Langerhans Cell Histiocytosis (LCH)
Description
● Introduction :
➣ Definition: Langerhans cells (antigen-presenting dendritic cells) proliferate uncontrollably to create LCH (previously histiocytosis X), an unusual haematological illness.
➣ Incidence: 2–5 cases per million annually (2009 estimate).
➣ Diagnosis: Clinically categorised using Greenberg's approach; based on histopathology
➣ Oral involvement: Frequently the initial sign; symptoms are vague and sometimes misdiagnosed.
➣ The goal of the case is to highlight the dentist's role in diagnosis and treatment while discussing the clinical, radiological, and histological aspects of LCH.
● Discussion
➣ Disease Nature
• The original cell type is mononuclear dendritic antigen-presenting cells, or Langerhans cells.
• Pathogenesis: Currently understood to be a monoclonal neoplastic growth, it was previously thought to be a reactive condition.
• Age group: Males predominate (2:1), with a peak age of 2-4 years and a majority of 1-15 years.
• About 1 in 200,000 in less than ten years.
● Case Report
➣ Patient: 3-year-old boy with painful gingival growth in his upper and lower jaws (left and right) for a month.
➣ Pain: sudden onset, intermittent, non-radiating, aggravated by chewing, spontaneously relieved.
➣ Past history: previously treated with antibiotics and analgesics; no improvement.
➣ Medical & family history: non-contributory.
➣Clinical Findings
→Extraoral:
•Diffuse, hard, and sensitive swelling on the left lower jaw (4 × 2 cm).
• The left submandibular lymph nodes are solid, palpable, and non-tender.
→ Intraoral:
• Necrotic slough over 55, 65, 75, and 85; diffuse erythematous, ulcerated gingival swellings.
• Affected teeth have Grade II mobility and little bleeding patches.
• Radiographs (IOPA): 55, 74, and 75 roots have a little radiolucent lesion.
➣Localized chronic periodontitis is the provisional first diagnosis.
➣Differential diagnoses:
•Eosinophilic granuloma;
• Papillon-Lefèvre syndrome
•Cyclic neutropenia
●Investigations
➣ Blood tests:
• Microcytic hypochromic anaemia: haemoglobin: 10.5 g%.
• Normal liver, kidney, urine, and CRP levels.
• HBsAg and HIV are negative.
➣Radiology:
•Orthopantomogram: The left mandible has several regions of bone loss.
• CT (axial + coronal): The left mandible, occiput, right maxilla, and right temporal bone have many soft-tissue lesions with punched-out bone damage.
•3D CT: Several osteolytic lesions in the head and jaw.
➣ Histopathology:
• Langerhans cells: Convoluted/retiform nuclei, abundant eosinophilic cytoplasm, and some binucleated forms.
• Background: Eosinophils, plasma cells, and lymphocytes.
★ The final diagnosis is multiple-bone LCH.
Protocol
● Management
Age, location, number of lesions, and systemic involvement all influence management.
➣ Localised lesions:
• Intralesional corticosteroids (prednisolone 20–30 mg/day × 2–4 weeks, then taper); surgical curettage or excision; low-dose radiation (rare; risk of cancer)
➣ Multisystemic illness:
• Systemic chemotherapy:
• Corticosteroids:
■etoposide, vinblastine, and vincristine
■Methotrexate, 6-mercaptopurine, 2-chlorodeoxyadenosine, and cytarabine
■ The combination of vincristine and prednisone lowers the chance of recurrence.
■ Cyclosporine with thalidomide (in some regimens).
1. Prompt Medical Care
• Due to multisystem bone involvement, local lesions were not surgically addressed at this stage; instead, the kid was transferred to a cancer treatment centre for additional systemic treatments.
2. Overview of Therapeutic Intervention
• For localised lesions, surgical excision or curettage is recommended.
• Less accessible or recurrent lesions: Care should be used while using low-dose radiation therapy.
•Intralesional corticosteroid therapy: Prednisolone (20–30 mg/day) used for two to four weeks with tapering is an effective way to reduce inflammation and swelling.
•Systemic or multifocal LCH: Usually treated with a combination of chemotherapeutic treatments, such as:
Prednisone or prednisolone corticosteroids; vinblastine, etoposide, or cytarabine
• 6-mercaptopurine or methotrexate for recurring or resistant instances.The
3. Supportive and Nursing Interventions
•Pain management: Monitoring the pain response and using regular analgesics for acute discomfort.
•Oral hygiene care: To lower the risk of bleeding and infection, gently clean your mouth.
• Nutritional support: Soft diet guidelines to keep oral lesions from getting worse.
• Psychosocial care: Parental counselling because of anxiety related to a chronic illness.
• Monitoring: Consistent monitoring to track lesion healing and identify systemic dissemination to organs or bones.
4. Coordination Across Disciplines
• Coordinated care was ensured by cooperation between paediatricians, oncologists, maxillofacial surgeons, and dental specialists.
•Oncologists oversaw systemic therapy, whereas dental surgeons handled diagnosis and treatment of mouth symptoms.
Notes
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