scholarly journals Rare Langerhans cell histiocytosis in children: a case report

2019 ◽  
Vol 7 (4) ◽  
pp. 1367
Author(s):  
Lina Fadil ◽  
Emtenan Almajid
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Plasma Cells ◽  
Oral Lesion ◽  
Langerhans Cell ◽  
Tissue Destruction ◽  
Total Period ◽  
Oral Manifestation ◽  
Initial Symptoms ◽  
Good General Condition

Langerhans cell histiocytosis (LCH) is a rare disease, formally known as histiocytosis X that is characterized by abnormal proliferation of histiocytes derived from bone marrow (Langerhans cells), joined with leucocytes, eosinophils, neutrophils, lymphocytes, plasma cells and giant multi-nucleated cells causing tissue destruction. One of the first signs of LCH is oral manifestation, in some cases, the oral cavity may be the only affected area. With the chance of oral lesion incidence in LCH being 77%.Initial symptoms are generally nonspecific, which can easily cause misdiagnoses.The purpose of reporting this case is to discuss the features of LCH clinically and radiographically and in the role of the dentist when diagnosing such lesions for a proper management.An 11-year-old boy reported a complaint of swelling in the left side of the lower jaw that is asymptomatic and had been gradually increasing in size for the past 6 months without any improvements. After preforming a biopsy and diagnosing the lesion as LCH, the patient was then treated with a dose of vinblastine (6 mg/m2 intravenous bolus) for 24 weeks as a total period. Two years follow up; the patient showed no sign of recurrence and is in good general condition. In conclusion, reporting this case serves as documentation of the proper route of clinical assessment and diagnosis of LCH with the best possible treatment as guidance.

Notch is active in Langerhans cell histiocytosis and confers pathognomonic features on dendritic cells

Blood ◽  
2012 ◽  
Vol 120 (26) ◽  
pp. 5199-5208 ◽  
Author(s):  
Caroline Hutter ◽  
Max Kauer ◽  
Ingrid Simonitsch-Klupp ◽  
Gunhild Jug ◽  
Raphaela Schwentner ◽  
...  
Keyword(s):  
Dendritic Cells ◽  
Notch Signaling ◽  
Langerhans Cell Histiocytosis ◽  
Langerhans Cell ◽  
Tissue Destruction ◽  
Notch Ligand ◽  
Functional Aspects ◽  
Notch Activation ◽  
Epidermal Langerhans Cells

Abstract Langerhans cell histiocytosis (LCH) is an enigmatic disease defined by the accumulation of Langerhans cell-like dendritic cells (DCs). In the present study, we demonstrate that LCH cells exhibit a unique transcription profile that separates them not only from plasmacytoid and myeloid DCs, but also from epidermal Langerhans cells, indicating a distinct DC entity. Molecular analysis revealed that isolated and tissue-bound LCH cells selectively express the Notch ligand Jagged 2 (JAG2) and are the only DCs that express both Notch ligand and its receptor. We further show that JAG2 signaling induces key LCH-cell markers in monocyte-derived DCs, suggesting a functional role of Notch signaling in LCH ontogenesis. JAG2 also induced matrix-metalloproteinases 1 and 12, which are highly expressed in LCH and may account for tissue destruction in LCH lesions. This induction was selective for DCs and was not recapitulated in monocytes. The results of the present study suggest that JAG2-mediated Notch activation confers phenotypic and functional aspects of LCH to DCs; therefore, interference with Notch signaling may be an attractive strategy to combat this disease.

scholarly journals Adult-Onset Langerhans Cell Histiocytosis And The Role of Vinblastine In The Treatment -Ankara Oncology Hospital Experience

2020 ◽  
Vol 53 (3) ◽  
pp. 492-499
Author(s):  
Ayşegül Tetik ◽  
Bahar Uncu Ulu ◽  
Mehmet Bakırtaş ◽  
Tuğçe Nur Yiğenoğlu ◽  
Jale Yıldız ◽  
...  
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Langerhans Cell ◽  
Adult Onset ◽  
Hospital Experience

scholarly journals Langerhans cell histiocytosis: A case report with oral manifestations and the role of pediatric dentists in the diagnosis

2020 ◽  
Vol 8 (3) ◽  
pp. 545-549
Author(s):  
Eman Hussein Hammouri ◽  
Hala Antoun Sweidan ◽  
Omar AShokaibi ◽  
Leen Al Omari
Keyword(s):  
Case Report ◽  
Langerhans Cell Histiocytosis ◽  
Langerhans Cell ◽  
Oral Manifestations ◽  
Pediatric Dentists

scholarly journals Premature Eruption Of Tooth: An Unusual Oral Manifestation In A Case Of Multisystem Langerhans Cell Histiocytosis

2018 ◽  
Vol 3 (3) ◽  
pp. S16-S17
Author(s):  
M. Madhuchandan ◽  
J. MuruguSarasu ◽  
Dewan Pooja ◽  
Gomber Sunil ◽  
Mahajan Supriya
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Langerhans Cell ◽  
Oral Manifestation

scholarly journals Primary oral manifestation of Langerhans cell histiocytosis refractory to conventional therapy but susceptible to BRAF-specific treatment: a case report and review of the literature

2019 ◽  
Vol 11 ◽  
pp. 175883591987801 ◽  
Author(s):  
Norbert Neckel ◽  
Andrej Lissat ◽  
Arendt von Stackelberg ◽  
Nadine Thieme ◽  
Mohemed-Salim Doueiri ◽  
...  
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Young Patients ◽  
Specific Treatment ◽  
Langerhans Cell ◽  
Adequate Treatment ◽  
Oral Manifestation ◽  
Stomatognathic System ◽  
Proper Diagnosis ◽  
Specific Agent

Langerhans cell histiocytosis (LCH) is a diagnostic and therapeutic challenge. We report on a rare case of its primary oral manifestation that was treated successfully with the BRAF-specific agent, vemurafenib, after insufficient standard LCH treatment. This case underlines the importance of proper diagnosis and the evaluation of targeted therapy as a valuable tool in LCH treatment. Furthermore, the close collaboration of surgeons, oncologists, and dentists is mandatory to ensure adequate treatment, restore the stomatognathic system in debilitating post-treatment situations, improve quality of life, and ensure effective disease control in infants and young patients.

scholarly journals Revealing a rare inflammatory oral manifestation in a 6-year-old child

2019 ◽  
Vol 12 (7) ◽  
pp. e229483
Author(s):  
Samapika Routray ◽  
Amit Kumar Adhya ◽  
Joseph John ◽  
Punit Dikhit
Keyword(s):  
Bone Loss ◽  
Buccal Mucosa ◽  
Langerhans Cell Histiocytosis ◽  
Langerhans Cell ◽  
Oral Manifestation ◽  
Atypical Cells ◽  
History Of ◽  
Permanent Molars ◽  
Superficial Dermis ◽  
Distal Aspect

A 6-year-old child with an episodic history of ulcerations over buccal mucosa was found to have severe inflammation on the palatal aspect of permanent first molars with grade 2 mobility bilaterally. Radiographical features were suggestive of bone loss around permanent molars extending to the distal aspect of the deciduous first molars. The clinical and radiographical findings were indicative of periodontal degeneration without any apparent cause visible intraorally. Further biopsy was done from the rashes present on the malar prominences, which showed nodular aggregates of atypical cells in superficial dermis. These large histiocytic cells with vesicular nuclei and nuclear grooves were immunopositive for CD1a and S100, concluding the diagnosis of Langerhans cell histiocytosis. For treatment, patient was referred to Department of Haemato-oncology and chemotherapy was suggested as per protocol.

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