The 5-Year EFS of Multisystem LCH With Risk-Organ Involvement Is Suboptimal

Abstract

The study describes an 8-year experience of a single center in managing patients with langerhans cell histiocytosis on the basis of the langerhans cell histiocytosis-III platform. A retrospective case-file review of children diagnosed during 2006 to 2013 was performed. Group 1 (multisystem with risk-organ involvement) patients received an initial treatment of 6 to 12 weeks, followed by continuation treatment to complete 12 months. Drugs included vinblastine, prednisolone, and 6-mercaptopurine. Group 2 (multisystem without risk-organ involvement) patients received a similar treatment, except for 6-mercaptopurine. Group 3 (single-system/multifocal bone disease) patients were treated for a duration of 6 months. Forty-nine patients were treated: 24 (49%), 14 (28.6%), and 11 (22.4%) in groups 1, 2, and 3, respectively. The mean age at diagnosis was 31.6 ± 28.4 months (range, 4 to 120 mo). Five patients abandoned treatment. There were 7 deaths, all in group 1. All patients who died had either a partial response or progressive disease after induction (P=0.000). Among patients with liver involvement, those with sclerosing cholangitis had a greater mortality (P=0.007). A relapse was observed in 12 (24.5%) patients. The frequency of relapse was not different in the 3 groups (P=0.833). The 5-year event-free survival in groups 1, 2, and 3 was 29.3 ± 10%, 58.9 ± 14.6%, and 69.3 ± 15%, respectively (P=0.019). The 5-year overall survival was 100% in groups 2/3 and 68.9 ± 9.8% in group 1 (P=0.011).

Clinical characteristics and outcomes of Langerhans cell histiocytosis at a single institution in Thailand: a 20-year retrospective study

“…1 and 5), who had hypoalbuminemia and/or conjugated hyperbilirubinemia, but had disease progression and subsequently died from the disease. Conjugated hyperbilirubinemia could be a sign of sclerosing cholangitis with greater mortality for MS-LCH [ 12 , 38 ]. Braier et al [ 37 ] reported that hypoalbuminemia was associated with inferior prognosis in MS-LCH.…”

Solitary tibial lesion as the initial presentation of Langerhans cell histiocytosis: report of two cases and literature review

“…2,3 LCH is classified as a single system disease (SS-LCH) or multisystem disease (MS-LCH), based on the extent of system involvement. Totadri et al 4 reported that patients with SS-LCH have favourable prognoses, while patients with MS-LCH have lower rates of either five-year event-free or overall survival, particularly those with risk-organ involvement. MS-LCH involving risk organs, such as the central nervous system (CNS), haematopoietic system or liver, may lead to a rapidly fatal outcome.…”

Langerhans cell histiocytosis: An enigmatic disease

“…Another study has also reported that event-free survival for MS-LCH with risk organ involvement is suboptimal in Indian patients. [9] A couple of recent studies have reported good results in relapsed/refractory LCH and given new hope in this difficult subset of patients. However, patients with refractory/progressive disease in our series had adverse outcome.…”