Pulmonary Langerhans Cell Granulomatosis (Histiocytosis X) A Clinicopathologic Study of 48 Cases

Travis, William D.; Borok, Zea; Roum, James H.; Zhang, Jun; Feuerstein, Irwin M.; Ferrans, Víctor J.; Crystal, Ronald G.

doi:10.1097/00000478-199310000-00002

Cited by 271 publications

(217 citation statements)

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“…Nine of 18 previously reported ECD patients with lung involvement have died of disease at 3 weeks to 8 years after diagnosis (14). In contrast, pulmonary LCH, a smoking-related disorder, generally has an indolent course and may regress either spontaneously or after steroid therapy and/or cessation of smoking (23).…”

Section: Discussionmentioning

confidence: 99%

Pulmonary Pathology of Erdheim-Chester Disease

et al. 2000

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Erdheim-Chester disease (ECD) is a rare non-Langerhans' cell histiocytosis that may present with pulmonary symptoms. The condition seems to be nonfamilial and typically affects middle-aged adults. Radiographic and pathologic changes in the long bones are diagnostic, but patients often present with extraskeletal manifestations. Advanced pulmonary lesions are associated with extensive fibrosis that may lead to cardiorespiratory failure. The clinical, radiologic, and pathologic features of six patients with ECD with lung involvement are presented. The patients were three men and three women (mean age, 57). Five presented with progressive dyspnea, and one presented with diabetes insipidus. Open-lung biopsies showed histiocytic infiltrates in a lymphangitic pattern with associated fibrosis and lymphoplasmacytic inflammatory infiltrates. The histiocytes did not stain with periodic acid-Schiff. Immunoperoxidase studies performed on specimens from five of six patients showed that the histiocytes were positive for CD68 and Factor XIIIa and negative for CD1a. Specimens from two patients exhibited immunoreactivity for S-100 protein. Electron microscopy studies performed on specimens from two patients showed phagocytic lysosomes but no Birbeck granules. Clinical follow-up of up to 16 years was available. At the end of that time, five patients were dead of complications related to their disease; one patient remains alive 4 years after diagnosis but with severe respiratory compromise. ECD is a rare nonLangerhans' cell histiocytosis that may present as interstitial lung disease and resemble other pulmonary conditions, particularly usual interstitial pneumonitis and pulmonary Langerhans' cell histiocytosis. Recognition of this entity will allow better assessment of its true incidence, therapeutic options, and prognosis.

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Section: Discussionmentioning

confidence: 99%

Pulmonary Pathology of Erdheim-Chester Disease

et al. 2000

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“…There are convincing data supporting a causal relationship between cigarette smoke and PLCH in adults [5,10]. The overwhelming majority (> 90%) of adult patients who develop PLCH smoke cigarettes or were exposed to substantial second-hand smoke exposure [5,10,12].…”

Section: Cigarette Smoking and Genetic Factorsmentioning

confidence: 99%

“…The overwhelming majority (> 90%) of adult patients who develop PLCH smoke cigarettes or were exposed to substantial second-hand smoke exposure [5,10,12]. In children with PLCH, the association with cigarette smoking is less clear [17], although it has been reported that the commencement of smoking in teenage years can precipitate PLCH in young adults with a history of non-pulmonary childhood LCH [30].…”

Section: Cigarette Smoking and Genetic Factorsmentioning

confidence: 99%

“…PLCH predominantly affects young adults between the ages of 20 to 40 years [5]. There does not appear to be a gender predilection [5,[10][11][12]. Although most published series in the English literature are composed of Caucasian subjects [5,10,11], its occurrence in Asian populations is increasingly recognized [13].…”

mentioning

confidence: 99%

“…There does not appear to be a gender predilection [5,[10][11][12]. Although most published series in the English literature are composed of Caucasian subjects [5,10,11], its occurrence in Asian populations is increasingly recognized [13]. Few familial cases have been reported and isolated PLCH is almost always a sporadic illness [14,15].…”

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confidence: 99%

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Pulmonary Langerhans'-Cell Histiocytosis

et al. 2000

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Pulmonary Langerhans Cell Histiocytosis (PLCH) is a relatively uncommon lung disease that generally, but not invariably, occurs in cigarette smokers. The pathologic hallmark of PLCH is the accumulation of Langerhans and other inflammatory cells in small airways, resulting in the formation of nodular inflammatory lesions. While the overwhelming majority of patients are smokers, mechanisms by which smoking induces this disease are not known, but likely involve a combination of events resulting in enhanced recruitment and activation of Langerhans cells in small airways. Bronchiolar inflammation may be accompanied by variable lung interstitial and vascular involvement. While cellular inflammation is prominent in early disease, more advanced stages are characterized by cystic lung destruction, cicatricial scarring of airways, and pulmonary vascular remodeling. Pulmonary function is frequently abnormal at presentation. Imaging of the chest with high resolution chest CT scanning may show characteristic nodular and cystic abnormalities. Lung biopsy is necessary for a definitive diagnosis, although may not be required in instances were imaging findings are highly characteristic. There is no general consensus regarding the role of immunosuppressive therapy in smokers with PLCH. All smokers must be counseled on the importance of smoking cessation, which may result in regression of disease and obviate the need for systemic immunosuppressive therapy. The prognosis for most patients is relatively good, particularly if longitudinal lung function testing shows stability. Complications like pneumothoraces and secondary pulmonary hypertension may shorten life expectancy. Patients with progressive disease may require lung transplantation.

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Successful Treatment of Adult Multisystemic Langerhans Cell Histiocytosis With Psoralen–UV-A, Prednisolone, Mercaptopurine, and Vinblastine

Stebut

Schadmand-Fischer²,

Bräuninger³

et al. 2008

Arch Dermatol

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Background: Langerhans cell histiocytosis (LCH) is a rare disease with a peak incidence in childhood. There is limited experience with treatment options for adult patients having multisystemic LCH involvement. We report successful treatment of a 70-year-old woman with adult onset of LCH and multisystem disease (diabetes insipidus centralis, bone marrow infiltration, and lung and skin involvement). Observations: A 70-year-old woman with erythematous plaques and papules of the submammary and inguinal skin attended our outpatient clinic and was diagnosed as having LCH. Organ involvement was found in the infundibulum of the pituitary gland, associated with diabetes insipidus centralis, bone marrow infiltration, and several micronodules of the thoracic and lumbar spine and lungs. Based on the Histiocyte Society's LCH-A1 study in adults, the patient was treated for 12 months with a combination of corticosteroids, vinblastine, and mercaptopurine. No major adverse effects were observed. The skin was also treated with a combination of psoralen-UV-A and local corticosteroids. Restaging revealed regression of all clinical symptoms (skin involvement and diabetes insipidus centralis) and regression of organ infiltration (pituitary gland, bone marrow, and lungs). Conclusion: Effective treatment of adult multisystemic LCH disease was achieved using prednisolone, vinblastine, and mercaptopurine, which was well tolerated.

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Pulmonary Langerhans Cell Granulomatosis (Histiocytosis X) A Clinicopathologic Study of 48 Cases

Cited by 271 publications

References 0 publications

Pulmonary Pathology of Erdheim-Chester Disease

Pulmonary Pathology of Erdheim-Chester Disease

Pulmonary Langerhans'-Cell Histiocytosis

Successful Treatment of Adult Multisystemic Langerhans Cell Histiocytosis With Psoralen–UV-A, Prednisolone, Mercaptopurine, and Vinblastine

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