The medical guidelines for childhood LCH have been published in Pediatric Blood & Cancer  (PBC), Wiley Online Library.

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Recommended Evaluation in Every Case

Author(s): Euro Histio Net Work Group for LCH Guidelines (see introduction page), Created: 2011/03/09, last update: 2014/02/09

Complete History; Complete Physical Examination; Laboratory and Radiographic Evaluation

Complete History

After having made an LCH diagnosis, it is important to collect further baseline information in order to decide on the therapeutic approach. A complete history should include special reference to the nature and duration of symptoms. Specific symptoms to be actively looked for are: pain, swelling, skin rashes, otorrhea, irritability, fever, loss of appetite, diarrhea, weight loss or poor weight gain, growth failure, polydipsia, polyuria, changes in activity level, dyspnea, smoke exposure, and behavioral and neurological changes.

Complete Physical Examination

Complete clinical examination considering the symptoms described in the section Complete History should be performed and the symptoms documented.

This history and examination should be performed at each follow-up visit in order to assess response to therapy, investigate for possible disease progression or reactivation, as well as to detect sequelae.

Laboratory and Radiography

Currently there is no specific biological marker of disease activity, however there is general agreement that biochemical and imaging evaluation at diagnosis and in case of disease reactivation should in any case include the examinations shown in the following Table 3.

Tab. 3. Laboratory and radiographic evaluation

Full blood count

  • Hemoglobin
  • White blood cell and differential count
  • Platelet count

Blood chemistry

  • Total protein
  • Albumin
  • Bilirubin
  • ALT (SGPT)
  • AST (SGOT)
  • Alkaline phosphatase
  • γGT
  • Creatinine
  • Electrolytes

Erythrocyte Sedimentation Rate (ESR)

Abdominal ultrasound (in particular for young children)

  • Size and structure of liver and spleen
  • Abdominal lymph-nodes

Coagulation Studies

  • INR/PT
  • Fibrinogen

Chest Radiograph (CXR)

Skeletal Radiograph Survey1, 2

  1. Note that other imaging techniques as bone Tc scan, PET scan or MRI are not an alternative to the standard skeletal survey. The real value of these images in LCH is still under study. In particular information from bone scan should not be considered for evaluation of disease extent and decision-making. PET scan has proven to be the most sensitive functional test used in the identification of LCH lesions and in evaluating patient response to therapy. However, it is currently expensive and not widely available [Phillips et al 2009].
  2. It is not recommended to change the method of bone evaluation (skeletal radiograph), as it may lead to discrepancy between assessments. It is important also to consider the ALARA principle (As Low As Reasonably Achievable) for ionizing radiation and, if possible, during follow up, limit the evaluation to the anatomic region initially involved.
  3. Abbreviations: ALT (SGPT), alanine transaminase (serum glutamic pyruvic transaminase); APTT/PTT, activated partial thromboplastin time/partial thromboplastin time; AST (SGOT), aspartate transaminase (serum glutamic oxaloacetic transaminase); γGT, gamma-glutamyltransferase; INR/PT, international normalized ratio/prothrombin time; MRI, magnetic resonance imaging; PET, positron emission tomography; Tc, technetium.

[Phillips et al 2009] Phillips M, Allen C, Gerson P, McClain K: Comparison of FDG-PET scans to conventional radiography and bone scans in management of Langerhans cell histiocytosis. Pediatric blood & cancer 2009, 52: 97 [PMID: 18951435]