Skip to main content

Histiocytosis, Pediatric

Michelle L. Hermiston, MD, PhD Reviewed 10/2018
 


BASICS

DESCRIPTION

  • Histiocytic disorders are derived from mononuclear phagocytic cells and dendritic cells. They are divided into three groups.

    • Dendritic cell disorders (e.g., Langerhans cell histiocyto...

DIAGNOSIS

HISTORY

  • Signs and symptoms:

    • Wide variation in presenting signs and symptoms depending on affected organ systems

    • Single-system skeletal disease may be asymptomatic, with incidental discovery of ...

TREATMENT

GENERAL MEASURES

  • Site and extent of disease determine therapy.

  • A multidisciplinary approach is imperative to ensure the best therapy. Patients should be enrolled on a clinical trial whenever p...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

  • Evaluation at regular intervals for recurrence of lesions or new high-risk organ involvement

  • Because of variable course, patients should be followed closely at cen...

ADDITIONAL READING

  • Badalian-Very G, Vergilio JA, Fleming M, et al. Pathogenesis of Langerhans cell histiocytosis. Annu Rev Pathol.  2013;8:1–20. [View Abstract on OvidInsights]

  • Filipovich A, McCla...

CODES

ICD9

  • 277.89 Other specified disorders of metabolism

  • 202.50 Letterer-siwe disease, unspecified site, extranodal and solid organ sites

ICD10

  • C96.6 Unifocal Langerhans-cell histiocytosis

  • C96.0 Multifoca...

FAQ

  • Q: Is LCH a cancer?

  • A: Historically, there has been controversy as to whether LCH was a cancer or an inflammatory disease. The discovery of BRAFV600E mutations has led to the conclusion that LCH is...

Subscribe to Access Full Content

Sign Up for a 10-Day Free Trial

Sign up for a 10-day FREE Trial now and receive full access to all content.

×