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

Summary 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 multicentr...

Full description

Saved in:
Bibliographic Details
Published in:British journal of haematology 2020-06, Vol.189 (5), p.869-878
Main Authors: Néel, Antoine, Artifoni, Mathieu, Fontenoy, Anne‐Maelle, Tessoulin, Benoit, Lorillon, Gwenaël, Cohen‐Aubart, Fleur, Haroche, Julien, Genereau, Thierry, Menthon, Mathilde, Guillevin, Loïc, Maillard, Hélène, Kahn, Jean‐Emmanuel, Hermine, Olivier, Araujo, Carla, Dromer, Claire, Jullien, Denis, Hamidou, Mohamed, Donadieu, Jean, Tazi, Abdellatif
Format: Article
Language:English
Subjects:
2‐chlorodeoxyadenosine
Adult
Age of Onset
Aged, 80 and over
Antimetabolites - adverse effects
Antimetabolites - therapeutic use
chemotherapy
Cladribine
Cladribine - adverse effects
Cladribine - therapeutic use
Cytarabine
Dose-Response Relationship, Drug
Female
France - epidemiology
Hematology
Histiocytosis
Histiocytosis, Langerhans-Cell - drug therapy
Histiocytosis, Langerhans-Cell - mortality
Humans
Immunosuppressive Agents - adverse effects
Immunosuppressive Agents - therapeutic use
Kaplan-Meier Estimate
Langerhans cell histiocytosis
Literature reviews
Male
Middle Aged
Neutropenia
Neutropenia - chemically induced
Prophylaxis
Proportional Hazards Models
Remission
Remission Induction
Retrospective Studies
Sepsis - etiology
Sepsis - mortality
Systematic review
Toxicity
treatment
Vinblastine
Virus Diseases - etiology
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Summary 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
ISSN:0007-1048
1365-2141
DOI:10.1111/bjh.16449