Summary
Definition
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
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)
Treatment algorithm
Contributors
Authors
Professor of Pediatrics
University of Arkansas for Medical Sciences
Arkansas Children’s Hospital
Little Rock
AR
Disclosures
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.
Disclosures
OA declares that he has no competing interests.
Peer reviewers
Director of Pediatric Immunology, Hematology, Oncology, Bone Marrow Transplantation, and Autoimmune Diseases
Leiden University Medical Center
Leiden
The Netherlands
Disclosures
RME has previously worked with Dr Abla on a review of LCH. RME is an author of several references cited in this topic.
Assistant Professor of Pediatrics
Keck School of Medicine
University of Southern California
Children's Hospital Los Angeles
Los Angeles
CA
Disclosures
RJ declares that she has no competing interests.
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