Pulmonary histiocytosis

Acute exposure of rats to high concentrations (up to 40,000 ppm) has resulted in convulsions, pulmonary edema, respiratory arrest, and death. In rats repeatedly exposed at 600 ppm, death was attributed to renal papillary necrosis renal toxicity was not present in rabbits similarly exposed. Exposure of rabbits to 300 or 600 ppm resulted in convulsions and hyperactivity, moderate inflammation of nasal tissues, and some inflammation of the trachea or bronchi. Subchronic studies found that rats exposed at 3 00 ppm had mottled incisor teeth, minimal renal effects, pulmonary histiocytosis, inflammation of nasal tissues, and cerebral vacuolation. 

Finally, WHO group V includes diagnoses not fitting into any of the above categories, such as sarcoidosis, pulmonary histiocytosis, extrinsic compression of pulmonary artery, etc. Sarcoidosis may involve chronic lung disease, an active vasculitis, extrinsic compression, or cardiac disease. 

Hammar SP. Pulmonary histiocytosis-X (Langerhans cell granulomatosis). In Dail DH, Hammar SP, eds. Pulmonary Pathology. 2nd ed New York Springer-Verlag 1994 567-596. 

Epithelioid hemangioendothelioma -Inflammatory pseudotumor (pulmonary inflammatory myoflbroblastic tumor) -Pulmonary histiocytosis X ... 

Moore AD, Godwin ID, Muller NL, et al. Pulmonary histiocytosis X comparison of radiographic and CT findings. Radiology 1989 172(l) 249-254. 

Schonfeld N, Frank W, Wenig S, et al. Chnical and radiologic features, lung function and therapeutic results in pulmonary histiocytosis X. Respiration 1993 60 38. ... 

Soler P, Chollet S, Jacque C, et al. Immunocytochemical characterization of pulmonary histiocytosis X cells in lung biopsies. Am J Pathol 1985 118 439-451. 

Tazi A, Bonay M, Grandsaigne M, et al. Surface phenotype of Langerhans cells and lymphocytes in granulomatous lesions from patients with pulmonary histiocytosis X. Am Rev Respir Dis 1993 147(6 pt 1) 1531-1536. 

Crausman RS, Jennings CA, Tuder RM, et al. Pulmonary histiocytosis X pulmonary function and exercise pathophysiology. Am J Respir Crit Care Med 1996 153(1) 426 35. 

Chollet S, Soler P, Dournovo P, et al. Diagnosis of pulmonary histiocytosis X by immunodetection of Langerhans cells in bronchoalveolar lavage fluid. Am J Pathol 1984 115(2) 225-232. 

Auerswald U, Barth J, Magnussen H. Value of CD-I-positive cells in bronchoalveolar lavage fluid for the diagnosis of pulmonary histiocytosis X. Lung 1991 169(6) 305-309. 

Friedman PJ,LiebowAA,Sokoloff J (1981) Eosinophilic granuloma of lung clinical aspects of primary pulmonary histiocytosis in the adult. Medicine 60 385-396 Fromm GB, Dunn LJ, Harris JO (1980) Desquamative interstitial pneumonitis characterization of free intraalveolar cells. Chest 77 552-554... 

Lacronique J, Roth C, Battesti J-P, Basset F, Chretien J (1982) Chest radiological features of pulmonary histiocytosis X a report based on 50 adult cases. Thorax 37 104-109 Lang MR, Fiaux GW, Gillooly M, Stewart JA, Hulmes DJS, Lamb D (1994) Collagen content of alveolar wall tissue in emphysematous and non-emphysematous lungs. Thorax 49 319-326... 

Brauner MW, Grenier P, Mouelhi MM, Monpoint D, Lenoir S (1989) Pulmonary Histiocytosis X evaluation with HR-CT. Radiology 172 255-258... 

Systemic disorders sarcoidosis, pulmonary Langerhans cell histiocytosis lymphangioleiomyomatosis, neurofibromatosis, vasculitis... 

Respiratory Bronchiolitis-Associated Interstitial Lung Disease 341 Desquamative Interstitial Pneumonia 341 Lymphoid Interstitial Pneumonia 342 Acute Interstitial Pneumonia 342 Sarcoidosis 343 Miscellaneous Rare Forms of Interstitial Lung Disease of Unknown Etiology 345 Pulmonary Langerhans Cell Histiocytosis 345 Lymphangioleiomyomatosis 345 Eosinophilic Pneumonia 346 Pulmonary Alveolar Proteinosis 348 Pulmonary Microlithiasis 348... 

Fig. 26.16a,b. Pulmonary Langerhans cell histiocytosis in a 26-year-old man. a Axial CT image demonstrates bilateral, thin-walled cysts of variable size and multiple, ill-defined nod-... 

The most important differential diagnoses for LAM are Langerhans cell histiocytosis, idiopathic pulmonary fibrosis, and panlobular emphysema. In contrast to LAM, in Langerhans cell histiocytosis, the costophrenic sulci are usually spared, the cysts can be thick-waUed and irregularly outlined, and nodules are predominant in the early stage of disease. Idiopathic pulmonary fibrosis shows a volume loss in contrast to LAM, and the honeycomb cysts are predominantly located in the lower lobes and subpleural (Bonelli et al. 1998). Panlobular emphysema is associated with alpha-1-antiprotease deficiency. The most distinct feature of emphysema is the absence of defined walls in the areas of low attenuation, whereas cysts in LAM almost invariably present with walls (Johnson 1999). 

Abbott GF, Rosado-de-Christenson ML et al. (2004) From the archives of the AFIP pulmonary Langerhans cell histiocytosis. Radiographics 24 821-841... 

Eosinophilic pneumonia Sarcoid, M. Wegener Histiocytosis X Systemic Lupus eythematodes Rheumatoide arthritis Bronchiolo-alveolar carcinoma Lymphangiosis carcinomatosa Pulmonary congestion... 

Pulmonary Langerhans cell histiocytosis (PLCH) is a rare disease of smokers often presenting as pneumothoraces or dyspnea (128-130). CT scans in PLCH shows numerous thin-walled cystic lesions, typically <10 mm in size however, cysts may coalesce, sometimes exceeding 5 cm and assume bizarre shapes... 

Figure 15 HRCT demonstrating numerous thin-walled cysts in the middle and upper lung zones in a patient with PLCH. Abbreviations HRCT, high-resolution computed tomography PLCH, pulmonary Langerhans cell histiocytosis.

Caminati A, Harari S. Smoking-related interstitial pneumonias and pulmonary Langerhans cell histiocytosis. Proc Am Thorac Soc 2006 3(4) 299-306. 

Tazi A, Soler P, Hance AJ. Adult pulmonary Langerhans cell histiocytosis. Thorax 2000 55(5) 405 16. 

Brauner MW, Grenier P, Tijani K, et al. Pulmonary Langerhans cell histiocytosis evolution of lesions on CT scans. Radiology 1997 204(2) 497-502. 

Vassallo R, Jensen EA, Colby TV, et al. The overlap between respiratory bronchiolitis and desquamative interstitial pneumonia in pulmonary Langerhans cell histiocytosis high-resolution CT, histologic, and functional correlations. Chest 2003 124(4) 1199-1205. 

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