Pulmonary langerhans cell histiocytosis

An update from the pathologists' perspective

Anja Roden, Eunhee S. Yi

Research output: Contribution to journalArticle

24 Citations (Scopus)

Abstract

Context. - Pulmonary Langerhans cell histiocytosis (PLCH) is a rare histiocytic disorder that almost exclusively affects the lungs of smokers. PLCH is characterized by bronchiolocentric nodules and/or cysts in an upper and mid lung distribution with sparing of the costophrenic angles. The diagnosis can be challenging and often requires transbronchial biopsy or surgical lung biopsy. Pulmonary hypertension is a relatively common and sometimes severe complication of PLCH. The pathogenesis of PLCH is still debated. Recently, BRAF V600E mutation and BRAF expression have been identified in some patients with PLCH, suggesting that at least a subset of PLCH has a clonal proliferation. While smoking cessation is the first-line treatment of PLCH, some patients might require additional treatment and eventually transplant. Given that the lesional cells of PLCH express BRAF in some patients, MAPKinase pathway-targeted treatment might be useful for therapy-resistant patients. Objective. - To present the more recently recognized clinical and pathologic aspects of PLCH, including pulmonary hypertension in PLCH, pathogenesis, and treatment, as well as the basic diagnostic approach to PLCH. Data Sources. - Authors' own research, and search of literature database (PubMed) and UpToDate. Conclusions. - Despite the recent progress, more studies are needed to elucidate the biology of PLCH for identification of prognostic factors and appropriate treatment options, especially for therapy-refractory PLCH cases.

Original languageEnglish (US)
Pages (from-to)230-240
Number of pages11
JournalArchives of Pathology and Laboratory Medicine
Volume140
Issue number3
DOIs
StatePublished - Mar 1 2016

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Langerhans Cell Histiocytosis
Lung
Pathologists
Pulmonary Hypertension
Therapeutics
Biopsy
Information Storage and Retrieval
Smoking Cessation

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Medical Laboratory Technology

Cite this

Pulmonary langerhans cell histiocytosis : An update from the pathologists' perspective. / Roden, Anja; Yi, Eunhee S.

In: Archives of Pathology and Laboratory Medicine, Vol. 140, No. 3, 01.03.2016, p. 230-240.

Research output: Contribution to journalArticle

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