Pulmonology Book

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Langerhans Cell Granulomatosis

Aka: Langerhans Cell Granulomatosis, 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 and common form of Langerhans-cell histiocytosis which involves localized nodular lesions predominantly of the bones but also of the gastric mucosa, small intestine, lungs, or skin, with infiltration by EOSINOPHILS.
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.
Concepts Pathologic Function (T046)
MSH D004803
ICD10 K13.4, C96.6
SnomedCT 77239009, 128810002, 129000002
English Eosinophilic Granuloma, Eosinophilic Granulomas, Granuloma, Eosinophilic, Granulomas, Eosinophilic, EOSINOPHILIC GRANULOMA, Eosinophilic xanthom granuloma, Unifocal Langerhans Cell Histiocytosis, eosinophilic granuloma (diagnosis), eosinophilic granuloma, Eosinophilic granuloma (morphologic abnormality), unifocal eosinophilic granuloma (diagnosis), unifocal eosinophilic granuloma, Eosinophilic Granuloma [Disease/Finding], granuloma eosinophilic, Eosinophilic granuloma, Eosinophilic xanthomatous granuloma, Eosinophilic granuloma (clinical), eosinophilic; granuloma, granuloma; eosinophilic, Eosinophilic granuloma -RETIRED-, Eosinophilic Xanthomatous Granuloma
Portuguese GRANULOMA EOSINOFILO, Granuloma eosinófilo, Granuloma Eosinófilo
Spanish GRANULOMA EOSINOFILO, Granuloma eosinofílico, granuloma eosinofílico (concepto no activo), granuloma eosinofílico xantomatoso, granuloma eosinofílico, granuloma eosinófilo, Granuloma Eosinófilo, Granuloma Eosinofilo
German EOSINOPHILES GRANULOM, eosinophiles Granulom, Eosinophiles Granulom, Granulom, eosinophiles
Swedish Eosinofilt granulom
Czech eozinofilní granulom, Eozinofilní granulom
Finnish Eosinofiilinen granulooma
Russian EOZINOFIL'NAIA GRANULEMA, GRANULEMA EOZINOFIL'NAIA, RETIKULEMA DOBROKACHESTVENNAIA, ГРАНУЛЕМА ЭОЗИНОФИЛЬНАЯ, РЕТИКУЛЕМА ДОБРОКАЧЕСТВЕННАЯ, ЭОЗИНОФИЛЬНАЯ ГРАНУЛЕМА
French GRANULOME EOSINOPHILIQUE, Granulome éosinophile
Croatian EOZINOFILNI GRANULOM
Dutch eosinofiel granuloom, eosinofiel; granuloom, granuloom; eosinofiel, Eosinofiel granuloom, Granuloma eosinophilicum
Japanese ｺｳｻﾝｷｭｳｾｲﾆｸｶﾞｼｭ, ｺｳｻﾝｷｭｳｾｲﾆｸｹﾞｼｭ, 肉芽腫-好酸球性, エオシン好性肉芽腫, 好酸球性肉芽腫, 好酸球肉芽腫
Polish Ziarniniak kwasochłonny
Hungarian Eosinophil granuloma
Italian Granuloma eosinofilo
Sources
Derived from the NIH UMLS (Unified Medical Language System)


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