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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 -
Otolaryngology--head and Neck Surgery :... Jan 2022To perform a systematic review to investigate common otologic manifestations of Langerhans cell histiocytosis, the incidence of these findings, methods for diagnosis, as...
OBJECTIVE
To perform a systematic review to investigate common otologic manifestations of Langerhans cell histiocytosis, the incidence of these findings, methods for diagnosis, as well as medical and surgical management.
DATA SOURCES
PubMed/MEDLINE, Embase, and Cochrane Library.
REVIEW METHODS
A search of PubMed/MEDLINE, Embase, and Cochrane Library for all articles published between 1963 to 2020 was performed with variations and combinations of the following search terms: Langerhans cell histiocytosis, eosinophilic granuloma, Letterer-Siwe, Hand-Schüller-Christian, otitis, otologic, ear. A review of the references of all included articles was also conducted.
RESULTS
Sixty-two articles encompassing 631 patients met inclusion criteria. Otologic symptoms at presentation were found in 246 (39%) patients in the reported studies with 48% reporting bilaterality. The mean age was 14.8 years with a male predominance (64%). The most common otologic presenting symptom was otorrhea (46%). A majority had the multisystem variant (52%). The most common treatment modalities were chemotherapy (52%), followed by surgery (50%), systemic steroids (45%), and radiotherapy (31%). Surgery was performed in 75.8% with unisystem involvement and in 50.6% with multisystem involvement. The most effective treatments included radiotherapy (56% success rate, 17% of treated patients), systemic steroids (44% success, 20% treated), chemotherapy (41% success, 21% treated), and surgical modalities (36% success, 19% treated).
CONCLUSIONS
Otologic manifestations that occur with the multisystem variant or are at high risk for central nervous system involvement necessitate systemic treatment. For unifocal lesions, surgery is recommended. Lastly, radiotherapy should be reserved for extensive lesions involving vital structures or presenting in older patients.
Topics: Ear Diseases; Female; Histiocytosis, Langerhans-Cell; Humans; Incidence; Male; Prognosis
PubMed: 33945752
DOI: 10.1177/01945998211004590 -
Oral and Maxillofacial Surgery Dec 2021The study aimed to identify, enlist, and analyze cases of unisystem LCH in the maxillofacial pediatric population to understand the clinical presentation and encourage...
BACKGROUND
The study aimed to identify, enlist, and analyze cases of unisystem LCH in the maxillofacial pediatric population to understand the clinical presentation and encourage the consideration of this rare disease in the differential diagnosis. Langerhans cell histiocytosis (LCH) is an aggressive benign condition affecting mainly the pediatric population. It can be easily masked as periodontal disease in the maxillofacial region. Early diagnosis and a systemic evaluation are of utmost importance.
METHODOLOGY
We are presenting a complete review of literature in the pediatric population according to PRISMA guidelines for clinicopathologic, histopathological, immunohistochemistry, and treatment for unisystem LCH. The risk of bias assessment across studies was done using a Case series appraisal checklist by Guo et al. 53 RESULTS: Forty-nine articles (152 cases) were selected which met our inclusion and exclusion criteria to be included in our review. Most of the patients fall in 6-12 years of age with the involvement of the mandibular body region in 40.79% cases. This disease mainly presents as erythematous gingiva, pain, swelling, and mobile teeth. Management can range from minimal intervention to chemotherapy and surgery.
CONCLUSION AND PRACTICAL IMPLICATIONS
Although this is a rare condition, it should be considered especially in the pediatric population with periodontitis type lesions and floating teeth and comprehensive management should be followed. Early diagnosis of the disease is very important.
Topics: Child; Diagnosis, Differential; Gingiva; Histiocytosis, Langerhans-Cell; Humans; Mandible; Pediatrics
PubMed: 33591444
DOI: 10.1007/s10006-021-00949-9 -
Orphanet Journal of Rare Diseases Jan 2021Langerhans cell histiocytosis (LCH) is a rare disease that originates from the uncontrolled proliferation and accumulation of bone marrow-derived immature myeloid... (Review)
Review
BACKGROUND
Langerhans cell histiocytosis (LCH) is a rare disease that originates from the uncontrolled proliferation and accumulation of bone marrow-derived immature myeloid dendritic cells. Dendritic cells are a type of histiocyte that play an important role in the human immune system and are found in the bone, skin, stomach, eyes, intestines, and lungs.
OBJECTIVE
This systematic review aimed to collect and report published case reports of rare bone disease caused by LCH to avoid misdiagnoses or delays in diagnosis.
METHODS
We systematically searched Scopus, PubMed, Embase, and Web of Sciences from August 1, 2000 to December 31, 2019. Studies reporting cases of LCH with rare bone involvement were included.
RESULTS
We identified 60 articles including 64 cases. Of the identified cases, 31 (48.4%) involved children, and 33 (51.6%) involved adults. Additionally, 46.9% (30 individuals) were from Asian countries. The mean age of the children was 7.6 ± 4.3 years and that of the adults was 36 ± 12 years. The findings indicated that unifocal bone involvements were the most prevalent form of the disease (68.7%), and, overall, the skull and chest wall were the most commonly affected bones in both adults and children. The spine and long bones were the second most commonly affected bones in children, and the spine and jaw were the second most commonly affected bones in adults. Pain and swelling were the most frequent presenting signs among the investigated cases, and loss of consciousness, myelopathy, nerve palsy, visual loss, torticollis and clicking sounds were rare signs. Osteolytic lesions were the most frequent radiologic feature (62.5%), and intracranial hemorrhage, fluid-fluid level, dura and intracranial extension and pathologic fractures were rare radiological features. Total excision, curettage and observation in the unifocal group of patients and systemic chemotherapy in the other groups (i.e., multifocal and multisystem) were the most frequent management approaches. The recovery rates of the unifocal and multifocal groups were 77.3% and 81.8%, respectively, while that of the multisystem group was 55.5%. The rates of recurrence and mortality in the multisystem group were 11% and were higher than those in the other groups.
CONCLUSIONS
LCH is a rare disease that can affect any organ in the human body. However, bone is the most commonly involved organ, and rare bone involvements may be the first or only symptom of the disease due to the rarity of such lesions; a lack of familiarity with them may result in misdiagnosis or delayed diagnosis.
Topics: Adult; Asia; Bone Diseases; Child; Child, Preschool; Histiocytosis, Langerhans-Cell; Humans; Retrospective Studies; Skull
PubMed: 33388073
DOI: 10.1186/s13023-020-01625-z -
Otolaryngology--head and Neck Surgery :... Dec 2022Acute mastoiditis is commonly attributed to infection. Rarely do clinicians encounter cases that do not respond to traditional antibiotics or surgical management. The... (Review)
Review
OBJECTIVE
Acute mastoiditis is commonly attributed to infection. Rarely do clinicians encounter cases that do not respond to traditional antibiotics or surgical management. The goal of this study was to systematically review the literature to characterize diseases masquerading as acute infectious mastoiditis.
DATA SOURCES
PubMed, Embase, and Scopus.
REVIEW METHODS
A systematic review was performed to identify all publications that reported on diseases with presentations mimicking acute mastoiditis, defined as postauricular redness, swelling, and tenderness. We included clinical prospective studies, retrospective studies, and case series/reports. Exclusion criteria included non-English articles, letters/commentaries, abstracts, and review articles.
RESULTS
Out of 3339 results, 35 studies met final inclusion criteria. In children, 11 diseases were reported to mimic mastoiditis, including solid tumors, hematologic diseases, and autoimmune/inflammatory diseases. The most common disease in children was Langerhans cell histiocytosis, followed by rhabdomyosarcoma and acute myelogenous leukemia. In adults, 8 additional diseases were reported. The most common disease in adults was squamous cell carcinoma, followed by nasopharyngeal carcinoma and Langerhans cell histiocytosis. Presenting symptoms are reviewed, as well as characteristic radiographic, laboratory, and intraoperative features that may assist with diagnosis. A diagnostic algorithm for atypical cases of acute mastoiditis is proposed.
CONCLUSION
A small but significant group of diseases in children and adults can mimic acute mastoiditis. In such cases, history and examination alone may be insufficient to reach a diagnosis, and further investigation may be necessary. Otolaryngologists should always be mindful of the possibility that noninfectious pathologies may present with a constellation of symptoms similar to mastoiditis.
Topics: Child; Humans; Infant; Mastoiditis; Retrospective Studies; Noncommunicable Diseases; Prospective Studies; Histiocytosis, Langerhans-Cell; Anti-Bacterial Agents; Acute Disease
PubMed: 34874762
DOI: 10.1177/01945998211064190 -
Orphanet Journal of Rare Diseases Mar 2022Even though a plethora of systemic therapies have been proposed for necrobiotic xanthogranuloma (NXG), there is no systematic review on this topic in literature. (Review)
Review
BACKGROUND
Even though a plethora of systemic therapies have been proposed for necrobiotic xanthogranuloma (NXG), there is no systematic review on this topic in literature.
OBJECTIVE
To review all existing literature on the systemic therapy of NXG in order to identify the most effective therapies.
METHODS
All reported papers in the literature were screened for systemic treatments of NXG. Papers without proper description of the therapies, papers describing topical therapy, and articles without assessment of effectiveness were excluded. Subsequently, we analyzed 79 papers and a total of 175 cases.
RESULTS
The most effective treatments for NXG are intravenous immunoglobulins (IVIG), corticosteroids, and combination therapies including corticosteroids.
CONCLUSIONS
Corticosteroids and IVIG should therefore be considered first-line treatments in patients with NXG.
Topics: Humans; Immunoglobulins, Intravenous; Necrobiotic Xanthogranuloma; Treatment Outcome
PubMed: 35331271
DOI: 10.1186/s13023-022-02291-z -
Liver Transplantation : Official... Aug 2021Langerhans cell histiocytosis (LCH) is the most common histiocytic disorder. Liver involvement is seen in 10.1% to 19.8% of patients with LCH and can lead to secondary...
Langerhans cell histiocytosis (LCH) is the most common histiocytic disorder. Liver involvement is seen in 10.1% to 19.8% of patients with LCH and can lead to secondary sclerosing cholangitis requiring liver transplantation (LT). We describe the characteristics and outcomes of patients undergoing LT for LCH. All patients undergoing a first LT for LCH in the United States were identified in the Scientific Registry of Transplant Recipients (SRTR) database (1987-2018). The Kaplan-Meier curve method and log-rank tests evaluated post-LT survival. A systematic literature review was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-analysis (PRISMA) statement. A total of 60 LCH LT recipients were identified in the SRTR, and 55 patients (91.7%) were children with median total bilirubin levels at LT of 5.8 mg/dL (interquartile range [IQR], 2.7-12.9). A total of 49 patients (81.7%) underwent deceased donor LT (DDLT). The 1-year, 3-year, and 5-year patient survival rates were 86.6%, 82.4%, and 82.4%, respectively. The systematic review yielded 26 articles reporting on 50 patients. Of the patients, 41 were children (82.0%), 90.0% had multisystem LCH, and most patients underwent DDLT (91.9%; n = 34/37). Pre-LT chemotherapy was administered in 74.0% and steroids in 71.7% (n = 33/46) of the patients, and a recurrence of LCH to the liver was reported in 8.0% of the patients. Of the 50 patients, 11 (22.0%) died during a median follow-up of 25.2 months (IQR, 9.0-51.6), and the 1-year patient survival rate was 79.4%. LT can be considered as a feasible life-saving option for the management of liver failure secondary to LCH in well-selected patients.
Topics: Child; Cholangitis, Sclerosing; Histiocytosis, Langerhans-Cell; Humans; Liver Failure; Liver Transplantation; Neoplasm Recurrence, Local; Retrospective Studies; United States
PubMed: 33484600
DOI: 10.1002/lt.25995 -
Pediatric Hematology and Oncology Feb 2023Almost half of the patients with Langerhans cell histiocytosis (LCH) are refractory to primary induction chemotherapy or undergo reactivation. The ideal treatment... (Meta-Analysis)
Meta-Analysis
Almost half of the patients with Langerhans cell histiocytosis (LCH) are refractory to primary induction chemotherapy or undergo reactivation. The ideal treatment modality for refractory/relapsed LCH is yet not evidenced. This review aimed to determine the efficacy and safety of vemurafenib (a BRAF pathway inhibitor) in LCH, particularly the refractory/relapsed cases. The literature search was conducted using PubMed, Embase, CENTRAL, and abstracts published in the SIOP meetings. Studies that described the outcome of patients of LCH being treated with vemurafenib, alone or in combination, were included. A total of 416 studies were screened, and after applying exclusion criteria, 22 studies ( = 107) were included in the final analysis. The first-line therapy was prednisolone plus vinblastine for most patients ( = 92, 86%), and vemurafenib was started upfront in 3 patients (3%). The median time to first clinical response with vemurafenib was one week. The median time to best response was 5.25 months. Out of 107 patients, 62 patients (58%) had ultimately no active disease (NAD) while 39 (36%) had active disease better (ADB), making the overall response rate (ORR) of 101/107, ie, 94.4% (CI 0.88; 0.98). The main adverse effects of vemurafenib were rash or photosensitivity (47%) and other cutaneous adverse events (15%). Vemurafenib is highly efficacious and safe in the treatment of refractory LCH; however, the timing of its commencement and duration of therapy is yet to be established. Larger prospective collaborative trials are needed to answer the appropriate treatment duration and effective maintenance therapy approach.
Topics: Humans; Vemurafenib; Prospective Studies; Proto-Oncogene Proteins B-raf; Histiocytosis, Langerhans-Cell; Protein Kinase Inhibitors
PubMed: 35616365
DOI: 10.1080/08880018.2022.2072986 -
Cureus Jun 2022Erdheim Chester disease (ECD) is a type of histiocytosis characterized by a variable clinical presentation. The treatment of ECD is complex and mainly unknown. We aim to... (Review)
Review
Erdheim Chester disease (ECD) is a type of histiocytosis characterized by a variable clinical presentation. The treatment of ECD is complex and mainly unknown. We aim to conduct a literature review of the treatment of ECD and consolidate the knowledge about the most recent and updated treatment for ECD. To conduct the systematic review, we used the preferred reporting items for systematic reviews and meta-analysis (PRISMA) protocol. To analyze the bias, we used the Cochrane collaboration risk-of-bias tool to assess the bias. We included observational studies and clinical trials on humans, which were written in English. Papers not fulfilling the objective of our study were excluded. Overall, the drug showed efficacy in the clinical trials, showing prolonged improvement and high rates of response rate. Overall, the drug was not well tolerated, and patients had a long list of side effects. Nevertheless, the drug seems to be a good option for second-line treatment for patients with ECD and BRAFV600 mutation.
PubMed: 35844342
DOI: 10.7759/cureus.25935 -
Substance Abuse Jul 2023The prevalence of marijuana use and its derivatives has surged over the past century, largely due to increasing legalization globally. Despite arguments advocating its... (Review)
Review
BACKGROUND
The prevalence of marijuana use and its derivatives has surged over the past century, largely due to increasing legalization globally. Despite arguments advocating its benefits, marijuana smoking exposes the lungs to harmful combustion byproducts, leading to various respiratory issues such as asthma, pneumonia, emphysema, and chronic obstructive pulmonary disease.
METHODS
We embarked on an extensive literature search, utilizing PubMed/Medline, Scopus, Web of Science, and Google Scholar databases, identifying 200 studies. After the elimination of duplicates, and meticulous review of abstracts and full texts, 55 studies were included in our analysis.
RESULTS
Current literature demonstrates that marijuana use negatively impacts lung function, triggering symptoms like chronic cough, sputum production, and wheezing, and diminishing FEV1/FVC ratio in spirometry tests. Moreover, prolonged or chronic marijuana use augments the risk of respiratory function impairment. While the carcinogenic effects of marijuana are still contested, a weak correlation between marijuana use and lung cancer has been observed in some studies. Additionally, instances of other pathologies linked to marijuana use have been reported, including the development of COPD, pulmonary bullae, spontaneous pneumothorax, pleuritic pain, chronic pulmonary aspergillosis, hemoptysis, and pulmonary Langerhans cell histiocytosis.
CONCLUSIONS
The evidence underscores that marijuana use is detrimental to respiratory health. In light of the escalating trend of marijuana use, particularly among the youth, it is imperative to advocate public health messages discouraging its consumption.
PubMed: 37728136
DOI: 10.1177/08897077231186228