Classification

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What is Castleman disease ?

Castleman disease is a non-malignant disorder of the lymph nodes.

  • It triggers the development of benign tumours in the lymph nodes.
  • It is not a type of cancer.
  • This disease is not contagious or hereditary.

It is diagnosed by examining a lymph node (biopsy) which has particular characteristics suggestive of the condition.

The disease can manifest in three different forms, which is significant in terms of presentation and treatment.

Simple laboratory tests conducted on a blood sample, combined with radiological examinations (scan or PET scan), can distinguish between the three different diseases:

Unicentric Castleman disease (localized)

  • Affects a single lymph node or a group of lymph nodes.
  • Generally non-symptomatic.

Treatment of Unicentric Castleman disease

 

Multicentric Castleman disease (disseminated) with no known cause (idiopathic)

  • Affected several lymph nodes
  • General inflammation with fever, sometimes accompanied by weight loss

Treatment of Multicentric Castleman disease

Multicentric Castleman disease associated with the HHV-8 virus.

  • Presents as a multicentric form but the cause is known.
  • This is a disease associated with the HHV-8 virus which can be found in the lymph nodes or the blood.
  • It is more common in patients infected with the HIV virus.

Treatment of Castleman disease associated with HHV8

Damaged lymph nodes such as those observed in Castleman disease may be symptomatic of other diseases. These are not true Castleman diseases.

 

Castleman disease must be diagnosed by a specialist, preferably in a reference centre or failing that, a haematology or internal medicine department.