Langerhan Cell Histiocytosis

LANGERHANS\’ CELL HISTIOCYTOSIS
Definition: "An accumulation or proliferation of a clonal population of cells bering the phenotype of a Landerhan\’s cell that has been arrested in early stage of activation and is functionally deficient."

History and background

  • Han-Sschuller Christian Disease or Triad
  • > 2 years of age
  • Exophthalmos
  • Lesions in the bones of the skull
  • Diabetes insipidus
  • the full triad is rarely seen and the prognosis is relatively good
  • Letterer-Siwe disease
  • Under age of 2 years old
  • Hepato- Splenomegaly
  • Lymphadenopathy
  • Anemia
  • Hemorrhagic diathesis (disposition)
  • Prognosis was quit poor
  • Solitary eosinophilic granuloma
  • > 2 years of age
  • Solitary, usually bony site of involvement
  • Histiocytosis X
  • 1953 Lichtenstein argued that eosinophilic granulmoa and Han-Schutler Christian disease were related manifestations of single entity which he named Histiocytosis X
  • Now we know that Histiocytosis X is is made of Langerhans\’ cells and thus the name has been changed to Langerhans\’ cell histiocytosis.

Presentation/Epidemiology
0.5 to 2 cases per 100K per children per year
Male predominence
Diabetes Insipidus most common complication of CNS involvement
Pulmonary involvement primarily affects young adults, smoking cessation is effective therapy

  • Organ Involvement
  • Radiographic Presentation
  • Langerhans\’ cell histiocytosis. Radiograph of the characteristic lytic bone lesion, as seen in the skull of a patient
  • Skin Involvement
  • Pathologic Diagnosis
  • Birbeck granules on electron microscopy
  • Figure 26.1 Electron micrograph of a Langerhans\’ cell showing Birbeck granules. This cell was obtained by needle aspiration from a patient with lymph node involvement of Langerhans\’ cell histiocytosis. (Principles & Practice of Pediatric Oncology)
  • CD1a expression in lesional cells (on left)
  • Birbeck granules (pointed on the right)
  • ATPase positive, S-100 positive

Treatment
Observation
Steroid injection

  • Surgery
  • Biopsy, curettage often enough to induce regression
  • Wide local excision is recommended for expendable bones
  • XRT indications:

Local relapse after surgery when bone is sole site of recurrence
When curettage is too risky
When surgery would result in poor cosmesis
Potential compromise of critical structures
Pain relief
Possibly for DI if symptoms have recent onset (<1 week)

  • Diabetes insipidus
  • Minehan et al. reported 60% (3/5) response for > 15 Gy as compared to 30% (7/23) treated with < 15 Gy. None of the 17 patients in the observation group improved.
  • 15 Gy or more should be given
  • Cover hypothalamic-pituitary axis
  • 5/6 complete responders were irradiated within 14 days of the diagnosis of diabetes insipidus
  • Bones and soft tissue
  • Dose controversial: 5-35 Gy for bone or soft tissue,
  • For bone/soft tissue lesions cover lesion with a small margin

Sequelae (of disease with and without XRT)
DI, short stature, hypothyroidism, deafness, orthopedic problems, tooth loss, hepatitis, neurologic problems, intellectual problems, secondary leukemia.

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