Pediatric langerhans cell histiocytosis: single center experience over a 17-year period

İnce, Dilek; Demirağ, Bengü; Özek, Gülcihan; Erbay, Ayşe; Ortaç, Ragıp; Oymak, Yeşim; Serra, Kamer; Yaman, Yöntem; Kundak, Selcen; Vergin, Canan

doi:10.24953/turkjped.2016.04.001

Cited by 10 publications

(19 citation statements)

References 25 publications

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“…This study presents data on 66 consecutive patients with LCH, treated over a 45‐year period by a single reference centre, the Department of Pediatric Hematology‐Oncology of the ‘Aghia Sophia’ Children's Hospital in Athens. The male predominance was 1.5:1, which was consistent with previous studies that reported male:female ratios of 1.2–1.8:1 1,18,19 . Two‐thirds of our patients were diagnosed with single system LCH, mostly of the bone.…”

Section: Discussionsupporting

confidence: 91%

“…This is consistent with previous results showing that single system LCH accounts for 42-69% of the total cases. 1,[18][19][20] The median age at diagnosis was significantly younger in patients with multisystem than single system LCH in our study (1.4 years vs.…”

Section: Discussionmentioning

confidence: 47%

“…The male predominance was 1.5:1, which was consistent with previous studies that reported male:female ratios of 1.2-1.8:1. 1,18,19 Two-thirds of our patients were diagnosed with single system LCH, mostly of the bone. This is consistent with previous results showing that single system LCH accounts for 42-69% of the total cases.…”

Section: Discussionmentioning

confidence: 96%

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Long‐term outcome, clinical course and treatment approaches of paediatric langerhans cell histiocytosis: A greek reference centre report

et al. 2021

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Aim: Langerhans cell histiocytosis (LCH) is an inflammatory myeloid neoplasia with diverse clinical behaviour. In this article, we studied the clinical course, management and long-term outcomes of a paediatric cohort treated by our reference centre. Methods:We retrospectively studied 66 children with LCH, consecutively diagnosed by a Greek reference centre from 1974 to 2020. Results:The patients had a median age of 3.9 (range 0.0-15.9) years, 39 and 6 patients were diagnosed with unifocal or multifocal single system disease and 14 and 7 had multisystem disease with or without risk organ involvement. No late occurrence of clinical neurodegenerative disease or diabetes insipidus were observed at a median follow-up period of 4.1 (range 0.5-27.7) years. The 10-year event-free survival and overall survival were 65.0% and 90.3% and improved significantly over a 45-year period. Survival was superior in single system than multisystem cases. BRAF V600E mutation was found in 8/14 tested patients. Reactivation occurred in 12/66 patients (18.2%); 11 achieved remission and one patient died after a second relapse. Conclusion:LCH survival rates significantly increased in our cohort over time.Reactivation occurred in 18.2% patients, but no late neurodegeneration was found.The prognostic value of single system disease status vs. multisystem LCH was confirmed.

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Section: Discussionsupporting

confidence: 91%

Section: Discussionmentioning

confidence: 47%

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Long‐term outcome, clinical course and treatment approaches of paediatric langerhans cell histiocytosis: A greek reference centre report

et al. 2021

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“…Su et al [ 11 ] reported 100% 5-year survival in pediatric LCH patients when the disease involved only bone[ 11 ]. In a long-term (over 17 years) follow-up study of pediatric LCH, SS-LCH showed a 100% regression rate and low relapse vs 73% regression in MS-LCH[ 12 ].…”

Section: Introductionmentioning

confidence: 99%

“…LCH involving the skin, bone, lymph nodes or pituitary gland is considered “low-risk” because of its good response to treatment. Involvement of the lungs, hematopoietic system and spleen, and liver is considered “high risk” with an unfavorable outcome[ 1 , 6 , 12 , 13 ].…”

Section: Introductionmentioning

confidence: 99%

Hepatic Langerhans cell histiocytosis: A review

Fu¹,

Li²,

Arslan³

et al. 2021

WJCO

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Hepatic Langerhans cell histiocytosis (LCH) is characterized by proliferation and accumulation of Langerhans cells in the liver, causing liver dysfunction or forming a mass lesion. The liver can be involved in isolation, or be affected along with other organs. A common clinical hepatic presentation is cholestasis with pruritis, fatigue and direct hyperbilirubinemia. In late stages, there may be hypoalbuminemia. Liver biopsy may be required for the diagnosis of hepatic LCH. Histologic finding may be diverse, including lobular Langerhans cell infiltrate with mixed inflammatory background, primary biliary cholangitis-like pattern, sclerosing cholangitis-like pattern, and even cirrhosis at later stages. Because of its non-specific injury patterns with broad differential diagnosis, establishing a diagnosis of hepatic LCH can be challenging. Hepatic LCH can easily be missed unless this diagnosis is considered at the time of biopsy interpretation. A definitive diagnosis relies on positive staining with CD1a and S100 antigen. Liver involvement is a high risk feature in LCH. The overall prognosis of hepatic LCH is poor. Treating at an early stage may improve the outcome. Systemic chemotherapy is the mainstay of treatment and liver transplantation may be offered. New molecular markers involved in pathogenesis of LCH are being explored with a potential for targeted therapy. However, further studies are needed to improve outcome.

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