The medical recommendations for adult LCH have been published in Orphanet Journal of Rare Diseases.

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Further investigations for specific clinical scenarios

Author(s): Euro Histio Net Work Group for LCH Guidelines for adults (see introduction page), Created: 2012/07/26, last update: 2014/02/09

Further investigations may be indicated based on the patient's symptoms and the findings of the basic diagnostic tests - see table 3 and table 4 (Grade D2 *).

Tab. 4 Specific clinical scenarios: recommended additional testing

 
  

History of polyuria or polydipsia

Grade *

  • Urine and plasma osmolality

D2

  • Water deprivation test

D2

  • MRI of the head

D2

Suspected other endocrine abnormality

Grade *

  • Endocrine assessment (including dynamic tests of the anterior pituitary, MRI of the head)

D2

Bi- or pancytopenia, or persistent unexplained single cytopenia

Grade *

  • Any other cause of cytopenia has to be ruled out according to standard medical practice.

D2

  • Bone marrow aspirate and trephine biopsy to exclude causes other than LCH.

D2

  • In case of morphological signs of hemophagocytosis additional tests like serum-ferritin should be performed (criteria of HLH)

D2

Liver or spleen abnormalities

Grade *

  • In case of any unclear sonographically pathology CT, PET-CT, MRI or Scans should be added (the choice is depending on the sonomorphology – discuss with your radiologist)

D2

  • Visuable lesions of the liver should be biopsied if possible

D2

  • Other causes of splenomegaly has to be ruled out before it may be assigned to LCH

D2

  • ERCP (Endoscopic Retrograde Cholangiopancreatography) or MRCP (Magnetic Resonance Cholangiopancreatography) should be performed in case of elevated serum cholestasis markers or sonomorphologically dilatated bile ducts. Primary biliary cirrhosis and primary sclerosing cholangitis have to be ruled out.

D2

Unexplained chronic diarrhea, weight loss, evidence of malabsorption or hematochezia

Grade *

  • GI-Exploration (Endoscopy with biopsies, capsule endoscopy)

D2

Enlarged Lymph Nodes (LN)

Grade *

  • If found by screening ultrasound or physical examination the best suitable LN should be exstirpated. A LN needle biopsy should be avoided.

D2

  • CT scans or a PET-CT should be performed additionally

D2

Lung involvement

Grade *

In case of abnormal Chest X Ray or symptoms/signs suggestive for lung involvement or suspicion of a pulmonary infection:

 
  • Lung high resolution computed tomography (HR-CT)

D2

  • Lung function tests (spirometry, diffusing capacity, oxygen desaturation during exercise (6MWT), blood gases)

D2

  • Bronchoalveolar lavage (BAL): > 5% CD1a + cells in BAL fluid may be diagnostic of LCH

D2

  • Lung biopsy (if BAL is not diagnostic), ideally Video-assisted thoracoscopic surgery (VATS)

D2

Osseous disease

Grade *

  • CT +/- MRI should be performed in case of craniofacial or vertebral lesions or signs of additional soft tissue involvement

D2

  • Biopsies should be taken from the most suitable region in case of multifocal bone disease

D2

Skin, oral and genital mucosa lesions

Grade *

  • Biopsies should be taken

D2

Aural discharge or suspected hearing impairment / mastoid involvement

Grade *

  • Formal hearing assessment

D2

  • MRI of head

D2

* Grades of recommendation see here.