Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Histiocytosis X developed in five patients (one woman and four men) when aged between 15 and 44 years. The initial sign in four of them was eosinophilic granuloma of the bone, in one it was pulmonary involvement. In three patients the disease remained confined to bone, while in two it involved the lungs and central nervous system, respectively. Osteolysis regressed spontaneously in one of the men, while in the woman there has been no recurrence 8 years after resection of the focus. In another man an osteolytic focus in a rib was noted after a 9-year recurrence-free interval. The man with pulmonary and bone involvement received chemotherapy with vinblastine and prednisone: dyspnoea and cough disappeared, vital capacity improved and the interstitial lung changes regressed. The osteolytic foci were repeatedly irradiated in the man with bone and CNS involvement. This brought about considerable reduction in pain but no significant radiological changes of the foci. Two courses of chemotherapy were given over 12 years, once with vincristine and prednisone, afterwards with cyclophosphamide. This arrested the progression of the osteolytic foci, but each time they recurred when the drugs were stopped.
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PMID:[Histiocytosis X. Clinical aspects and course in 5 patients]. 193 52

The pulmonary primary localization of the histiocytosis X is rather rare. We describe here a case of histiocytosis X with a localization exclusively at pulmonary level in a 38 years old young man who is a heavy smoker. A dry cough, asthenia and an increasing dyspnoea were the first symptoms of the disease. The radiological picture showed a widespread pulmonary intersticepathy. The diagnosis has been obtained testing some fragments of the pulmonary parenchyma taken through a diagnostic thoracotomy with an electron microscope.
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PMID:[A case of primary pulmonary histiocytosis X]. 215 51

A 22-year-old black male presented with progressive dyspnea, a nonproductive cough, and new skin lesions. He was severely hypoxic, and had a severe restrictive defect on pulmonary function testing. A 2-cm lytic defect was noted on skull radiographs. A lung biopsy demonstrated pulmonary fibrosis. A biopsy of a skin lesion was consistent with a diagnosis of multifocal eosinophilic granuloma, or disseminated histiocytosis X. The case presents several unusual features of this uncommon disorder.
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PMID:Multifocal eosinophilic granuloma. 326 19

A 71-year-old white man developed an increasing number of 1-to-10 mm, erythematous nodules, many with central ulceration, most prominent on the head and trunk. Biopsy of a nodule showed infiltration of the dermis and epidermis by large cells with multilobulated nuclei and numerous mitoses. Electron microscopy showed that most tumor cells contained Langerhans' cell granules. Immunohistochemical studies demonstrated a pattern of antigen expression similar to that of Langerhans' cells including Ia and Leu-6 (T6) antigens. Chest x-ray showed diffuse pulmonary infiltration and similar tumor cells were present in the sputum and urine. He developed increasing dyspnea and jaundice despite chemotherapy, and died 6 months after the onset of the disease. Autopsy showed massive tumor infiltration of the lungs, liver, spleen, and lymph nodes, and focal involvement of the myocardium, skin and bladder. Clinical and cytologic features indicated this case to be a rare example of highly malignant histiocytosis X in an elderly man.
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PMID:Malignant histiocytosis X. Report of a rapidly fatal case in an elderly man. 620 93

A 28-month-old boy had firm, red nodules for 4 months that were mostly localized to the face. The eruption was preceded by conjunctivitis, eyelid edema, and swelling of the root of the nose. He also had dyspnea, anisocoria with areflexia, swelling of the parotid glands, and hepatosplenomegaly. A bone marrow aspirate showed 25% eosinophils. A skin biopsy specimen revealed a lymphohistiocytic infiltrate that involved the entire dermis. Most of the cells expressed antimacrophage markers; S-100 was negative. Electron microscopy showed poorly differentiated histiocytes without any specific marker. Steroid therapy induced complete clearing. Two years later, after several cutaneous recurrences, the patient is free of disease. We believe that this case represents a nodular, benign non-Langerhans cell histiocytosis with cutaneous lesions that differ from previously described histiocytopathies.
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PMID:An unusual case of non-Langerhans cell histiocytosis. 829

The anatomical lesions of three subjects suffering from pulmonary histiocytosis X and treated with lung transplantation were studied in order to detail the changes occurring in late stages of the disease; the early stages are known on account of lung biopsies done with a diagnostic aim. All the subjects were, at the time of transplantation, in a precarious clinical state and were practically bed-bound as a result of their dyspnoea with a history of histiocytosis X which had been known for 9, 7 and 3 years respectively; one patient had a cardiopulmonary bypass and two unilateral lung transplant. The results of the histological study produced data which was slightly discordant from the communly held ideas; the de Langerhans cells persist in large numbers and after many years of the evolution of the disease, even though other elements of the granuloma have practically disappeared. Fibrosis is absent playing no role in the genesis of the respiratory failure. The lesions are destructive, leading to a disappearance of the alveolar capillary bed which is the source of the dyspnea. The honeycomb cavities to the destroyed lung are varied in nature: most correspond to the disappearance of the dividing walls of the alveoli or the pulmonary acinus, some others related to dilated terminal bronchioles and finally others to hollowed out granulomas which have become cavities. The peculiar characteristic of the lung allows for destructive cavities to be produced by the airflow, submitted to a process of auto-aggravation by the elastic forces and the tension of the pulmonary parenchyma.
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PMID:[Severe late stage lesions of pulmonary histiocytosis X. Report of 3 transplantations]. 834 66

Juvenile Xanthogranuloma. Report of a case with hepatic involvement. The Authors present a case of Juvenile Xanthogranuloma (JX) in a 3 months female child with cutaneous and hepatic nodules associated to dyspnea attributable to obstructive bronchopneumopathy. Histologically the lesions are xanthomatous with proliferation of fat-laden histiocytes. The hepatic involvement is characterized by hepatomegaly and yellow nodules on liver surface as seen at laparoscopy. On liver biopsy there is remarkable expansion of portal triad caused by aggregates of large foamy mono-polynuclear histiocytes with Touton giant cells. The cutaneous nodule biopsy shows histiocytic infiltrate in inter-adnexal dermal space with many giant cells holding great lipidic vacuoles. The patient's follow-up is characterized by slow and progressive clinical improvement with resolution of cutaneous, hepatic and pulmonary pathology. The Authors emphasize the differential diagnosis between this systemic form of JX and Langerhans cell Histiocytosis (Histiocytosis X) with multiorgan involvement. This diagnosis is necessary in order to establish therapy and prognosis.
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PMID:[Juvenile xanthogranuloma. Description of a case with liver involvement]. 851 25

We report the case of a man with biopsy-proved pulmonary Langerhans' cell granulomatosis (histiocytosis X) and pulmonary precapillary hypertension. Pulmonary vascular resistances and mean pulmonary artery pressure decreased with corticosteroid therapy and dyspnea improved.
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PMID:Steroid-responsive pulmonary hypertension in a patient with Langerhans' cell granulomatosis (histiocytosis X). 868 45

A 48-year-old female with a history of accentuated dyspnea, pleural thickening in anteromedial portion with left patchy parenchymal shadowing invading adjoining parasternal structure of the rib cage, presented 13 months later marked superclavicular, anterior mediastinic and parahilar left lymphadenopathy. Open surgical biopsies on the pleural lesion invading the hypodermic tissues of parasternal region showed morphological and immunocytochemical patterns of Langerhans' cell histiocytosis (LCH). 13 months later the superclavicular lymph node biopsy diagnosed Hodgkin's lymphoma (HD), mixed cellularity type II, stage AE. On a total of 29 cases with association of LCH and HD, the described case in the second case that shows morphologically demonstrated LCh with subsequent development of HD. It is postulated that the development of HD in a patient with LCH, might represent malignant evolution of this hyperplastic process.
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PMID:Langerhans' cell granulomatosis and Hodgkin's lymphoma. Report of a case. 870 Mar 50

A 23-year-old woman was supposed to undergo thoracoscopic surgery for the 10th pneumothorax that accompanied histiocytosis X. The past history included Lylle's disease, asthma, myocarditis, drug-induced leucocytopenia and bronchitis obliterans. The preoperative arterial blood gas analysis under receiving O2 at rate of 2 l.min-1 via a nasal cannula revealed normal values. General anesthesia and intubation with a double-lumen endotracheal tube would have been preferable, but regional anesthesia was chosen because of her medical history and positive results of the skin tests for vecuronium, pancronium, diazepam and midazolam. During the first 10 min of thoracoscopic procedure, her respiration became rapid and shallow and she was restless and comatose. The operation was cancelled. Arterial blood gas analysis under receiving O2 at rate of 4 l.min-1 via a face mask revealed: pH 7.025, PaO2 113.8 mmHg, PaO2 244.8 mmHg, HCO3- 29.7 mEq.l-1, BE-5.6, and O2 saturation 99.1%. Manual artificial ventilation with a mask and bag was initiated. Her spontaneous respiration and consciousness recovered in next 30 min. The postoperative course was uneventful. Tachypnea, caused from anxiety, dyspnea and stimulation of irritant receptors in the airway, were considered to be responsible for the event. The duration of inspiration became shorter as tachypnea developed, that made the tidal volume to decrease and hypercapnea ensued.
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PMID:[Hypercapnea during thoracoscopic surgery under regional anesthesia]. 925 15


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