Which lab tests and imaging studies are performed in the long-term monitoring of Langerhans cell histiocytosis (LCH)?

Updated: Sep 16, 2020
  • Author: Cameron K Tebbi, MD; Chief Editor: Vikramjit S Kanwar, MBBS, MBA, MRCP(UK), FAAP  more...
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Answer

Table 2 shows minimal frequencies of follow-up. Testing more frequent than that shown might be necessary.

Table 2. Laboratory and Imaging Studies in Patients With LCH (Open Table in a new window)

Type of Study

Study

Involvement

With Monostotic Lesion

Laboratory

Hemoglobin and/or hematocrit

Monthly

Every 6 mo

None

Leukocyte count and differential cell count

Monthly

Every 6 mo

None

Liver function tests*

Monthly

Every 6 mo

None

Coagulation studies

Monthly

Every 6 mo

None

Urine osmolality test after overnight water fast

Every 6 mo

Every 6 mo

None

Radiography

Chest, posteroanterior and lateral

Monthly

Every 6 mo

None

Skeletal survey

Every 6 mo

None

Once at 6 mo

* Measurements of alanine transaminase (ALT), aspartate transaminase (AST), and alkaline phosphatase.

Table 3 lists the indications for various laboratory evaluations, with the minimal frequencies of follow-up. Testing more frequent than that shown might be necessary.

Table 3. Indication for Laboratory Evaluations Based on Findings in LCH (Open Table in a new window)

Evaluation

Indication

Follow-Up Interval

Bone-marrow aspiration biopsy

Anemia, leukopenia, or thrombocytopenia

6 mo

Pulmonary function tests

Abnormal chest radiographic findings, tachypnea, intercostal retractions

6 mo

Lung biopsy after bronchoalveolar lavage, if available*

Abnormal findings on pretreatment chest radiography to rule out infection

None

Small-bowel series and biopsy

Unexplained chronic diarrhea, failure to thrive, malabsorption

None

Hepatic ERCP, angiography, or biopsy

High liver enzyme levels and hypoproteinemia not caused by protein-losing enteropathy to rule out active LCH vs liver cirrhosis

When all evidence of disease resolves but hepatic dysfunction persists

IV gadolinium-enhanced MRI of brain and hypothalamic-pituitary

Visual, neurologic, hormonal abnormalities

6 mo

Panoramic radiography of the teeth, mandible, and maxilla; consultation with an oral surgeon

Oral involvement

6 mo

PET scan

Surveillance

6 mo

Endocrine investigation

Growth failure, diabetes insipidus, hypothalamic syndromes, galactorrhea, precocious or delayed puberty; hypothalamic and/or pituitary abnormality on CT or MRI

None

Consultation with an audiologist and an otolaryngologist

Aural discharge, impaired hearing

6 mo

Note.—ERCP = endoscopic retrograde cholangiopancreatography; IV = intravenous.

* Diagnostic findings on bronchoalveolar lavage obviate lung biopsy.

Although high serum levels of interleukin-17 (ILITA), which is a T-cell–specific cytokine involved in chronic inflammation processes, have been found in LCH with correlation to the activity of the disease. However, this is not expressed by CD207 t-cells in LCH lesions. [228, 229]


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