Updated: 5/2/2020

Langerhans Cell Histiocytoses

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Snapshot
  • A 12-year-old boy presents to the emergency room for pain in in his right forearm with minimal trauma while playing tag. Radiography shows a fracture of the radius. A skeletal survey also shows 2 other lytic bone lesions on the cranium. He is referred to the pediatric oncologist for further workup and possible biopsy for a Langerhans cell histiocytosis.
Introduction
  • Overview
    • Langerhans cell histiocytosis (LCH) describes a group of conditions caused by proliferation of dendritic (Langerhans) cells
      • traditionally classified by 4 subtypes, but more recent classification is based on extent of dissemination
 
Langerhans Cell Histiocytosis (LCH)

Letterer-Siwe Hand-Schuller-Christian Hashimoto-Pritzker (Congenital Self-Healing Reticulohistiocytosis)
Eosinophilic Granuloma
Demographics
  • < 2 years of age
  • 2-6 years of age
  • Neonatal period
  • 7-12 years of age
  • Pulmonary involvement seen in ages 30-40 years
Involvement
  • Skin, bone, and viscera
    • pulmonary infiltrates, lymph nodules, liver, and spleen
  • Skin, bone, and viscera
    • liver, spleen, and lymph nodes
  • Skin-limited
  • Primarily bone > skin
  • Pulmonary involvemen
Prognosis
  • Poor prognosis with disseminated disease
  • Chronic and progressive
  • Rapid spontaneous resolution is likely
  • Good prognosis
 
  • Epidemiology
    • incidence
      • rare
    • demographics
      • males > females
    • risk factor     
      • smoking
        • pulmonary involvement
  • Pathogenesis
    • mechanism
      • proliferation of Langerhans cells, which are immature and are unable to stimulate T-cells via antigen presentation
        • thought to be reactive or neoplastic
      • BRAF V600E mutation can be found in LCH
  • Prognosis
    • worse with BRAF V600E mutation
    • worse if there’s failure to respond to 6 weeks of treatment
Presentation
 
Clinical Presentation of LCH 

Letterer-Siwe Hand-Schuller-Christian Hashimoto-Pritzker (Congenital Self-Healing Reticulohistiocytosis)
Eosinophilic Granuloma
Symptoms
  • Acutely disseminated lesions affecting the skin, bone, and internal organs
  • Fever
  • Bone pain
  • Triad of diabetes insipidus, osteolytic bone lesions (cranium), and exophthalmos
  • Skin lesions that crust and resolves spontaneously
  • Solitary indolent bone lesions (cranium most common) with spontaneous bone fractures
Physical exam
  • Pink papules, pustules, vesicles, petechiae, purpura, and erosions on the scalp, flexural surfaces, and trunk
  • Secondary impetiginization common
  • Skin findings similar to Letterer-Siwe
  • Multipe red to brown papulonodules, some with erosions
  • Tenderness at site of bone lesions
 
  • Other symptoms
    • recurrent ear infections
    • dizziness
    • headache
    • limping (due to leg pain)
    • failure to thrive
    • developmental delay
Imaging
  • Bone radiograph
    • views
      • cranium or other skeletal survey
    • findings
      • punched out lytic lesions
  • Chest radiograph
    • findings
      • may show pulmonary nodules or infiltrate
Studies
  • Serum studies
    • liver function test
      • to evaluate for liver involvement
    • baseline complete blood count
  • Urine studies
    • to evaluate for diabetes insipidus
  • Pulmonary function test
    • o evaluate for pulmonary involvement
  • Histology
    • S100+, CD1a+, and Langerin+
    • proliferation of histiocytes with mixed inflammatory infiltrate
  • Electron microscopy
    • Birbeck granules or “tennis rackets"
Differential
  • Multiple myeloma
    • key distinguishing factors
      • may also have “punched out” lytic bone lesions
      • typically affects older adults, and protein electrophoresis of the serum (SPEP) will reveal monoclonal gammopathy
Treatment
  • Medical
    • corticosteroids
      • indications
        • to slow autoimmune reaction
    • chemotherapy
      • indications
        • for severe cases in children
      • drugs
        • cyclophosphamide, etoposide, methotrexate, and vinblastine
    • radiation therapy
      • indications
        • bone lesions
    • vasopressin
      • indications
        • diabetes insipidus
    • bisphosphonates
      • indication
        • prevent bone destruction from bone lesion
  • Surgical
    • curettage or excision
      • indications
        • bone lesions
Complications
  • Bone fractures
  • Spontaneous pneumothorax
  • Infections
  • Hearing impairment

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