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Long‐term efficacy and safety of 2CdA (cladribine) in extra‐pulmonary adult‐onset Langerhans cell histiocytosis: analysis of 23 cases from the French Histiocytosis Group and systematic literature review
- Source :
- British Journal of Haematology. 189:869-878
- Publication Year :
- 2020
- Publisher :
- Wiley, 2020.
-
Abstract
- Langerhans cell histiocytosis (LCH) is a rare protean disease that usually affects children. Few data are available for management of adult-onset cases. A complete picture of the efficacy and safety of 2CdA (2-chlorodeoxyadenosine, cladribine) is lacking. We report a retrospective multicentre study of 23 adult LCH (a-LCH) patients who received single-agent 2CdA and a systematic literature review. All had previously received systemic therapy (vinblastine, n = 19). Response to 2CdA was evaluable in 22 cases. Overall response rate (ORR) was 91%. Complete response (CR) occurred in 11 cases (50%). Nine patients (39%) developed grade 3-4 neutropenia and/or severe infection. A literature review yielded 48 additional cases. A pooled analysis confirmed our findings (ORR: 88%, CR: 49%). CRs were rare with cumulative dose
- Subjects :
- Adult
Male
medicine.medical_specialty
Neutropenia
Antimetabolites
medicine.medical_treatment
Kaplan-Meier Estimate
Gastroenterology
03 medical and health sciences
0302 clinical medicine
Langerhans cell histiocytosis
Sepsis
Internal medicine
medicine
Humans
Age of Onset
Cladribine
Proportional Hazards Models
Retrospective Studies
Aged, 80 and over
Chemotherapy
Dose-Response Relationship, Drug
Cumulative dose
business.industry
Remission Induction
Hematology
Middle Aged
medicine.disease
Vinblastine
Histiocytosis, Langerhans-Cell
Histiocytosis
Virus Diseases
030220 oncology & carcinogenesis
Cytarabine
Female
France
business
Immunosuppressive Agents
030215 immunology
medicine.drug
Subjects
Details
- ISSN :
- 13652141 and 00071048
- Volume :
- 189
- Database :
- OpenAIRE
- Journal :
- British Journal of Haematology
- Accession number :
- edsair.doi.dedup.....9c1537d8c8eea94e1c0426535df1e0c0