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Pulmonary eosinophilic granuloma

MedGen UID:
264101
•Concept ID:
C1455705
•
Disease or Syndrome
Synonyms: Histiocytosis X, Pulmonary; Langerhans Cell Granulomatosis, Pulmonary; Pulmonary Histiocytosis X; Pulmonary Langerhans Cell Granulomatosis
SNOMED CT: Langerhans cell histiocytosis, unifocal, pulmonary (28122003); Langerhans cell histiocytosis of lung (417152008); Eosinophilic granuloma of lung (28122003); Isolated histiocytosis X of lung (28122003); Pulmonary Langerhans cell histiocytosis (417152008); Pulmonary eosinophilic granuloma (28122003)

Definition

Langerhans cell histiocytosis affecting the lungs. It is characterized by an interstitial proliferation of Langerhans cells in the lung parenchyma. Most patients are current or former smokers. Steroids are the mainstay therapy for this condition. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPulmonary eosinophilic granuloma

Professional guidelines

PubMed

MECHANISMS IN ENDOCRINOLOGY: Hypophysitis: diagnosis and treatment.
Joshi MN, Whitelaw BC, Carroll PV
Eur J Endocrinol 2018 Sep;179(3):R151-R163. Epub 2018 Jun 7 doi: 10.1530/EJE-17-0009. PMID: 29880706
Consensus recommendations for the diagnosis and clinical management of Rosai-Dorfman-Destombes disease.
Abla O, Jacobsen E, Picarsic J, Krenova Z, Jaffe R, Emile JF, Durham BH, Braier J, Charlotte F, Donadieu J, Cohen-Aubart F, Rodriguez-Galindo C, Allen C, Whitlock JA, Weitzman S, McClain KL, Haroche J, Diamond EL
Blood 2018 Jun 28;131(26):2877-2890. Epub 2018 May 2 doi: 10.1182/blood-2018-03-839753. PMID: 29720485Free PMC Article
Diabetes insipidus--diagnosis and management.
Di Iorgi N, Napoli F, Allegri AE, Olivieri I, Bertelli E, Gallizia A, Rossi A, Maghnie M
Horm Res Paediatr 2012;77(2):69-84. Epub 2012 Mar 16 doi: 10.1159/000336333. PMID: 22433947

Recent clinical studies

Etiology

Accuracy of high-resolution CT in diagnosing lung diseases.
Bonelli FS, Hartman TE, Swensen SJ, Sherrick A
AJR Am J Roentgenol 1998 Jun;170(6):1507-12. doi: 10.2214/ajr.170.6.9609163. PMID: 9609163
Reactive eosinophilic pulmonary vascular infiltration in patients with spontaneous pneumothorax.
Luna E, Tomashefski JF Jr, Brown D, Clarke RE, Kleinerman J
Am J Surg Pathol 1994 Feb;18(2):195-9. doi: 10.1097/00000478-199402000-00009. PMID: 8291658
Transbronchial biopsy in patients with pulmonary eosinophilic granuloma. Comparison with findings on open lung biopsy.
Housini I, Tomashefski JF Jr, Cohen A, Crass J, Kleinerman J
Arch Pathol Lab Med 1994 May;118(5):523-30. PMID: 7514863
Neoplasms associated with pulmonary eosinophilic granuloma.
Tomashefski JF, Khiyami A, Kleinerman J
Arch Pathol Lab Med 1991 May;115(5):499-506. PMID: 2021319
Reactive eosinophilic pleuritis: a lesion to be distinguished from pulmonary eosinophilic granuloma.
Askin FB, McCann BG, Kuhn C
Arch Pathol Lab Med 1977 Apr;101(4):187-91. PMID: 576785

Diagnosis

Transbronchial biopsy in patients with pulmonary eosinophilic granuloma. Comparison with findings on open lung biopsy.
Housini I, Tomashefski JF Jr, Cohen A, Crass J, Kleinerman J
Arch Pathol Lab Med 1994 May;118(5):523-30. PMID: 7514863
Immunohistochemical diagnosis of pulmonary eosinophilic granuloma on lung biopsy.
Cagle PT, Mattioli CA, Truong LD, Greenberg SD
Chest 1988 Dec;94(6):1133-7. doi: 10.1378/chest.94.6.1133. PMID: 2461275
S-100 staining in the diagnosis of eosinophilic granuloma of lung.
Webber D, Tron V, Askin F, Churg A
Am J Clin Pathol 1985 Oct;84(4):447-53. doi: 10.1093/ajcp/84.4.447. PMID: 2412435
Pulmonary eosinophilic granuloma complicating pregnancy.
Lavin JP Jr, Miodovnik M
Obstet Gynecol 1981 Oct;58(4):516-9. PMID: 7279346
Reactive eosinophilic pleuritis: a lesion to be distinguished from pulmonary eosinophilic granuloma.
Askin FB, McCann BG, Kuhn C
Arch Pathol Lab Med 1977 Apr;101(4):187-91. PMID: 576785

Therapy

Transbronchial biopsy in patients with pulmonary eosinophilic granuloma. Comparison with findings on open lung biopsy.
Housini I, Tomashefski JF Jr, Cohen A, Crass J, Kleinerman J
Arch Pathol Lab Med 1994 May;118(5):523-30. PMID: 7514863
Pulmonary eosinophilic granuloma.
Gervais DA, Whitman GJ, Chew FS
AJR Am J Roentgenol 1993 Dec;161(6):1158. doi: 10.2214/ajr.161.6.8249718. PMID: 8249718
Neoplasms associated with pulmonary eosinophilic granuloma.
Tomashefski JF, Khiyami A, Kleinerman J
Arch Pathol Lab Med 1991 May;115(5):499-506. PMID: 2021319

Prognosis

Diagnosis of pulmonary lymphangioleiomyomatosis by HMB45 in surgically treated spontaneous pneumothorax.
Tanaka H, Imada A, Morikawa T, Shibusa T, Satoh M, Sekine K, Abe S
Eur Respir J 1995 Nov;8(11):1879-82. doi: 10.1183/09031936.95.08111879. PMID: 8620956
Neoplasms associated with pulmonary eosinophilic granuloma.
Tomashefski JF, Khiyami A, Kleinerman J
Arch Pathol Lab Med 1991 May;115(5):499-506. PMID: 2021319
Eosinophilic granuloma of the skeleton with involvement of the lung: a report of three cases.
Meier B, Rhyner K, Medici TC, Kistler G
Eur J Respir Dis 1983 Oct;64(7):551-6. PMID: 6628588
Pulmonary eosinophilic granuloma complicating pregnancy.
Lavin JP Jr, Miodovnik M
Obstet Gynecol 1981 Oct;58(4):516-9. PMID: 7279346
Pulmonary eosinophilic granuloma. Its natural history and prognosis.
BICKERS JN, BUECHNER HA, EKMAN PJ
Am Rev Respir Dis 1962 Feb;85:211-9. doi: 10.1164/arrd.1962.85.2.211. PMID: 13868973

Clinical prediction guides

Accuracy of high-resolution CT in diagnosing lung diseases.
Bonelli FS, Hartman TE, Swensen SJ, Sherrick A
AJR Am J Roentgenol 1998 Jun;170(6):1507-12. doi: 10.2214/ajr.170.6.9609163. PMID: 9609163
Immunohistochemical and ultrastructural examination of histiocytosis X cells in pulmonary eosinophilic granuloma.
Sakuma N, Kamei T, Ohta M, Oda T, Hongo H, Okamura H, Ishihara T
Acta Pathol Jpn 1992 Oct;42(10):719-26. doi: 10.1111/j.1440-1827.1992.tb03221.x. PMID: 1466244
Reactive eosinophilic pleuritis. A sequela of pneumothorax in pulmonary eosinophilic granuloma.
McDonnell TJ, Crouch EC, Gonzalez JG
Am J Clin Pathol 1989 Jan;91(1):107-11. doi: 10.1093/ajcp/91.1.107. PMID: 2642637
Eosinophilic granuloma of the skeleton with involvement of the lung: a report of three cases.
Meier B, Rhyner K, Medici TC, Kistler G
Eur J Respir Dis 1983 Oct;64(7):551-6. PMID: 6628588
Reactive eosinophilic pleuritis: a lesion to be distinguished from pulmonary eosinophilic granuloma.
Askin FB, McCann BG, Kuhn C
Arch Pathol Lab Med 1977 Apr;101(4):187-91. PMID: 576785

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