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Balkan Medical Journal Jan 2022
Topics: Histiocytosis, Langerhans-Cell; Humans; Palatine Tonsil
PubMed: 35330544
DOI: 10.4274/balkanmedj.galenos.2021.2021-6-145 -
Indian Journal of Otolaryngology and... Dec 2022Langerhans cell histiocytosis (LCH) is a rare neoplasm characterized by accumulation of histiocytes in various tissues. It has a wide clinical spectrum and its...
Langerhans cell histiocytosis (LCH) is a rare neoplasm characterized by accumulation of histiocytes in various tissues. It has a wide clinical spectrum and its presentation may mimic clinical features of common diseases. High level of suspicion is required for early diagnosis. Here is a rare case of a rapidly aggressive LCH which first presented with right zygomatic swelling.
PubMed: 36742809
DOI: 10.1007/s12070-021-02480-5 -
JAAD Case Reports Sep 2020
PubMed: 32875038
DOI: 10.1016/j.jdcr.2020.06.035 -
Cell Communication and Signaling : CCS Dec 2022Langerhans cell histiocytosis (LCH) is an inflammatory myeloid malignancy in the "L-group" histiocytosis. Mitogen-activated protein kinase (MAPK) pathway activating... (Review)
Review
Langerhans cell histiocytosis (LCH) is an inflammatory myeloid malignancy in the "L-group" histiocytosis. Mitogen-activated protein kinase (MAPK) pathway activating mutations are detectable in nearly all LCH lesions. However, the pathogenic roles of MAPK pathway activation in the development of histiocytosis are still elusive. This review will summarize research concerning the landscape and pathogenic roles of MAPK pathway mutations and related treatment opportunities in Langerhans cell histiocytosis. Video abstract.
Topics: Humans; MAP Kinase Signaling System; Histiocytosis, Langerhans-Cell; Signal Transduction; Mitogen-Activated Protein Kinases; Mutation; Proto-Oncogene Proteins B-raf
PubMed: 36536400
DOI: 10.1186/s12964-022-00917-0 -
World Journal of Clinical Cases Jul 2019Langerhans cell histiocytosis (LCH) is a rare disease of unknown aetiology. While it may affect any organ of the body, few cases of solitary lung involvement are... (Review)
Review
BACKGROUND
Langerhans cell histiocytosis (LCH) is a rare disease of unknown aetiology. While it may affect any organ of the body, few cases of solitary lung involvement are published in the literature. Here, we report a rare case of pulmonary LCH (PLCH) in an adult.
CASE SUMMARY
A 52-year-old male presented to hospital in July 2018 with complaints of progressively worsening cough with sputum, breathlessness, easy fatigability, and loss of appetite since 2016, and a 32-year history of heavy cigarette smoking (average 30 cigarettes/d). Physical examination showed only weakened breathing sounds and wheezing during lung auscultation. Chest computed tomography (CT) showed irregular micronodules and multiple thin-walled small holes. Respiratory function tests showed a slight decrease. Ultrasonic cardiogram showed mild tricuspid regurgitation and no pulmonary hypertension. Fibreoptic bronchoscopy was performed with transbronchial biopsies from the basal segment of right lower lobe. LCH was confirmed by immunohistochemistry. The final diagnosis was PLCH without extra-pulmonary involvement. We suggested smoking cessation treatment. A 3-mo follow-up chest CT scan showed clear absorption of the nodule and thin-walled small holes. The symptoms of cough and phlegm had improved markedly and appetite had improved. There was no obvious dyspnoea.
CONCLUSION
Imaging manifestations of nodules, cavitating nodules, and thick-walled or thin-walled cysts prompted suspicion of PLCH and lung biopsy for diagnosis.
PubMed: 31417936
DOI: 10.12998/wjcc.v7.i14.1892 -
Clinical Oral Investigations Jun 2021To explore the evidence of periodontal manifestations and treatment modalities in patients with Langerhans cell histiocytosis (LCH). (Review)
Review
OBJECTIVES
To explore the evidence of periodontal manifestations and treatment modalities in patients with Langerhans cell histiocytosis (LCH).
MATERIAL AND METHODS
A systematic literature search was performed and the criteria for PRISMA and risk of bias assessment were applied. Human clinical studies (≥10 patients) presenting patients with LCH and periodontal findings were considered for inclusion.
RESULTS
From 298 titles identified, six case series with a total of 1278 patients suffering from LCH were included. In these studies, oral symptoms were reported in a frequency ranging from 10 to 100%. Overall, in 216 patients (17%), oral symptoms were observed. Out of these patients, 49-100% demonstrated periodontal symptoms. The most common oral findings were pain, swelling, tooth loss/mobility, and bone lesions. Specific periodontal findings comprised varying frequencies of gingival ulcerations, increased pocket depths, and gingival bleeding. Treatment measures constituted of surgical curettage of bone lesions, soft tissue excision and/or tooth extractions, radiotherapy, systemic chemotherapy, or a combination of these approaches. Healing without recurrence of oral lesions was reported in most of the cases.
CONCLUSIONS
The available evidence on periodontal manifestations in LCH patients is heterogeneous. Several oral and periodontal findings were reported and may occur as initial symptoms and/or at later stages of the disease.
CLINICAL RELEVANCE
The dentist should be aware of possible oral involvement of systemic diseases such as LCH, and these manifestations may mimic periodontal disease.
Topics: Gingivitis; Histiocytosis, Langerhans-Cell; Humans; Oral Ulcer; Pain; Periodontal Diseases
PubMed: 33751219
DOI: 10.1007/s00784-021-03873-0 -
Acta Odontologica Latinoamericana : AOL Dec 2023Langerhans cell histiocytosis (LCH) is a disease with unknown etiology. It presents as single-system (affecting a single organ or tissue) or as multisystem (with or...
UNLABELLED
Langerhans cell histiocytosis (LCH) is a disease with unknown etiology. It presents as single-system (affecting a single organ or tissue) or as multisystem (with or without risk organ involvement). The oral cavity may be involved or be the site of the first manifestation.
AIM
To describe, group, and determine the frequency of oral lesions in pediatric patients with LCH, and to relate these lesions to age and the different disease subtypes.
MATERIALS AND METHOD
Clinical and radiographic examinations were used to evaluate 95 patients diagnosed with LCH, aged 0 to 16 years, who were referred to the Department of Comprehensive Pediatric Dentistry at the School of Dentistry, University of Buenos Aires. Clinical histories were prepared and informed consents obtained. Lesions were diagnosed by observation, palpation and biopsies, and grouped according to affected tissues into bone, mucosal, and bone-mucosal.
RESULTS
42.1% presented oral lesions, and in 14.73%, these lesions were the first manifestation of LCH. Ninety percent presented only bone lesions, while the remaining 10% presented bone-mucosal and mucosal lesions. In the single-system subtype, 52.5% presented bone lesions. In the multisystem subtypes (with or without risk organs), all three types of lesions were found. The association between age at which LCH was diagnosed and oral tissue involvement showed that bone-mucosal lesions occur in young children (average age 1.4 years) diagnosed with multisystem LCH. Oral mucosa was only affected in reactivations of the disease.
CONCLUSIONS
A high frequency of oral lesions was observed, which were sometimes the first manifestation of the disease, most often affecting bone tissue. Dentists can play an active role in the initial diagnosis of the disease.
Topics: Child; Humans; Child, Preschool; Infant; Histiocytosis, Langerhans-Cell; Bone and Bones; Mouth; Mouth Mucosa; Retrospective Studies
PubMed: 38345277
DOI: 10.54589/aol.36/3/156 -
Orphanet Journal of Rare Diseases Jul 2022Langerhans cell histiocytosis (LCH) is a myeloid dendritic cell disorder frequently affecting children more than adults. The presentation of LCH varies with age,...
BACKGROUND
Langerhans cell histiocytosis (LCH) is a myeloid dendritic cell disorder frequently affecting children more than adults. The presentation of LCH varies with age, however, the clinical characteristics and genetic profiles of adolescent LCH remain elusive. To address the knowledge gap, we performed a single-centre retrospective study of 36 adolescent LCH patients aged between 14 and 17 years at Peking Union Medical College Hospital.
RESULTS
At the time of diagnosis, 10 patients were classified as unifocal single system LCH (27.8%), 2 patients had pulmonary single system LCH (5.6%), 5 patients had multifocal single system LCH with bone involvement (13.9%), and 19 patients had multisystem LCH (52.8%). The most prevalent involvement in multisystem patients was the pituitary gland (78.9%), followed by the bone (42.1%), lung (42.1%), and lymph nodes (42.1%). Eight (42.1%) patients had risk organ involvement. BRAF was detected in 50% of patients who underwent next generation sequencing, and BRAF was detected in one patient. Chemotherapies were the first line treatment in 24 patients. One patient died and thirteen patients relapsed during the follow-up. The estimated 5-year OS rate and EFS rate were 94.7% and 59.0%, respectively.
CONCLUSIONS
In this study, we report a large series of adolescent LCH patients. The clinical characteristics of adolescent LCH patients may be close to adult LCH. Compared with pediatric cases, adolescent LCH tends to have more pituitary lesions and pulmonary involvement, fewer skin and hematopoietic involvement, a higher frequency of BRAF deletion mutation, and a lower frequency of BRAF mutation.
Topics: Adolescent; Adult; Child; Histiocytosis, Langerhans-Cell; Humans; Lymph Nodes; Mutation; Proto-Oncogene Proteins B-raf; Retrospective Studies
PubMed: 35841042
DOI: 10.1186/s13023-022-02436-0