histiocytosis…

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eosinophilic granuloma, x-ray, the skull, respiratory system, eosinophilic granuloma, pulmonary histiocytosis x, nonlipid reticuloendotheliosis, pulmonary langerhans cell granulomatosis, hand-schuller-christian disease, letterer-siwe disease, langerhans cell histiocytosis, histiocytosis, generic name, group of syndromes characterized, abnormal increase, in the number of certain immune cells, histiocyte cells, monocytes, macrophages, dendritic cells, 3 major classes of histiocytoses, article focuses on langerhans cell histiocytosis, histiocytosis x, 2 classes, non-langerhans cell histiocytosis, hemophagocytic syndrome, malignant histiocytosis syndrome, t-cell lymphoma, most cases of histiocytosis x affect children between ages 1, 15 years old, incidence peaks amongst children between 5, histiocytosis x is thought to affect roughly 1 in 200, 000 people each year, extra immune cells, form tumors, affect various parts, in children, histiocytosis x, involves, bones, 80%.consist of single, multiple sites, skull is frequently affected, tumors produce a punched-out appearance on bone x-ray, tumors in weight-bearing bones, legs, spine, fracture spontaneously, systemic, whole body, involvement, causing rashes, lung problems, gum infiltration, lymph gland swelling, hormonal problems, enlargement, the spleen, liver, anemia, not surprisingly, a disease, many systems, organs, histiocytosis deadly, children over 5 years old often have only bone involvement, unfortunately, surviving, long periods often continue to have problems related, young children, infants, more likely to have systemic involvement, a fatal outcome, histiocytosis x has typically been thought of, a cancer-like condition, researchers have begun to suspect that it is actually an autoimmune phenomenon, rogue immune cells attack the body, rather than fighting infections, forms, genetic, another type, pulmonary histiocytosis x, characterized by inflammation, the small airways, bronchioles, small blood vessels, in the lungs, most common in adults, inflammation leads to stiffening, fibrosis, destruction, the walls, the alveoli, parts, the lung, people 30, 40 years old, affected most often, cigarette smokers make up 90% of patients, spontaneous pneumothorax occurs frequently in this condition, symptoms that affect children, adults, listed below, some overlap, adult, cough, shortness of breath, chest pain, fever, weight loss, general discomfort, uneasiness, ill feeling, malaise, increased amount, urine output, thirst, increased drinking of fluids, bone pain, rash, children, failure to thrive, weight loss, irritability, fever, seborrheic dermatitis, the scalp, abdominal pain, jaundice, vomiting, limping, thirst, frequent urination, short stature, delayed puberty, mental deterioration, headache, dizziness, seizures, increased eyeball protrusion, swollen lymph glands, generalized rash, petechiae, purpura, chronically draining ears, bone pain, adult, chest x-ray, bronchoscopy, biopsy showing typical x bodies in histiocytes, pulmonary function tests, children, bone x-ray showing punched-out appearance of bone lesions, skeletal survey x-ray, x-rays, the entire skeleton, the degree of bone involvement, biopsy of skin to check, langerhans cells, bone marrow biopsy to check, langerhans cells, complete blood count, cbc, tests, clinical symptoms direct, disorder is treated, corticosteroids, suppress immune function, the dangerous cells, smoking, worsen the response to treatment, children given other medications depending on estimated prognosis, methotrexate, vinblastine, cyclophosphamide, etoposide, radiation therapy, limited surgery, treat bone lesions, patients, supportive care to reduce symptoms, treat infections, the immune system is compromised, antibiotics, breathing assistance, a respirator, physical therapy, selenium-based shampoo, scalp problems, hormone replacement to deal, hormonal dysfunction, patients, wish to contact the histiocytosis association of america at 800-548-2758, in pulmonary histiocytosis, disease stabilizes, improves in, one-half, the affected people, half progress to a permanent loss of lung function, pediatric versions, depends on the actual disease category, severity, expected to have a normal life span, minimal disease involvement, expect a poor outcome, spontaneous pneumothorax, diffuse interstitial pulmonary fibrosis, children, pituitary dysfunction, growth failure, diabetes insipidus, bone marrow invasion, subsequent anemia, lung problems, eventual pulmonary failure, depending on the extent, severity of symptoms, shortness of breath, chest pain, symptoms of this disorder develop, avoid smoking, quitting smoking, improve the outcome in people whose lungs, effected, no prevention is known, childhood forms.