ABSTRACT
Objective To study the ultrastructural features of Langerhans cell(LC),especially Birbeck granules in Langerhans cell histiocytosis(LCH) and provide some ultrastructural evidence to clarify the origin,function and stereoscopic structure of Birbeck granules.Methods Pathologic tissues of 8 LCH patients were treated with routine transmission electron microscope (TEM) procedures,fixture,dehydration,permeation,embedding,polymerization,ultramicrocut,staining and examined by TEM.Results Under the TEM,8 cases of LCH revealed typical LC cell,and LC cell in 6 cases revealed typical rod,tennnis-racquet like Birbeck granules.Some double membrane structures revealed irregular shape,sometimes accompanied with vacuole-like structure.Most of Birbeck granules locate inside the cytoplasmic membrane,in continuity with the cell membrane.There was Birbeck granule-like structure outside the cytoplasmic membrane in one case of multisystem LCH.Conclusions The ultrastructure of Birbeck granules could be various and irregular.The irregular Birbeck granule-like structure can also be useful for the diagnosis of LCH.Birbeck granules may arise from the cell membrane.The stereoscopic structure of Birbeck granules could be irregular.
ABSTRACT
La histiocitosis de células de Langerhans se describió desde el siglo XIX. Su origen y patogénesis se basan actualmente en tres eventos: clonalidad comprobada, un proceso reactivo a una infección viral, una proliferación y daño mediados por citocinas. El diagnóstico es histopatológico con la demostración de gránulos de Birbeck o la positividad para CD1a. Afecta a pacientes de cualquier edad y se manifiesta como una lesión osteolítica única o hasta como una enfermedad sistémica. El tratamiento es la resección quirúrgica, la radioterapia o la quimioterapia. El pronóstico varía de acuerdo a la presentación clínica.
Langerhanss cell histiocytosis was first described in the XIX century. Its origin and pathogenesis are based mainly on 3 events: clonal proliferation, a cytokines mediated reactive process secondary to a viral infection. Diagnosis is based on histopathologic studies by the presence of Birbeck granules or CD1a positivity. Langerhans cell histiocytosis affects patients of any age; it may present as a single osteolitic lesion or as a systemic disease. Treatment is based on surgical resection, radiotherapy or chemotherapy. Outcome varies on the clinical presentation of the disease.
Subject(s)
Male , Infant , Histiocytosis , Histiocytosis, Langerhans-Cell , Antigens, CD1 , Case Management , Hepatomegaly , Langerhans Cells , Osteolysis , SplenomegalyABSTRACT
Pulmonary histiocytosis X is a granulomatous disorder of the lung of unknown cause. Patients with this disease often complain of cough, dyspnea on exertion and , occaionally, chest pain from pneumothorax or bone involvement. However, DI is uncommon in these patients. We report a case of primary pulmonary histiocytosis X with central diabetes insipidus. A 23-year-old man presented with dyspnea suffered from dry cough, exertional dyspnea, polydipsia and polyuria for 4 months. He was a heavy smoker. He was found to have reticulonodular interstitial opacities on chest X-ray film. High-resolution computed tomography revealed thin-walled cysts of various sizes in both lungs. Open lung biopsy was done. On light microscopic examination revealed proliferation and infiltration of Langerhans cells. Immunohistochemically, Langerhans cells showed strong cytoplasmic staining with S-100 protein and electronmicroscopic examination showed Birbeck granules in Langerhans cells. Water deprivation test showed central-type diabetes insipidus and brain MRI showed no abnormal lesion on suprasellar region. Smoking cessation was recommended. He was treated with oral desmopressin.