Langerhans Cell Histiocytosis

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Disease Entity

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Disease

Langerhans Cell Histiocytosis (LCH)

Risk Factors

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General Pathology

Langerhans Cell Histiocytosis (LCH), formerly known as “histiocytosis X,” historically includes three subgroups: eosinophilic granuloma, Hand-Schuller-Christian disease, and Letterer-Siwe disease, representing a spectrum from unifocal to multifocal/multisystem involvement.[1]

Pathophysiology

The lesions of LCH may involve the bones, skin, hypothalamus-pituitary region, as well as other organs. The characteristic granuloma-like lesions of Langerhans Cell Histiocytosis consists of langerhans cells, macrophages, eosinophils, T lymphocytes, and plasma cells. While interactions between these cells are believed to contribute to the final pathology, the pathogenetic element is thought to be the clonal expansion of immature Langerhans cells.[2]

Etiology

Langerhans cell histiocytosis remains an enigmatic disease and the dispute over its neoplastic versus reactive nature is unsettled. The current understanding of histiocyte development is based on in vitro studies and suggests an immune/inflammatory reaction leads susceptible langerhans cells to undergo a clonal expansion resulting in LCH.[3]

Incidence 

LCH is predominantly a disease of childhood with a peak incidence between 1 and 4 years, but it can be seen at any age.  The estimated annual incidence is 2-5 in one million children.[4]  In a recent retrospective chart review of 24 consecutive patients with LCH treated at a tertiary referral center, the orbit was involved in 9 (37.5%) of patients. The most common site of involvement was the frontal bone (n = 6) followed by zygomatic (n = 3), sphenoid (n = 3), and maxillary (n = 2).5 LCH represents approximately 1% to 3% of pediatric orbital tumors.[5]

Diagnosis

The histological diagnosis of LCH lesions is based on staining for S-100 protein and CD1a antigen or finding Birbeck granules (shaped like tennis racquets) on electron microscopy.[6][7]

History

Epidermal langerhans cells were first described by the German physician Paul Langerhans in 1868. Because the branched cells demonstrated an affinity for gold chloride (a stain for nervous tissue) the cells were thought to be of neural origin. The hematopoietic origin and antigen presenting function of the dendritic cells would remain unknown for more than a century.[8]

Physical examination

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Signs

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Symptoms

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Clinical diagnosis

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Diagnostic procedures

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Laboratory test

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Differential diagnosis

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Management and Treatment

All patients with unifocal orbital disease require a biopsy to establish and confirm the diagnosis and rule out other malignant disease. An evaluation by a pediatric oncologist to assess for other sites of involvement and follow-up to monitor for recurrence or progression post treatment is needed. Single-system disease confined to a single site (e.g., small, anterior lesions of the orbit without intracranial extension) usually only requires local therapy (e.g., curettage, steroid injection, or radiation therapy) or observation. On the other hand, multi-focal/system disease usually requires systemic therapy with prednisone with or without chemotherapeutic agents (vinblastine and/or etoposide) depending on the extent of the disease.[9]

General treatment

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Medical therapy

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Medical follow up

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Surgery

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Surgical follow up

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Complications

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Prognosis

Children less than one year tend to have more aggressive disease with a worse prognosis.[10]

Additional Resources

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References


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  1. Savasan, S. Int J Dermatol 2006;45:182– 188.
  2. Savasan, S. Int J Dermatol 2006;45:182– 188.
  3. Savasan S. An enigmatic disease: childhood Langerhans cell histiocytosis in 2005. Int J Dermatol 2006;45:182-8.
  4. Savasan, S. Int J Dermatol 2006;45:182– 188.
  5. Vosoghi, H. et al. Ophthal Plast Reconstr Surg 2009;25:430–433.
  6. Savasan, S. Int J Dermatol 2006;45:182–188.
  7. Harris, G. J. Am J Ophthalmol 2006;141:374–378.
  8. Namazi, M. R. Arch Dermatol 2008;144:1109.
  9. Vosoghi, H. et al. Ophthal Plast Reconstr Surg 2009;25:430–433.
  10. Vosoghi, H. et al. Ophthal Plast Reconstr Surg 2009;25:430–433.