Carta al Director. Histiocitosis de células de Langerhans en rango de edad poco frecuente. Langerhans’ cell histiocytosis in a rare age range. Cristina San Juan. Translation for ‘histiocitosis de células de Langerhans (HCL)’ in the free Spanish- English dictionary and many other English translations. La histiocitosis de células de Langerhans (HCL), anteriormente conocida como histiocitosis X, es una enfermedad poco frecuente caracterizada por la.
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Thirty-seven percent of adults with LCH have skin histiocitosis de celulas de langerhans, usually as part of multisystem disease. Involvement of specific organs such as the liver, spleen, and hematopoietic system separates multisystem LCH into a high-risk group and lanberhans low-risk group, where risk indicates the risk of death from histiocitosis de celulas de langerhans.
Treatment options for patients with high-risk multisystem disease spleen, liver, and bone marrow involving one or more sites include hostiocitosis following:. Full text is only aviable in PDF.
Tumor tissue from progressive or recurrent disease must be available for molecular characterization.
Histiocitosis de células de Langerhans en niños. Descripción de 10 casos
Board members review recently published articles each month to determine whether an article should:. American journal of clinical pathology.
In accordance with these findings, the pathologic histiocyte or LCH cell has a gene expression profile closely resembling that of a myeloid dendritic cell. LCH may involve a histiocitosis de celulas de langerhans organ single-system LCHwhich may be a single site unifocal or involve multiple sites multifocal ; or LCH may involve multiple organs multisystem LCHwhich may involve a limited number of organs or be disseminated.
Cl 10 A- Reactivation of Langerhans cell histiocytosis LCH after complete response is common.
Classically, the disease is symmetrical and predominates in the upper and ce,ulas lung fields, sparing the costophrenic angle and giving a very characteristic picture on high-resolution CT scan. Over many years, national and international study groups have histiocitosis de celulas de langerhans risk groups for allocation of Langerhans cell histiocytosis LCH patients to risk-based therapy on the basis of mortality risk and risk of late effects of the disease. The frequency and natural history of diabetes insipidus in chindren with Langerhans-cell histiocytosis.
Langerhans cell histiocytosis
Circulating cells with the VE mutation could be detected in all high-risk patients and in a subset of low-risk multisystem patients. Views Read Edit View history.
A biopsy of the pituitary gland may be indicated when the pituitary gland is the only site of disease and the stalk is greater than 6. The complete evaluation of histiocitosis de celulas de langerhans patient, presenting with either single-system or multisystem disease, should include the following:. Arch Dis Child, 75pp. Sixty-four percent of adults with diabetes insipidus monitored for an average of 6 years developed other endocrine problems.
If this is your first time in the Web: Treatment options for patients with recurrent, refractory, or progressive high-risk multisystem disease include the following:. Contemporany classification histiocitosis de celulas de langerhans histiocytic disorders. Because of the increased risk of diabetes insipidus, systemic treatment is recommended for these patients.
This is a privacy protected site that provides up-to-date information for individuals interested in the latest scientific news, trials, and treatments related to rare lung diseases. Int J Clin Exp Pathol. British Journal of Dermatology. Ortiz Romero aF. Lesions of the facial bones or anterior or middle cranial fossae e. Robbins and Cotran pathologic basis of disease. Excessive neurologic toxicity from vinblastine, for example, prompted closure of the LCH-A1 trial.
Five patients had neurologic dysfunction.
Seventy-five percent of children with sclerosing langefhans will not respond to chemotherapy because the LCH is no longer active, but the fibrosis and sclerosis remain. Refer to the Multiple bone lesions in combination with skin, histiocitosis de celulas de langerhans node, or diabetes histiocitosis de celulas de langerhans [low-risk multisystem LCH] section of this summary for information about additional agents used to treat multifocal bone LCH.
However, some patients may have LCH involving only the skin, mouth, pituitary gland, or other sites not studied in these international trials. Patients with craniofacial lesions had lower performance and verbal intelligence quotient scores than did those with other LCH lesions. Histiocytosis Monocyte- and macrophage-related cutaneous conditions Rare diseases.
Gastroenterología y Hepatología
These patients also had a higher incidence of diabetes insipidus, correlating with the higher frequency of lesions. Extensive surgery is also not indicated. Presence of Birbeck granules on electron microscopy and immuno-cytochemical features e.
CT or MRI scans are histiocitosis de celulas de langerhans in assessing response of a soft tissue mass associated with a bone lesion, but are not particularly helpful in assessing the response of lytic bone lesions. Recurrent cytogenetic or genomic abnormalities would also be required to demonstrate convincingly that LCH is a malignancy.
CT and ultrasound imaging abnormalities are often found.