Eosinophilic Granuloma (Histiocytosis X) Clinical Presentation

Updated: Mar 29, 2018
  • Author: Eleanor M Summerhill, MD, FACP, FCCP; Chief Editor: Zab Mosenifar, MD, FACP, FCCP  more...
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Presentation

History

Presentations of pulmonary Langerhans cell histiocytosis X (PLCH) are variable. [9] Approximately 25% of patients are asymptomatic, and their disease is diagnosed after an evaluation of incidental findings on chest radiographs. Others present with respiratory or constitutional symptoms. In order of decreasing frequency, common presenting symptoms are as follows:

  • Nonproductive cough (56-70%)

  • Dyspnea (40%)

  • Fatigue (30%)

  • Weight loss (20-30%)

  • Chest pain (21%)

  • Spontaneous pneumothorax, which may be recurrent, is a classic presentation found in 10-20% of patients.

  • Fever (15%)

  • Cystic bone lesions (4-20%): These may be painful and may predispose the patient to pathologic fracture.

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Physical Examination

Patients with pulmonary Langerhans cell histiocytosis X (PLCH) present with nonspecific physical findings. Neither inspiratory rales (crackles) nor clubbing is common. Cor pulmonale may develop; therefore, the following related findings may be present:

  • Loud second heart sound with accentuated pulmonic component

  • Tricuspid regurgitation murmur

  • Right ventricular lift

  • Peripheral edema

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Complications

Spontaneous pneumothorax is a common complication (10-20%) in pulmonary Langerhans cell histiocytosis X (PLCH).

PLCH is associated with an increased risk of malignancy, including Hodgkin and non-Hodgkin lymphoma, myeloproliferative disorders, and bronchogenic carcinoma. [10]

Pathologic fracture may occur at the site of bone lesions.

Diabetes insipidus occurs in 10-15% of patients and indicates disease in the central nervous system.

Pulmonary arterial hypertension and, rarely, cor pulmonale, may develop as a result of hypoxemia and/or vascular occlusion due to PLCH lesions.

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