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Characteristics and Treatment Outcomes of Pediatric Langerhans Cell Histiocytosis with Thymic Involvement.
Yao, Ja-Feng; Wang, Dong; Ma, Hong-Hao; Lian, Hong-Yun; Zhang, Li; Wang, Tian-You; Li, Zhi-Gang; Jiang, Jin; Cui, Lei; Zhang, Rui.
Afiliação
  • Yao JF; Hematology Center, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China; Beijing Key Laboratory of Pediatric Hematology Oncology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China;
  • Wang D; Hematology Center, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China; Beijing Key Laboratory of Pediatric Hematology Oncology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China;
  • Ma HH; Hematology Center, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China; Beijing Key Laboratory of Pediatric Hematology Oncology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China;
  • Lian HY; Hematology Center, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China; Beijing Key Laboratory of Pediatric Hematology Oncology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China;
  • Zhang L; Hematology Center, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China; Beijing Key Laboratory of Pediatric Hematology Oncology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China;
  • Wang TY; Hematology Center, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China; Beijing Key Laboratory of Pediatric Hematology Oncology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China;
  • Li ZG; Beijing Key Laboratory of Pediatric Hematology Oncology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China; National Key Discipline of Pediatrics, Capital Medical University, Beijing, China; Key Laboratory of Major Diseases in Children, Mi
  • Jiang J; Hematology Center, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China; Beijing Key Laboratory of Pediatric Hematology Oncology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China;
  • Cui L; Beijing Key Laboratory of Pediatric Hematology Oncology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China; National Key Discipline of Pediatrics, Capital Medical University, Beijing, China; Key Laboratory of Major Diseases in Children, Mi
  • Zhang R; Hematology Center, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China; Beijing Key Laboratory of Pediatric Hematology Oncology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China;
J Pediatr ; 244: 194-202.e5, 2022 05.
Article em En | MEDLINE | ID: mdl-35065150
OBJECTIVE: To evaluate the characteristics and treatment outcomes of patients with pediatric Langerhans cell histiocytosis (LCH) with thymic involvement. STUDY DESIGN: We retrospectively described the clinical, biological, and imaging characteristics of a series of 19 patients with pediatric LCH with thymic involvement in our center between September 2016 and December 2019. We further analyzed the treatment response and outcomes of patients treated with chemotherapy or targeted therapy. RESULTS: Thymic involvement was found in 4.4% of a 433-consecutive pediatric LCH cohort; all LCH-thymic involvement presented with multisystem disease. Patients with thymic involvement were typically younger, harboring more lung and thyroid involvement and less bone involvement than those without thymic involvement. Most patients with thymic involvement had alteration of immunocompetence with decreased numbers of T-lymphocyte subsets and immunoglobulin G levels. Overall, 47.1% of patients demonstrated a response after 6 weeks of induction therapy, and 92.3% of the patients who did not respond to the first-line treatment had resolution of thymus after the second-line and/or targeted therapy. The progression/relapse rate showed no difference between patients who shifted to second-line therapy and those to dabrafenib (33.3% vs 25%, P = 1.000). The survival for patients with thymic involvement did not differ from those without thymic involvement. More patients treated with second-line chemotherapy had severe adverse events than those given dabrafenib (88.9% vs 0, P < .001). CONCLUSIONS: Thymic involvement was observed rarely in LCH and had specific clinical characteristics. Chemotherapy could resolve most thymic lesions, and BRAF inhibitors might provide a promising treatment option with less toxicity for infants with BRAF-V600E mutation. TRIAL REGISTRATION: http://www.chictr.org.cn, identifier: ChiCTR2000030457 (BCH-LCH 2014 study); ChiCTR2000032844 (dabrafenib study).
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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Histiocitose de Células de Langerhans / Proteínas Proto-Oncogênicas B-raf Tipo de estudo: Diagnostic_studies / Observational_studies / Prognostic_studies Limite: Child / Humans / Infant Idioma: En Revista: J Pediatr Ano de publicação: 2022 Tipo de documento: Article País de publicação: Estados Unidos

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Histiocitose de Células de Langerhans / Proteínas Proto-Oncogênicas B-raf Tipo de estudo: Diagnostic_studies / Observational_studies / Prognostic_studies Limite: Child / Humans / Infant Idioma: En Revista: J Pediatr Ano de publicação: 2022 Tipo de documento: Article País de publicação: Estados Unidos