Langerhans Cell Histiocytosis

The Langerhans cell histiocytosis (LCH) is characterized by a proliferation of dendritic cells with mononuclear or localized infiltration of organs. Most cases occur in childhood. Clinically, the disease can manifest by pulmonary infiltrates, bone lesions, rashes and hepatic, hematopoietic and endocrine dysfunction. The diagnosis is made by biopsy. Unfavorable prognosis factors: age at diagnosis <2 years and a disseminated stage, especially with the participation of hematopoietic system, liver, lung, or a combination of these factors. Depending on the spread of the disease, the treatment includes both supportive care measures and the use of cytotoxic and / or the local treatment by surgical intervention or radiotherapy.

The Langerhans cell histiocytosis (LCH) is characterized by a proliferation of dendritic cells with mononuclear or localized infiltration of organs. Most cases occur in childhood. Clinically, the disease can manifest by pulmonary infiltrates, bone lesions, rashes and hepatic, hematopoietic and endocrine dysfunction. The diagnosis is made by biopsy. Unfavorable prognosis factors: age at diagnosis <2 years and a disseminated stage, especially with the participation of hematopoietic system, liver, lung, or a combination of these factors. Depending on the spread of the disease, the treatment includes both supportive care measures and the use of cytotoxic and / or the local treatment by surgical intervention or radiotherapy.

(Pulmonary Langerhans cell histiocytosis.) The Langerhans cell histiocytosis (LCH) is characterized by a proliferation of dendritic cells with mononuclear or localized infiltration of organs. Most cases occur in childhood. Clinically, the disease can manifest by pulmonary infiltrates, bone lesions, rashes and hepatic, hematopoietic and endocrine dysfunction. The diagnosis is made by biopsy. Unfavorable prognosis factors: age at diagnosis <2 years and a disseminated stage, especially with the participation of hematopoietic system, liver, lung, or a combination of these factors. Depending on the spread of the disease, the treatment includes both supportive care measures and the use of cytotoxic and / or the local treatment by surgical intervention or radiotherapy. Langerhans cell histiocytosis is a disease of the dendritic cells. You can show different clinical pictures that were formerly called eosinophilic granuloma, disease Hand-Schuller-Christian disease and Abt-Letterer-Siwe. Today these names usually have only historical significance (except for the eosinophilic granuloma), since these are different clinical manifestations of the same underlying disease and show up in most patients with Langerhans cell histiocytosis manifestations of more than one syndrome. Estimates of the frequency of Langerhans cell histiocytosis vary much (for example, from about 1:. 50000-1: 200,000). In the LZH abnormally proliferating dendritic cells infiltrate one or more organs. Bone, skin, teeth, gingiva, ears, endocrine organs, lung, liver, spleen, lymph nodes or bone marrow may be affected, either by direct infiltration and resultant dysfunction or by compression due to the increase of adjacent structures. multiple organs are affected in about half of patients. Symptoms and signs The symptoms and clinical signs are very variable and dependent on the affected organs. The historic designations are indeed still used to describe the disease, but only in a few patients manifested the classic categories according to, and with the exception of eosinophilic granuloma these terms are no longer used. Eosinophilic granuloma Solitary or multifocal eosinophilic granuloma (60-80% of Langerhans cell histiocytosis cases) occurs mainly in older children and young adults, usually up to the age of 30. The incidence peaks between 5 and 10 years of age. Most painful bone lesions occur, often with heavy load restriction and overlying pressure sensitive, occasionally overheated Schwellungen.Morbus Hand-Schuller-Christian This syndrome accounts for about 15-40% of Langerhans cell histiocytosis cases and occurs in children between the ages of 2 and 5 years, less common in older children and adults. It is a systemic disease that typically affects the flat bones of the skull, ribs, the pelvis and / or the scapula. Bones and lumbosacral vertebrae are less frequently affected. Very rarely there is an infection of the wrists, hands, knees, feet or cervical vertebrae. In classic cases, exophthalmos occurs caused by an orbital tumor. However, a loss of visual acuity or strabismus is rarely involvement of the optic nerve or the eye muscles. In the elderly, often a loss of teeth by apical or gingival infiltration occurs. Chronic otitis media or otitis externa is temporal because of the involvement of the mastoid or petrous frequently. Here, there is a partial obstruction of the ear canal. The classic triad of the disease includes insipidus participation of flat bones, proptosis and diabetes. Approximately 5-50% of patients suffering from diabetes insipidus, the proportion of children of the orbit and the skull is with systemic disease and involvement even higher. Up to 40% of children with systemic disease are dwarfed. Hyperprolactinemia and hypogonadism can This syndrome accounts for about 10% of Langerhans cell histiocytosis cases by a hypothalamic infiltration due sein.Morbus Abt-Letterer-Siwe. In this systemic disease is the most severe form of the LZH. Typically it occurs in children <2 years and presents itself as scaly forming, seborrheic, eczema-like and occasionally purple rash of the scalp, the ears, the abdomen and the intertriginous areas of the neck and face. The exposed skin is a portal of entry for microorganisms that can cause sepsis. Often show a discharge from the ear, lymphadenopathy and hepatosplenomegaly, and in severe cases, it leads to liver dysfunction with hypoproteinemia and reduced synthesis of coagulation factors. Furthermore, anorexia, irritability, developmental disorders and pulmonary manifestations (eg. As cough, tachypnea, pneumothorax) may occur. The presence of thrombocytopenia is considered very unfavorable prognostic sign. In addition, clinically significant anemia and neutropenia occasionally be observed. The parents of the affected children often report early tooth growth; However, this is actually based on the receding gums through which the immature teeth are exposed. Frequently asked due to the appearance of these patients misdiagnoses include abuse and neglect. Diagnostic biopsy Suspicion of a Langerhans cell histiocytosis occurs in (especially young) patients with unexplained pulmonary infiltrates, bone lesions, abnormalities of the eye or facial bones and in children <2 years with typical rashes or severe unexplained multi-organ diseases. Eosinophilic granuloma of the skull with permission of the publisher. For example Swearingen, Schaefer P., J. Primavera, Klibanski A. Atlas of Clinical Endocrinology: Neuroendocrinology and Pituitary Disease. Edited by S. Korenman (Series Editor) and M. E. Molitch. Philadelphia, Current Medicine, 2000. var model = {thumbnailUrl: '/-/media/manual/professional/images/eosinophilic_granuloma_high_de.jpg?la=de&thn=0&mw=350' imageUrl: '/ - / media / manual / professional / images / eosinophilic_granuloma_high_de.jpg lang = en & thn = 0 ', title:'? Eosinophilic granuloma of the skull ', description:' u003Ca id = "v38395997 " class = ""anchor "" u003e u003c / a u003e u003cdiv class = ""para "" u003e u003cp u003eBei a patient with Langerhans cell histiocytosis is shown a typical monostotisches eosinophilic granuloma of the skull in the radiological image. The lesion is sharply defined

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