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
Autor: | Claire Dromer, Antoine Néel, Hélène Maillard, Denis Jullien, Anne-Maelle Fontenoy, Carla Araujo, Gwenaël Lorillon, Fleur Cohen-Aubart, Mohamed Hamidou, Loïc Guillevin, Mathilde de Menthon, Benoit Tessoulin, Abdellatif Tazi, Mathieu Artifoni, Jean Donadieu, Julien Haroche, Jean-Emmanuel Kahn, Olivier Hermine, Thierry Généreau |
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Rok vydání: | 2020 |
Předmět: |
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 |
Zdroj: | British Journal of Haematology. 189:869-878 |
ISSN: | 1365-2141 0007-1048 |
DOI: | 10.1111/bjh.16449 |
Popis: | 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 |
Databáze: | OpenAIRE |
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