Outcomes of Children Younger Than 24 Months With Langerhans Cell Histiocytosis and Bone Involvement

Kamath, Sonia; Arkader, Alexandre; Jubran, Rima

doi:10.1097/bpo.0000000000000218

Cited by 8 publications

(3 citation statements)

References 27 publications

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“…17 The clinical course of the first patient described was unique in that his LCH reactivated not in the bone, which was the primary site of the disease, but in the lungs. A similar case was reported by Kamath et al, 18 who described a patient who initially presented with single-system LCH of the bone and was treated with surgical curettage but later developed reactivation of disease in the bone, liver, spleen, and lungs.…”

Section: Discussionsupporting

confidence: 76%

Success of Trametinib in the Treatment of Langerhans Cell Histiocytosis With Novel MAPK Pathway Mutations

Orr

Hustak

Beaudoin

et al. 2022

Journal of Pediatric Hematology/Oncology

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Approximately a third of patients with Langerhans cell histiocytosis (LCH) experience recurrence of disease. Genomic analysis has revealed that this condition is often driven by oncogenic mutations in the MAP kinase (MAPK) pathway, and agents that target components of this pathway have been explored as a second-line treatment for LCH. Here, we examine 2 pediatric patients with LCH and confirmed but rarely reported MAPK pathway mutations treated with trametinib, a MAP2K inhibitor approved to treat several cancers with a BRAF V600E mutation. Each patient achieved or maintained complete resolution of disease and remain on the drug with no adverse effects.

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Section: Discussionsupporting

confidence: 76%

Success of Trametinib in the Treatment of Langerhans Cell Histiocytosis With Novel MAPK Pathway Mutations

Orr

Hustak

Beaudoin

et al. 2022

Journal of Pediatric Hematology/Oncology

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“…LCH is more common in males compared to females (9). LCH can involve a single lesion or multiple organs such as bone, skin, lung, spleen and liver (5, 10). Bone involvement occurs in approximately 80% of patients with LCH and often occurs in the skull.…”

Section: Discussionmentioning

confidence: 99%

Pericardial Effusion in Langerhans Cell Histiocytosis: A Case Report

Gholami

2016

Iran Red Crescent Med J

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IntroductionLangerhans cell histiocytosis (LCH) is a proliferative disorder of histiocytes in multiple organs. Langerhans cell histiocytosis involves bones, skin, lung and other organs.Case PresentationThis study describes a seven-month-old Iranian girl who presented with skin rash and cervical lymphadenopathy. Langerhans cell histiocytosis was suspected when it was associated with anemia, splenomegaly and lytic bone lesions. A skin biopsy confirmed the diagnosis of Langerhans cell histiocytosis. During hospitalization, the patient looked ill with respiratory distress. A chest X-ray showed a ground glass view, and echocardiography showed moderate pericardial effusion.ConclusionsPericardial effusion was a rare finding in this case of Langerhans cell histiocytosis. Pericardial effusion in Langerhans cell histiocytosis, which is an unusual presentation, should be considered when the patient experiences respiratory distress.

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“…Single system involvement can be unifocal or multifocal in nature and have an excellent prognosis but patients with multisystem disease (MS-LCH) (involvement of two or more risk organ systems) have an unpredictable course and is associated with a poor prognosis. 6 Treatment of LCH depends on the site and extent of disease at diagnosis, it may include clinical examination and observation on follow up, surgery, systemic chemotherapy or radiotherapy. 7 The aim of the study was to find out the clinical manifestations, treatment given, response to the treatment and clinical outcome in children with the diagnosis of LCH.…”

Section: Introductionmentioning

confidence: 99%

Clinical Features and Outcome in Children With Langerhans Cell Histiocytosis. A Single Institution Experience From Pakistan

Noor

Wali

Rehman

et al. 2022

PAFMJ

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Objective: To find out the clinical manifestations, treatment given and outcome of children with diagnosis of Langerhans cell histiocytosis. Study Design: Retrospective observational study. Place and Duration of Study: Shaukat Khanum Cancer Hospital, Lahore Pakistan, from Jan 2005 to Dec 2015. Methodology: Medical charts were reviewed in detail along with the available imaging for the patients. The data included age at the time of diagnosis, extent of the disease, involvement of risk organs, treatment given, response at 6th week of chemotherapy and at the end of the treatment, and outcome in terms of disease progression during the treatment, relapse of disease on follow up and cause of death either due to treatment related mortality or disease complications. Results: There were 29 patients, 12 patients (41%) had single system and 17 (58%) had multisystem involvement. 7 patients (41%) had risk organ involvement in the multisystem group. All the patients of multisystem and 6 patients of single system were treated according to the Langerhans cell histiocytosis III protocol. Commonest sites of involvement were bone in 22 (75%), followed by lymph nodes in 18 (62%) patients. Disease relapse was seen in 6 patients and all of them had multisystem disease. Mortality was observed only in multisystem Langerhans cell histiocytosis patients and more than 50% were risk organ positive. Conclusion: Langerhans cell histiocytosis is a highly heterogeneous disease. Some forms are curable without chemotherapy, while the multisystem disease requires aggressive treatment. However, despite intensive treatment, the multisystem disease and risk organs involved have poor...........

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Outcomes of Children Younger Than 24 Months With Langerhans Cell Histiocytosis and Bone Involvement

Abstract: As a retrospective review of all cases at our institution over a 26-year period, this article represents level IV evidence.

Cited by 8 publications

References 27 publications

Success of Trametinib in the Treatment of Langerhans Cell Histiocytosis With Novel MAPK Pathway Mutations

Success of Trametinib in the Treatment of Langerhans Cell Histiocytosis With Novel MAPK Pathway Mutations

Pericardial Effusion in Langerhans Cell Histiocytosis: A Case Report

Clinical Features and Outcome in Children With Langerhans Cell Histiocytosis. A Single Institution Experience From Pakistan

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