experience from a single center.

Clinical profile, disease-distribution and outcome of Langerhans cell histiocytosis (LCH) is presented in this retrospective analysis. There were 69 children with LCH from January 1986 to December 2004. Diagnosis was presumptive in the majority. The age ranged from 2 months to 12 years. Multisystem disease was documented in 48 (69.6%) children. Evidence of hepatic dysfunction was detected in 25 (36.2%). An elevated serum alkaline phosphatase was a prominent observation in patients with hepatic involvement. Children with localized disease received oral steroids, while cases with disseminated/multi-system disease were treated with prednisolone and, vinblastine or etoposide. 20 (54%) children with disseminated disease and organ dysfunction died. A favorable outcome was documented in all but one case with localized disease. Portal hypertension developed in 3 cases, all of whom had a fatal outcome. Twelve (17.4%) patients had diabetes insipidus. Disseminated disease with organ dysfunction was observed to be a predictor of fatal outcome.

Pulmonary langerhans cell histiocytosis presenting as simultaneous bilateral spontaneouse pneumothorax in a non-smoker patient

Pulmonary Langerhans Cell Histiocytosis [PLCH] is a rare idiopathic disorder that primarily affects young adult cigarette smokers. Affected patients often present with cough and dyspnea and about 20% of patients present with or later develop pneumothorax. It is striking that more than 90% of patients are smokers. We report a very unusual case of PLCH in a 20-year- old male patient with no smoking history in whom a life- threatening complication such as simultaneous bilateral pneumothorax was the presenting feature. The final diagnosis was made by open surgical biopsy and recurrent pneumothoraces necessitated surgical management with pleurodesis. We emphasize the early use of pleurodesis in managing patients with PLCH and spontaneous pneumothorax

Diagnósticos diferenciales de la histiocitosis a células de Langerhans / The differential diagnostics of Langerhans cell histiocytosis

La histiocitosis a células de Langerhans (HCL) debe diferenciarse de las siguientes entidades: eritema tóxico neonatorum (ETN), dermatitis seborreica (DS), foliculitis pustulosa eosinofílica (FPE), incontinencia pigmenti (IP), mastocitosis/urticaria pigmentosa (M/UP), acrodermatitis enteropática (ADE), síndrome de Wiskott-Aldrich (WAS), acropustulosis infantil (API). Además se deben considerar la enfermedad de Rosai- Dorfman (ERD), xantomas diseminados, melanosis pustulosa neonatal (MPN), candidiasis congénita, listeriosis neonatal, herpes simple perinatal y la varicela neonatal. Debido a que los métodos auxiliares de laboratorio no siempre están disponibles o los resultados laboratoriales algunas veces son extemporáneos, y puesto que el médico práctico a menudo necesita tomar decisiones precozmente, es que la epidemiología resulta útil, pues brinda el marco adecuado para ordenar y jerarquizar las sospechas diagnósticas frente a un caso concreto, con un paciente determinado, en un momento específico.

The differential diagnostics of Langerhans cell histiocytosis should include the following disorders: erythema toxicum neonatorum, seborrheic dermatitis, eosinophilic pustular folliculitis, incontinentia pigmenti, mastocytosis / urticaria pigmentosa, acrodermatitis enteropathica, Wiskott-Aldrich syndrome, infantile acropustulosis, Rosai- Dorfman disease, xanthoma disseminatum, neonatal pustular melanosis, congenital candidiasis, perinatal listeriosis, perinatal herpes simplex, neonatal varicella. Since the auxiliary methods of lab are not always available, or lab results are sometimes extemporaneous, the physicians often needs to make quick decisions. The epidemiology is useful because it offers the appropriate mark to prioritize the diagnostic in specific cases.

histiocytose a cellules de Langerhans pulmonaire de l' adulte

Pulmonary Langerhans cell histiocytosis belongs to the spectrum of the Langerhans' cell histiocytosis. The common point is tissue infiltration by Langerhans' cell granuloma. But the pulmonary type occurs predominantly in young adults and is associated with tobacco. The most frequently encountered symptoms are cough and dyspnea. Twenty-five to 35% of the cases are incidental findings. 20% of cases present with extra-thoracic lesions including skin, bone and hypothalamo-pituitary axis. On scanning images the lung parenchyma is infiltrated at the beginning by stellar nodules of less than 10 mm, which evolve either gradually to form cysts or towards remission, spontaneous or induced by the treatment. With pulmonary function testing, the two types of anomalies [obstruction and restriction] are observed. The reduction in diffusing capacity observed in 60 to 90% of the cases reflects the pulmonary vascular involvement. Detec-tion of more than 5% of CD-la cells in bronchiolo-alveolar lavage fluid and transbronchial biopsies are not sensitive. Treatment is controversial and includes primarily smoking cessation and corticosteroid therapy at the early nodular stage

Estudo epidemiológico da doença da célula de langerhans: análise de 33 casos em Natal/RN / Epidemiologic estudy of the langerhans cell disease: analysis of 33 cases in Natal/RN

A doença da célula da Langerhans é uma desordem de etiologia incerta, caracterizada por um amplo aspecto clínico e comportamento bastante variado. Neste estudo foram analisados 33 casos de pacientes com diagnóstico de Doenca de célula de Langerhans no Hospital Infantil Varela Santiago em Natal, RN-Brasil, entre 1997 e 1999. Deste total, 42,4 por cento dos pacientes manifestaram o Granuloma Eosinofílico Solitário, 33 por cento dos pacientes tinham a Doença de Hand-Schüller- Christian e 24,3 por cento destes pertenciam a forma clínica da Doença de Letterer-Siwe. Do total, 54,5 por cento dos pacientes eram do sexo masculino e 45,5 por cento do feminino, com uma média de idade de 3 anos e 7 meses (variando de 06 meses a 15 anos). Setenta por cento dos pacientes apresentavam envolvimento extra-oral, sendo os ossos do crânio e extremidades inferiores, os sítios mais comuns da doença. O tratamento consistiu primariamente da excisão cirúrgica para pacientes com lesões localizadas e terapia com drogas para aquelas com a Doença de Letterer-Siwe. Nosso estudo demonstrou que a forma clínica mais freqüente foi o Granuloma Eosinofílico Solitário e que pacientes mais jovens com disfunção orgânica têm prognóstico pouco favóravel

Lesöes pseudotumorais de maior prevalência na infância / Pseudotumoral lesions of more prevalence at childhood

O autor apresenta uma revisäo bibliográfica referente aos principais aspectos no estudo das lesöes pseudotumorais que acometem a populaçäo infantil com maior freqüência. Säo enfocados aspectos clínicos, radiográficos e histopatológicos, bem como fatores relacionados à etilogia, prevalência e conduta terapêutica frente a tais lesöes

Enfermedad de células de Langerhans crónica localizada: reporte de caso con lesiones periodontales. Revisión de la literatura / Chronic localized Langerhans cells disease. Report of a case with periodontal lesions. Review of the literature

La enfermedad de células de Langerhans es una enfermedad de etiología desconocida, la cual sucede con mayor frecuencia en la edad infantil. El presente es un reporte de un caso de enfermedad de células de Langerhans en un adulto. El paciente presentó lesiones destructivas a nivel periodontal como única manifestaciónb de la enfermedad. Los pacientes con enfermedades periodontales producto de la enfermedad de células de Langerhans son frecuentemente diagnosticados como otras patologías. Este caso constituye una muestra del diagnóstico precoz de esta enfermedad por un abordaje multidisciplinario. Las manifestaciones bucales de esta enfermedad, sus métodos de diagnóstico y tratamiento son discutidos

Pulmonary dirofilariasis in a patient with multisystem Langerhans cell histiocytosis--the first reported case in Thailand.

Despite a high prevalence of canine dirofilariasis, there is no case of pulmonary dirofilariasis reported from Thailand. We herein report a case of multisystem Langerhans cell histiocytosis who had an incidental pulmonary dirofilariasis found at the time of autopsy as a solitary nodule at the periphery of the right lower lobe. This is the first reported case in Thailand. Association between pulmonary dirofilariasis and Langerhans cell histiocytosis has not been described before in the literature.

Histiocitosis X pulmonar primaria (granuloma eosinofílico pulmonar)

La histiocitosis X pulmonar primaria es una patología infrecuente que afecta predominantemente a personas entre los 20 y los 40 años, usualmente con historia de tabaquismo. Presentamos un grupo de tres pacientes con esta entidad que se estudiaron en el Hospital Santa Clara en los últimos 20 años. Los síntomas más frecuentes fueron tos con expectoración mucosa y disnea. En todos se documentó historia de tabaquismo. La radiografía de tórax mostró infiltrado intersticial reticulonodular difuso, con volumen pulmonar normal y signos de hipertensión pulmonar precapilar. Todos se presentaron con hipoxemia severa y cor pulmonale. La histopatología confirmó el diagnóstico de histiocitosis X pulmonar primaria.

Histiocytosis-X in Saudi children.

A retrospective analysis of 21 Saudi children with Histiocytosis-X were reviewed. 13 were males, 8 were females, with a ratio of 1.62:1. Five had unifocal disease while 16 were with multifocal disease. Bone involvement was seen in 19 patients (90.4%), and three patients had organ dysfunction. One patient had family history of Histiocytosis-X. Follow-up was from 1-10 years (Median 3 years). Three patients died, three had recurrences and eight patients had various disabilities (38%). Overall disease free survival was 84.2 percent.