How I treat Langerhans cell histiocytosis

Abstract

“Langerhans cell histiocytosis” (LCH) describes a spectrum of clinical presentations ranging from a single bone lesion or trivial skin rash to an explosive disseminated disease. Regardless of clinical severity, LCH lesions share the common histology of CD1a+/CD207+ dendritic cells with characteristic morphology among an inflammatory infiltrate. Despite historical uncertainty defining LCH as inflammatory vs neoplastic and incomplete understanding of mechanisms of pathogenesis, clinical outcomes have improved markedly over the past decades through cooperative randomized clinical trials based on empiric therapeutic strategies. Significant advances include recognition of high- and low-risk clinical groups defined by hematopoietic and/or hepatic involvement, and of the importance of optimal intensity and of duration of chemotherapy. Nevertheless, mortality of high-risk patients, disease recurrence, lack of robustly tested salvage strategies, and significant disease morbidity of both high- and low-risk patients remain challenges. Recent discovery of recurrent somatic mutations in mitogen-activated protein kinase pathway genes at critical stages of myeloid hematopoietic differentiation in LCH patients supports redefinition of the disease as a myeloproliferative disorder and provides opportunities to develop novel approaches to diagnosis and therapy.

Langerhans Cell Histiocytosis in Children: A Single Center Experience from Turkey.

“…For this group of patients, intensive chemotherapy, alternative agents such as 2-CDA, clofarabine, cytarabine, BRAF inhibitors and HSCT should be considered. 16 In addition, it is possible to make transplantation from an HLA mismatched donor in patients with a bad clinical course.…”

“…Previously used treatments that have demonstrated success include the intralesional injection of steroids 16 ; the combination of vincristine, prednisone, and cytarabine; and 2-chlorodeoxyadenosine as a single agent. 17 In our study, 3 of 9 patients with MS risk disease were treated with 2-CDA combined ARA-C as second line therapy.…”

Adult disseminated Langerhans cell histiocytosis: incidence, racial disparities and long‐term outcomes

“…The diagnosis of LCH relies on identification of neoplastic Langerhans cells within tissue biopsies, often confirmed using phenotypic markers such as CD207 (langerin) and CD1a (Allen et al, 2015). The exact incidence of LCH in adults is unclear, and previous studies have estimated it to be 1-2 cases per million adults (Baumgartner et al, 1997).…”

Tratamiento con sirolimus y prednisona en enfermedad de Erdheim-Chester con compromiso cardiaco: Caso clínico

“…En ellos se han ensayado drogas antineoplásicas, biológicas e inmunosupresoras 3,7,[10][11][12][13][14][15][16][17][18][19][20][21] .…”