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Updated: Mar 30 2023

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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