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Histiocitose de células de langerhans: um estudo de 20 casos
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Abstract(s)
Objectivo: Descrever os aspectos clínico-radiológicos da Histiocitose de Células de Langerhans (HCL) numa série de doentes. Material e Métodos: Reviram-se 20 casos de HCL, nove com diagnóstico segundo os actuais critérios anátomo-patológicos
(positividade para CD1a e/ou presença de grânulos de Birbeck em microscopia electrónica), outros nove apenas com critérios morfológicos, e dois casos com diagnóstico patológico estabelecido fora da instituição, não susceptível de aferição anátomo- patológica.
Resultados: A razão sexo masculino:feminino foi 4:1 e a média de idades de apresentação da doença 8 anos (variação: 2 meses -
44 anos). Os quatro órgãos mais frequentemente envolvidos foram, por ordem decrescente: osso, gânglios linfáticos, pele e hipófise. Dois doentes tinham doença unifocal óssea, oito apresentavam doença multifocal-unissistémica, em sede óssea ou ganglionar, e dez doença multissistémica. As lesões líticas foram o achado radiológico mais frequente, distribuindo-se predominantemente na calote craniana, ossos ilíacos e fémures. As alterações pulmonares constituiram achado minoritário. Conclusão: A série em estudo incluiu uma proporção grande de casos com gravidade clínica (10 doentes com HCL multissistémica),
tendo-se observado em número relativamente restrito a forma menos grave da doença, correspondente ao clássico granuloma
eosinófilo. A aparência das alterações radiológicas foi contudo similar à descrita na literatura.
Objective: To describe the clinical and radiological features of Langerhans Cell Histiocytosis (LCH) in a series of patients. Material And Methods: We reviewed 20 cases of LCH, nine with diagnosis by the current pathological criteria (positivity for CD1a and/or presence of Birbeck granules identified at ultrastructural examination), and nine only with morphological criteria. In the other two cases, the pathological diagnosis was made outside our institution and could not be reviewed. Results: Male:female ratio was 4:1 and the average age of presentation of the disease was 8 years (range: 2 months - 44 years). The four most affected organs were, in decreasing order: bone, lymph nodes, skin and hypophysis. Two patients had unifocal bone disease, eight multifocal unisystem involvement in the bone or lymph nodes, and ten presented multisystem LCH. Lytic lesions were the most common radiological finding, affecting mainly the skull, iliac bones and femurs. Pulmonary lesions were rather infrequent. Conclusion: This series included a large proportion of severe cases (10 patients with multisystem LCH), while the mildest form of the disease, classically designated as eosinophilic granuloma, was observed only in a few patients. The appearance of the radiological findings was however similar to the described in the literature.
Objective: To describe the clinical and radiological features of Langerhans Cell Histiocytosis (LCH) in a series of patients. Material And Methods: We reviewed 20 cases of LCH, nine with diagnosis by the current pathological criteria (positivity for CD1a and/or presence of Birbeck granules identified at ultrastructural examination), and nine only with morphological criteria. In the other two cases, the pathological diagnosis was made outside our institution and could not be reviewed. Results: Male:female ratio was 4:1 and the average age of presentation of the disease was 8 years (range: 2 months - 44 years). The four most affected organs were, in decreasing order: bone, lymph nodes, skin and hypophysis. Two patients had unifocal bone disease, eight multifocal unisystem involvement in the bone or lymph nodes, and ten presented multisystem LCH. Lytic lesions were the most common radiological finding, affecting mainly the skull, iliac bones and femurs. Pulmonary lesions were rather infrequent. Conclusion: This series included a large proportion of severe cases (10 patients with multisystem LCH), while the mildest form of the disease, classically designated as eosinophilic granuloma, was observed only in a few patients. The appearance of the radiological findings was however similar to the described in the literature.
Description
Keywords
Histiocitoses Histiocitose de células de langerhans Granuloma eosinófilo Doença de hand-schuller-christian Doença de letterer-siwe Histiocytosis Langerhans cell histiocytosis Eosinophilic granuloma Hand-schuller-christian disease Letterer-siwe disease
Citation
Sofia Vinhais; Pedro Farinha; Teresa Margarida Cunha. Langerhans Cell Histiocitosis: A Study of 20 Cases, Acta Radiológica Portuguesa, 16, 64, 27-32, 2004.