When viewing this topic in a different language, you may notice some differences in the way the content is structured, but it still reflects the latest evidence-based guidance.

Histiocitose das células de Langerhans

Ver PDF
  • Visão geral
  • Teoria
  • Diagnóstico
  • Tratamento
  • ACOMPANHAMENTO
  • Recursos
Última revisão das evidências: 12 Mar 2026
Última atualização do tópico: 04 Dec 2025

Resumo

Definição

História e exame físico

Principais fatores diagnósticos

  • idade <15 anos
  • dor óssea e/ou inchaço
  • erupção cutânea
  • poliúria e polidipsia
  • insuficiência de crescimento ou de maturação sexual
  • vértebra plana
  • hepatoesplenomegalia
Detalhes completos

Outros fatores diagnósticos

  • tosse, dispneia
  • dor torácica
  • anormalidades orbitais
  • icterícia
  • ascite
  • citopenias
  • febre
  • anorexia
  • perda de peso
  • irritabilidade
  • dor de ouvido
  • otorreia
  • perda auditiva
  • membrana timpânica perfurada
  • cefaleia
  • sinais neurológicos
  • diarreia hemorrágica
  • lesões da mucosa oral
  • linfadenopatia
  • aumento da tireoide
Detalhes completos

Fatores de risco

  • tabagismo
  • história familiar de doença tireoidiana
  • infecções perinatais
Detalhes completos

Investigações diagnósticas

Primeiras investigações a serem solicitadas

  • Hemograma completo
  • TFHs
  • albumina sérica
  • função renal
  • eletrólitos séricos
  • cálcio sérico
  • marcadores inflamatórios
  • avaliação endócrina (adultos)
  • Análise da mutação BRAF V600E
  • radiografia de esqueleto (crianças)
  • radiografia torácica (crianças)
  • ultrassonografia abdominal (crianças)
  • tomografia por emissão de pósitrons com fluordesoxiglucose (FDG-PET) (adultos)
  • RNM da cabeça com contraste (adultos)
  • biópsia tecidual
Detalhes completos

Investigações a serem consideradas

  • avaliação endócrina (crianças)
  • estudos de coagulação (crianças)
  • testes de função pulmonar
  • ecocardiograma
  • tomografia computadorizada (TC) de alta resolução do tórax
  • audiometria
  • aspirado e biópsia de medula óssea
  • RNM da cabeça com contraste (crianças)
  • RNM da coluna vertebral
  • tomografia computadorizada (TC) do crânio
Detalhes completos

Algoritmo de tratamento

AGUDA

doença de sistema único: comprometimento ósseo

doença de sistema único: comprometimento da pele

doença de sistema único: comprometimento dos linfonodos isolado

doença de sistema único: comprometimento do pulmão

doença de sistema único: comprometimento do SNC

doença de sistema único: doença neurodegenerativa

doença multissistêmica

CONTÍNUA

doença recidivante/refratária

Colaboradores

Autores

Gaurav Goyal, MD

Assistant Professor of Medicine

University of Alabama at Birmingham

Department of Medicine

Division of Hematology/Oncology

Birmingham

AL

Disclosures

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

Acknowledgements

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

Disclosures

OA declares that he has no competing interests. KS declares that he serves on the Board of the Histiocytosis Association (a non-profit 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

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.

Rima Jubran, MD, MPH

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.

Peer reviewer acknowledgements

BMJ Best Practice topics are updated on a rolling basis in line with developments in evidence and guidance. The peer reviewers listed here have reviewed the content at least once during the history of the topic.

Disclosures

Peer reviewer affiliations and disclosures pertain to the time of the review.

References

Our in-house evidence and editorial teams collaborate with international expert contributors and peer reviewers to ensure that we provide access to the most clinically relevant information possible.

Key articles

Haupt R, Minkov M, Astigarraga I, et al; Euro Histio Network. Langerhans cell histiocytosis (LCH): guidelines for diagnosis, clinical work-up, and treatment for patients till the age of 18 years. Pediatr Blood Cancer. 2013 Feb;60(2):175-84.Full text  Abstract

Goyal G, Tazi A, Go RS, et al. International expert consensus recommendations for the diagnosis and treatment of Langerhans cell histiocytosis in adults. Blood. 2022 Apr 28;139(17):2601-21.Full text  Abstract

Rodriguez-Galindo C, Allen CE. Langerhans cell histiocytosis. Blood. 2020 Apr 16;135(16):1319-31.Full text  Abstract

National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: histiocytic neoplasms [internet publication].Full text

Reference articles

A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.
  • Histiocitose das células de Langerhans images

  • Differentials

    • Dermatite seborreica
    • Xantogranuloma juvenil (XGJ)
    • Doença de Rosai-Dorfman (DRD)
    More Differentials

  • Guidelines

    • Clinical practice guidelines in oncology: histiocytic neoplasms
    • International expert consensus recommendations for the diagnosis and treatment of Langerhans cell histiocytosis in adults
    More Guidelines
  • padlock-lockedLog in or subscribe to access all of BMJ Best Practice

Use of this content is subject to our disclaimer