Histiocitose das células de Langerhans

Histiocyte Society[1]Emile JF, Abla O, Fraitag S, et al. Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages. Blood. 2016 Jun 2;127(22):2672-81. https://ashpublications.org/blood/article/127/22/2672/35156/Revised-classification-of-histiocytoses-and http://www.ncbi.nlm.nih.gov/pubmed/26966089?tool=bestpractice.com [4]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. https://ashpublications.org/blood/article/139/17/2601/484364/International-expert-consensus-recommendations-for http://www.ncbi.nlm.nih.gov/pubmed/35271698?tool=bestpractice.com [67]National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: histiocytic neoplasms [internet publication]. https://www.nccn.org/guidelines/category_1

O diagnóstico de HCL é baseado em achados clínicos e radiológicos em combinação com análises histopatológicas que identificam infiltração tecidual por histiócitos com características ultraestruturais ou imunofenotípicas de células de Langerhans. As células da lesão demonstram positividade para S100, CD1a e langerina (CD207).

Rede Euro Histio: estratificação de risco (HCL pediátrica)[2]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. https://onlinelibrary.wiley.com/doi/10.1002/pbc.24367 http://www.ncbi.nlm.nih.gov/pubmed/23109216?tool=bestpractice.com

HCL multissistêmica

• Baixo risco: excelente prognóstico e nenhum comprometimento de órgão de risco (ou seja, fígado, baço, medula óssea)

• Alto risco: prognóstico desfavorável e que envolve pelo menos um órgão de risco.