Reactivations in Multisystem Langerhans Cell Histiocytosis: Data of the International LCH Registry

• Published in: The Journal of pediatrics Vol. 153; no. 5; pp. 700 - 705.e2
• Main Authors: Minkov, Milen, MD, PhD, Steiner, Manuel, MD, Pötschger, Ulrike, PhD, Aricò, Maurizio, MD, Braier, Jorge, MD, Donadieu, Jean, MD, Grois, Nicole, MD, Henter, Jan-Inge, MD, PhD, Janka, Gritta, MD, McClain, Kenneth, MD, PhD, Weitzman, Sheila, MD, Windebank, Kevin, MD, Ladisch, Stephan, MD, Gadner, Helmut, MD, FRCP (Glasgow)
• Format: Journal Article
• Language: English
• Published: Maryland Heights, MO Mosby, Inc 2008; Elsevier
• Subjects: Biological and medical sciences; Clinical Trials as Topic; Diabetes Insipidus, Neurogenic - diagnosis; Diabetes Insipidus, Neurogenic - pathology; Female; General aspects; Hematologic and hematopoietic diseases; Histiocytosis, Langerhans-Cell - diagnosis; Histiocytosis, Langerhans-Cell - mortality; Histiocytosis, Langerhans-Cell - physiopathology; Humans; Infant; International Cooperation; Male; Medical sciences; Medicin och hälsovetenskap; Other diseases. Hematologic involvement in other diseases; Pediatrics; Probability; Prospective Studies; Recurrence; Registries; Retrospective Studies; Risk; Treatment Outcome; World; SS-R; NAD1; DI; NAD2; Diabetes insipidus; MS-LCH; Second complete disease resolution (non-active disease); SS-LCH; MS-R; Single-system reactivation; R1; Permanent consequences; R2; Langerhans cell histiocytosis; Second reactivation of the disease; Multisystem reactivation; Risk-organ involvement not present; LCH; Single-system LCH; Multisystem LCH; PC; Risk-organ involvement present; First complete disease resolution (non-active disease); First reactivation of the disease; RO; Pediatrics; Reactivation; Register; Hemopathy; Data; International
• ISSN: 0022-3476; 1097-6833; 1097-6833
• DOI: 10.1016/j.jpeds.2008.05.002

Objective To assess multisystem Langerhans cell histiocytosis reactivation and its impact on morbidity and mortality. Study design Retrospective analysis of 335 patients with MS-LCH and documented complete disease resolution (NAD1). Results The probability of a reactivation within 5 years of NAD1 was 46%. The first reactivation occurred within 2 years after NAD1 in most of the patients. Of 134 events, 35% were confined to skeleton, 24% were single-system nonbony lesions, 24% were multisystem reactivations without risk-organ involvement, and 10% with risk-organ involvement. In 7%, the location was unspecified. Only 3 deaths (2.2%) were documented within the context of a first reactivation. Second disease resolution (NAD2) was achieved in 85% of the cases. The probability of a second reactivation within 5 years of NAD2 was 44%. The risk for permanent consequences in patients with reactivations was higher, compared with patients without reactivation (RHR 2.2, P = .046). Conclusions Reactivation is a frequent and early event in MS-LCH, but involvement of risk organs at reactivation is rare and mortality is minimal. However, reactivations increase the risk for permanent consequences by about 2-fold. Prospective trials targeting reduction of acute morbidity and permanent disabilities through nontoxic treatment of the reactivations are warranted.