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Langerhans Cell GranulomatosisAka: Eosinophilic Granuloma, Pulmonary Histiocytosis X

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  1. Pathophysiology
    1. Dendritic cell disorder
  2. Related Conditions
    1. Letterer-Siwe
    2. Hand-Schuller-Christian Syndrome
    3. Histiocytosis X with Bone involvement
  3. Epidemiology
    1. Ages 20-40 years old
    2. Prior Tobacco use (90%)
  4. Signs
    1. See Interstitial Lung Disease
  5. Complications
    1. Pneumothorax (10-30%)
    2. Diabetes Insipidus
    3. Lytic bone lesions (5-10%)
  6. Management
    1. None available
  7. Coarse
    1. Unpredictable prognosis
    2. Mortality 2-6%

Eosinophilic Granuloma (C0014461)

Definition (MSH)The most benign clinical form of Langerhans-cell histiocytosis (HISTIOCYTOSIS, LANGERHANS-CELL), which involves localized nodular lesions of the gastric mucosa, small intestine, bones, lungs, or skin, with infiltration by eosinophils. The proliferating cell that appears to be responsible for the clinical manifestations is the Langerhans cell.
Definition (CSP)most benign clinical form of Langerhans-cell histiocytosis, which involves localized nodular lesions of the gastric mucosa, small intestine, bones, lungs, or skin, with infiltration by eosinophils; the proliferating cell that appears to be responsible for the clinical manifestations is the Langerhans cell.
Definition (NCI)A clinical variant of Langerhans cell histiocytosis characterised by unifocal involvement of a bone (most often), skin, or lung. Patients are usually older children or adults usually presenting with a lytic bone lesion. The etiology is unknown. Morphologically, eosinophilic granuloma is characterised by the presence of Langerhans cells in a characteristic milieu which includes histiocytes, eosinophiles neutrophiles, and small, mature lymphocytes.
ConceptsPathologic Function (T046)
MSHD004803
EnglishEosinophilic Granuloma, Eosinophilic granuloma -RETIRED-, Eosinophilic Granulomas, Eosinophilic xanthomatous granuloma, Unifocal Langerhans Cell Histiocytosis
Spanishgranuloma eosinofilico, granuloma eosinofilico xantomatoso, granuloma eosinofilo
Parent ConceptsHistiocytosis, Langerhans-Cell (C0019621), Bone Diseases (C0005940), Eosinophilia (C0014457), Granuloma (C0018188), Reason not stated concept (C1276325)
SourcesCOSTAR, CSP, CST, DXP, LCH, MSH, NCI, NDFRT, SCTSPA, SNOMEDCT
Derived from the NIH UMLS (Unified Medical Language System)



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