Last reviewed: 27 Dec 2021
Last updated: 08 Feb 2019



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

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

Risk factors

  • smoking
  • family history of thyroid disease
  • perinatal infections

Diagnostic investigations

1st investigations to order

  • CBC
  • LFTs
  • serum albumin
  • coagulation studies
  • renal function
  • serum electrolytes
  • bone x-ray (skull series)
  • skeletal survey
  • chest x-ray
  • abdominal ultrasound
  • tissue biopsy

Investigations to consider

  • urine osmolality
  • full pituitary hormone evaluation
  • pulmonary function tests
  • audiometry
  • bone marrow aspirate and biopsy
  • high-resolution CT chest
  • CT abdomen
  • CT head
  • MRI brain (with contrast)
  • radionuclide bone scan (technetium-99)

Emerging tests

  • fluorodeoxyglucose positron emission tomography (FDG-PET)

Treatment algorithm



Kimo Stine, MD

Professor of Pediatrics

University of Arkansas for Medical Sciences

Arkansas Children’s Hospital

Little Rock



KS declares that he serves on the Board of the Histiocytosis Association (a nonprofit organisation). He has provided advice for a legal firm reviewing a case of LCH. He receives honoraria for membership of advisory boards to Pfizer and Behring.


Professor Kimo Stine would like to gratefully acknowledge Dr Oussama Abla, a previous contributor to this topic.


OA declares that he has no competing interests.

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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