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Pulmonary Langerhans cell histiocytosis

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)
 
HPO: HP:0034769

Definition

Accumulation of monoclonal CD1a-positive Langerhans cells in the bronchioles and alveolar interstitium that can cause damage to the lungs. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPulmonary Langerhans cell histiocytosis

Professional guidelines

PubMed

Pulmonary Langerhans cell histiocytosis - an update on pathogenesis and treatment.
Jouenne F, Benattia A, Tazi A
Curr Opin Pulm Med 2023 Sep 1;29(5):451-458. Epub 2023 Jul 6 doi: 10.1097/MCP.0000000000000988. PMID: 37410483
Current understanding and management of pulmonary Langerhans cell histiocytosis.
Vassallo R, Harari S, Tazi A
Thorax 2017 Oct;72(10):937-945. Epub 2017 Jul 8 doi: 10.1136/thoraxjnl-2017-210125. PMID: 28689173
Diagnosis and treatment of cystic lung disease.
Park S, Lee EJ
Korean J Intern Med 2017 Mar;32(2):229-238. Epub 2017 Feb 28 doi: 10.3904/kjim.2016.242. PMID: 28264540Free PMC Article

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) Histiocytic Neoplasms, 2024

Recent clinical studies

Etiology

Smoking-Related Interstitial Lung Diseases.
Alarcon-Calderon A, Vassallo R, Yi ES, Ryu JH
Immunol Allergy Clin North Am 2023 May;43(2):273-287. Epub 2023 Mar 1 doi: 10.1016/j.iac.2023.01.007. PMID: 37055089
Smoking-associated interstitial lung disease: update and review.
Dawod YT, Cook NE, Graham WB, Madhani-Lovely F, Thao C
Expert Rev Respir Med 2020 Aug;14(8):825-834. Epub 2020 May 22 doi: 10.1080/17476348.2020.1766971. PMID: 32379511
Diffuse Cystic Lung Diseases.
Obaidat B, Yazdani D, Wikenheiser-Brokamp KA, Gupta N
Respir Care 2020 Jan;65(1):111-126. Epub 2019 Oct 15 doi: 10.4187/respcare.07117. PMID: 31615921
Pulmonary Langerhans' cell histiocytosis in adults.
Radzikowska E
Adv Respir Med 2017;85(5):277-289. Epub 2017 Oct 30 doi: 10.5603/ARM.a2017.0046. PMID: 29083024
Rare pulmonary diseases: a common fight.
Harari S, Humbert M
Eur Respir Rev 2017 Sep 30;26(145) Epub 2017 Sep 6 doi: 10.1183/16000617.0059-2017. PMID: 28877977Free PMC Article

Diagnosis

Smoking-Related Interstitial Lung Diseases.
Alarcon-Calderon A, Vassallo R, Yi ES, Ryu JH
Immunol Allergy Clin North Am 2023 May;43(2):273-287. Epub 2023 Mar 1 doi: 10.1016/j.iac.2023.01.007. PMID: 37055089
Birt-Hogg-Dubé syndrome.
Daccord C, Good JM, Morren MA, Bonny O, Hohl D, Lazor R
Eur Respir Rev 2020 Sep 30;29(157) Epub 2020 Sep 17 doi: 10.1183/16000617.0042-2020. PMID: 32943413Free PMC Article
Smoking-associated interstitial lung disease: update and review.
Dawod YT, Cook NE, Graham WB, Madhani-Lovely F, Thao C
Expert Rev Respir Med 2020 Aug;14(8):825-834. Epub 2020 May 22 doi: 10.1080/17476348.2020.1766971. PMID: 32379511
Pulmonary Langerhans Cell Histiocytosis.
Shaw B, Borchers M, Zander D, Gupta N
Semin Respir Crit Care Med 2020 Apr;41(2):269-279. Epub 2020 Apr 12 doi: 10.1055/s-0039-1700996. PMID: 32279297
Pulmonary Langerhans' cell histiocytosis in adults.
Radzikowska E
Adv Respir Med 2017;85(5):277-289. Epub 2017 Oct 30 doi: 10.5603/ARM.a2017.0046. PMID: 29083024

Therapy

Smoking-Related Interstitial Lung Diseases.
Alarcon-Calderon A, Vassallo R, Yi ES, Ryu JH
Immunol Allergy Clin North Am 2023 May;43(2):273-287. Epub 2023 Mar 1 doi: 10.1016/j.iac.2023.01.007. PMID: 37055089
Smoking-associated interstitial lung disease: update and review.
Dawod YT, Cook NE, Graham WB, Madhani-Lovely F, Thao C
Expert Rev Respir Med 2020 Aug;14(8):825-834. Epub 2020 May 22 doi: 10.1080/17476348.2020.1766971. PMID: 32379511
Pulmonary Langerhans Cell Histiocytosis.
Shaw B, Borchers M, Zander D, Gupta N
Semin Respir Crit Care Med 2020 Apr;41(2):269-279. Epub 2020 Apr 12 doi: 10.1055/s-0039-1700996. PMID: 32279297
Diffuse Cystic Lung Diseases.
Obaidat B, Yazdani D, Wikenheiser-Brokamp KA, Gupta N
Respir Care 2020 Jan;65(1):111-126. Epub 2019 Oct 15 doi: 10.4187/respcare.07117. PMID: 31615921
Pulmonary Langerhans' cell histiocytosis in adults.
Radzikowska E
Adv Respir Med 2017;85(5):277-289. Epub 2017 Oct 30 doi: 10.5603/ARM.a2017.0046. PMID: 29083024

Prognosis

Smoking-Related Interstitial Lung Diseases.
Alarcon-Calderon A, Vassallo R, Yi ES, Ryu JH
Immunol Allergy Clin North Am 2023 May;43(2):273-287. Epub 2023 Mar 1 doi: 10.1016/j.iac.2023.01.007. PMID: 37055089
Pulmonary Langerhans Cell Histiocytosis.
Shaw B, Borchers M, Zander D, Gupta N
Semin Respir Crit Care Med 2020 Apr;41(2):269-279. Epub 2020 Apr 12 doi: 10.1055/s-0039-1700996. PMID: 32279297
Pulmonary Langerhans' cell histiocytosis in adults.
Radzikowska E
Adv Respir Med 2017;85(5):277-289. Epub 2017 Oct 30 doi: 10.5603/ARM.a2017.0046. PMID: 29083024
Rare pulmonary diseases: a common fight.
Harari S, Humbert M
Eur Respir Rev 2017 Sep 30;26(145) Epub 2017 Sep 6 doi: 10.1183/16000617.0059-2017. PMID: 28877977Free PMC Article
Pulmonary langerhans cell histiocytosis.
Suri HS, Yi ES, Nowakowski GS, Vassallo R
Orphanet J Rare Dis 2012 Mar 19;7:16. doi: 10.1186/1750-1172-7-16. PMID: 22429393Free PMC Article

Clinical prediction guides

Bronchoalveolar cytokine profile differentiates Pulmonary Langerhans cell histiocytosis patients from other smoking-related interstitial lung diseases.
Barril S, Acebo P, Millan-Billi P, Luque A, Sibila O, Tarín C, Tazi A, Castillo D, Hortelano S
Respir Res 2023 Dec 18;24(1):320. doi: 10.1186/s12931-023-02622-z. PMID: 38111019Free PMC Article
New insights in lymphangioleiomyomatosis and pulmonary Langerhans cell histiocytosis.
Torre O, Elia D, Caminati A, Harari S
Eur Respir Rev 2017 Sep 30;26(145) Epub 2017 Sep 27 doi: 10.1183/16000617.0042-2017. PMID: 28954765Free PMC Article
Current understanding and management of pulmonary Langerhans cell histiocytosis.
Vassallo R, Harari S, Tazi A
Thorax 2017 Oct;72(10):937-945. Epub 2017 Jul 8 doi: 10.1136/thoraxjnl-2017-210125. PMID: 28689173
Multiple cystic lung disease.
Ferreira Francisco FA, Soares Souza A Jr, Zanetti G, Marchiori E
Eur Respir Rev 2015 Dec;24(138):552-64. doi: 10.1183/16000617.0046-2015. PMID: 26621970Free PMC Article
Pulmonary Langerhans cell histiocytosis: a comprehensive analysis of 40 patients and literature review.
Elia D, Torre O, Cassandro R, Caminati A, Harari S
Eur J Intern Med 2015 Jun;26(5):351-6. Epub 2015 Apr 17 doi: 10.1016/j.ejim.2015.04.001. PMID: 25899682

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
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    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Curated

    • NCCN, 2024
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) Histiocytic Neoplasms, 2024

    Consumer resources

    Reviews

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