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Langerhans cell histiocytosis

Last reviewed: 30 Oct 2023
Last updated: 31 Oct 2023



History and exam

Key diagnostic factors

  • age <15 years
  • bone pain and/or swelling
  • skin rash
  • polyuria and polydipsia
  • growth or sexual maturation failure
  • vertebra plana
  • hepatosplenomegaly
More key diagnostic factors

Other diagnostic factors

  • cough, dyspnea
  • chest pain
  • orbital abnormalities
  • jaundice
  • ascites
  • cytopenias
  • fever
  • anorexia
  • weight loss
  • irritability
  • ear pain
  • ear discharge
  • hearing loss
  • perforated tympanic membrane
  • headache
  • neurologic signs
  • bloody diarrhea
  • oral mucosa lesions
  • lymphadenopathy
  • thyroid enlargement
Other diagnostic factors

Risk factors

  • smoking
  • family history of thyroid disease
  • perinatal infections
More risk factors

Diagnostic investigations

1st investigations to order

  • CBC
  • LFTs
  • serum albumin
  • renal function
  • serum electrolytes
  • serum calcium
  • inflammatory markers
  • endocrine evaluation (adults)
  • BRAF V600E mutation analysis
  • skeletal survey (children)
  • chest x-ray (children)
  • abdominal ultrasound (children)
  • fluorodeoxyglucose positron emission tomography (FDG-PET) (adults)
  • MRI head with contrast (adults)
  • tissue biopsy
More 1st investigations to order

Investigations to consider

  • endocrine evaluation (children)
  • coagulation studies (children)
  • pulmonary function tests
  • echocardiogram
  • high-resolution CT chest
  • audiometry
  • bone marrow aspirate and biopsy
  • MRI head with contrast (children)
  • MRI spine
  • CT head
More investigations to consider

Treatment algorithm


single-system disease: bone involvement

single-system disease: skin involvement

single-system disease: isolated lymph node involvement

single-system disease: lung involvement

single-system disease: CNS involvement

single-system disease: neurodegenerative disease

multisystem disease


relapsed/refractory disease



Gaurav Goyal, MD

Assistant Professor of Medicine

University of Alabama at Birmingham

Department of Medicine

Division of Hematology/Oncology




GG has been reimbursed for advisory board by Opna Bio LLC, and receives royalties from UpToDate.


Dr Gaurav Goyal would like to gratefully acknowledge Dr Oussama Abla and Professor Kimo Stine, previous contributors to this topic.


OA declares that he has no competing interests. KS declares that he serves on the Board of the Histiocytosis Association (a nonprofit organization), has provided advice for a legal firm reviewing a case of LCH, and receives honoraria for membership of advisory boards to Pfizer and Behring.

Peer reviewers

R. Maarten Egeler, MD, PhD

Director of Pediatric Immunology, Hematology, Oncology, Bone Marrow Transplantation, and Autoimmune Diseases

Leiden University Medical Center


The Netherlands


RME has previously worked with Dr Abla on a review of LCH. RME is an author of several references cited in this topic.

Rima Jubran, MD, MPH

Assistant Professor of Pediatrics

Keck School of Medicine

University of Southern California

Children's Hospital Los Angeles

Los Angeles



RJ declares that she has no competing interests.

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  • Guidelines

    • Clinical practice guidelines in oncology: histiocytic neoplasms
    • International expert consensus recommendations for the diagnosis and treatment of Langerhans cell histiocytosis in adults
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