Langerhans cell histiocytosis: progress and controversies

Abla, Oussama; Rollins, Barrett J.; Ladisch, Stephan

doi:10.1111/bjh.16099

Cited by 25 publications

(20 citation statements)

References 20 publications

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“…2,19 Although survival is highly dependent on whether risk organs are involved or not, tremendous progress has been made in the management of LCH, with significant improvement of survival in high risk LCH up to around 90%. 17 Our study showed that among patients having MS-RO+ (n=32, 49%) of total cohort only 34% completed treatment and are doing well. Of the remaining, 32 % abandoned treatment andthe rest of 34% expired.…”

Section: Discussionmentioning

confidence: 58%

“…Continued efforts are needed to decrease reactivations, but unfortunately the toxicity of the treatment of LCH cannot be ignored. 17,18 In LCH reactivation is not uncommonwith relapses in 20% to 50% of patients. Generally, in both groups of patients with low-risk SS-LCH having reactivation of multifocal bone disease or low-risk MS-LCH/RO-, reactivations happen in approximately one-third of patients with good response to second-line regimens.…”

Section: Discussionmentioning

confidence: 99%

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Overview of Langerhans Cell Histiocytosis among Children

Ahmad¹,

Hussain²,

Yaseen³

et al. 2021

PJMHS

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Aim: To analyze clinical manifestations, course and outcome of Langerhans Cell Histiocytosis in children in resource limited settings lacking salvage therapy. Study design: Observational retrospective study Place and duration of study: Department of Haematology/Oncology, The Children’s Hospital, Lahore Pakistan from 1st January 2011 to 31st December 2018. Methodology: Sixty-five patients with age range from<1 to 8 years included analysing their age, gender, clinical classification, course of therapy and outcome. The major treatment was composed of either prednisolone and vinblastine or cytarabine pulses. Results: There were 59% males and 41% females. Forty-seven (72%) patients presented with multi system-LCH with 49% Risk Organ involvement. Most of them 42 (65%) had bone lesions while 15patients (23%) presented with central nervous system involvement. Forty patients (61%) have completed treatment, 11(17%) left against medical advice and 12(18%)patients expired due to progressive disease and worsening infection. Only 2 patients were put on palliation with progressive brain parenchyma disease. 22 patients (34%) had reactivations of disease requiring therapy for more than one-year (p-value=0.06), while 15 (23%) patients received two cycles of initiation therapy before continuation therapy started. The treatment initiated >6 months after the onset of symptoms in 48 (74%) patients. Conclusion: Early diagnosis and timely initiation of therapy are of utmost importance to reduce mortality and morbidity. There is a dire need of social support to reduce treatment abandonment in low-middle-income countries LMIC. Keywords: Paediatric Langerhans cell histiocytosis, Resource-limited settings, Delayed diagnosis, Abandonment

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Section: Discussionmentioning

confidence: 58%

Section: Discussionmentioning

confidence: 99%

Overview of Langerhans Cell Histiocytosis among Children

Ahmad¹,

Hussain²,

Yaseen³

et al. 2021

PJMHS

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“…LCH is a histiocytosis disease described by aberrant cellular functions, proliferation or differentiation of mononuclear phagocyte system cells (14). LCH is clinically heterogeneous, ranging from single system involvement to multiple system involvements.…”

Section: Discussionmentioning

confidence: 99%

A Risk Model Composed of Complete Blood Count, BRAF V600E and MAP2K1 Predicts Inferior Prognosis of Langerhans Cell Histiocytosis in Children

Wang

Yuan

et al. 2022

Front. Oncol.

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BackgroundIn children, Langerhans cell histiocytosis (LCH), which is the most prevalent histiocytic disorder, exhibits a wide variety of manifestations and outcomes. There is no standard prognosis evaluation system for LCH. We investigated the combined predictive significance of complete blood counts (CBCs), BRAF V600E and MAP2K1 in childhood LCH.MethodsA cohort of 71 childhood LCH patients was retrospectively studied. The prognosis predictive significance of platelet-to-lymphocyte ratio (PLR), neutrophil-to-lymphocyte ratio (NLR), systemic inflammation response index (SIRI), systemic immune inflammation index (SII), BRAF V600E, and MAP2K1 were analyzed.ResultsHistiocyte Society (HS) classification of LCH patients was correlated with NLR, SIRI, and progression free survival (PFS), bone involvement was correlated with SIRI, liver involvement was correlated with NLR, SII, SIRI, and PFS, spleen involvement was correlated with SIRI, lung involvement was correlated with NLR and PFS, CNS involvement was correlated with PFS, while BRAF V600E was correlated with PLR, NLR, SIRI, SII, PFS, and OS (p <0.05). MAP2K1 was correlated with NLR, SIRI, PFS, and OS (p <0.05). Elevated NLR, PLR SIRI, and SII predicted inferior PFS and OS (p <0.05). PLR, NLE, SIRI, SII, BRAF V600E, and MAP2K1 were used to establish a risk model for stratifying the LCH patients into 3 different risk groups. Respective median PFS for low-, mediate-, and high-risk groups were not reached, 26, and 14 months (p <0.001), and all median OS were not reached (p <0.001).ConclusionThe risk model combined with CBCs, BRAF V600E, and MAP2K1 might be a promising prognostic system for LCH in children.

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“…Langerhans cell histiocytosis (LCH), the most common histiocytic disorder (Allen, Merad & McClain, 2018) previously known as Histiocytosis X (the "X" denoting unknown etiology) (Abla, Rollins & Ladisch, 2019), is a disorder characterized by abnormal proliferation of CD1a+/S-100/CD207+ Langerhans cells (Rodriguez-Galindo & Allen, 2020;Tenorio et al, 2020), with a peak of incidence among children between 1 and 3 years of age (Postini et al, 2012), and is more prevalent in males than females (Guyot-Goubin et al, 2008). LCH may affect the bones, skin, liver, lung, and hematopoietic and neuroendocrine systems.…”

Section: Introductionmentioning

confidence: 99%

Langerhan’s cell histiocytosis with oral manifestation in a 3-year-old child: a case report

Vendruscolo

Wastner

Patussi

et al. 2021

RSD

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Langerhans cell histiocytosis (LCH) is a disorder that may affect the bones, skin, liver, lung, and hematopoietic and neuroendocrine systems. This condition may manifest as a single lesion, multiple lesions, or as a disseminated and potentially fatal disease. We aim to report a case of a 3-year-old child with LCH in the mandible, sharing with the readers the challenging process of this diagnosis. A three-year-old male patient with persistent swelling in the right submandibular region was referred to the Department of Pediatrics of the Erasto Gaertner Hospital for an evaluation. Initial physical exam revealed a diffuse flaccid swelling occupying the entire right mandibular ramus, from the angle to the preauricular region and CT scan showed an osteolytic lesion with erosion of the internal and external cortices of the mandible and an extension to soft tissues that displaced the masseter muscle. Immunohistochemical analysis confirmed the diagnosis of Langerhans cell histiocytosis through positive tests for CD1a, CD68, S-100, and Vimentin. The treatment proposed was a combination of Vinblastine 6 mg/m3 for 6 weeks and Prednisone 40mg for 4 weeks.The differential diagnosis included pathologies such as rhabdomyosarcoma, Ewing's sarcoma, and, less likely, osteosarcoma and central giant cell granuloma.

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Langerhans cell histiocytosis: progress and controversies

Cited by 25 publications

References 20 publications

Overview of Langerhans Cell Histiocytosis among Children

Overview of Langerhans Cell Histiocytosis among Children

A Risk Model Composed of Complete Blood Count, BRAF V600E and MAP2K1 Predicts Inferior Prognosis of Langerhans Cell Histiocytosis in Children

Langerhan’s cell histiocytosis with oral manifestation in a 3-year-old child: a case report

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