Managing a patient with 2+ enlarged lymph nodes and no clear diagnosis / a suspicion of CD / iMCD?
Return to the diagnostic journey overview- Signs & symptoms
- Pathology services
- Diagnostic Criteria
Idiopathic multicentric Castleman disease (iMCD) – Signs and symptoms1
Often, the most clinically obvious sign of iMCD is two or more enlarged lymph nodes. A typical patient may have had enlarged lymph nodes for some time. They often have an associated constellation of symptoms, but no clear diagnosis.
Because of the presence of enlarged lymph nodes and associated symptoms, there may be an early suspicion of cancer, an autoimmune condition, or an infection. A patient with iMCD may have a mix of symptoms which can look like any of these conditions. Once these have been partially or fully ruled out, clinicians should start to consider whether the patient has iMCD.
Clinical hallmarks include1:
Fever
Night Sweats
Lymphadenopathy
Ascites
Hepatosplenomegaly
Elevated C-reactive protein (CRP)
Hypoalbuminemia
Anemia
Some patients experience mild flu-like symptoms, whereas others experience severe sepsis-like multiple organ system failure, anasarca, and death.1
Please note, not all iMCD patients will exhibit all / any of
the symptoms described here. See the
diagnostic criteria
for more information.
Idiopathic multicentric Castleman disease (iMCD) – Collaboration with pathology services1
All forms of Castleman Disease share similar changes in lymph nodes. A diagnosis of iMCD requires the confirmation of two or more enlarged lymph nodes AND the confirmation of histopathologic changes consistent with the CD / iMCD spectrum.
For this reason, if it is suspected that a patient has CD / iMCD, it is essential to involve pathology services in an assessment of the lymph node
(excisional biopsy recommended where possible).
Why are lymph node excisional biopsies important?2,3
A comprehensive assessment of the lymph node for CD / iMCD features requires sufficient tissue. Fine‑needle aspiration or core biopsy is unlikely to capture the affected tissue within the lymph node and may be inadequate for an accurate diagnosis of iMCD.
Idiopathic multicentric Castleman disease (iMCD) – International diagnostic criteria1
International diagnostic criteria for iMCD exist.
These criteria are based on the principle of establishing the two major criteria, adding two or more minor criteria including at least one laboratory abnormality, and excluding potential mimics, in order to reach a diagnosis of iMCD.
The major criteria (both must be present for a diagnosis) are:
-
Multiple (2+) enlarged lymph nodes
-
Measuring ≥ 1 cm in short-axis diameter in multiple
lymph node stations
-
Measuring ≥ 1 cm in short-axis diameter in multiple
-
Histopathologic lymph node features consistent with
the iMCD spectrum- Requires lymph node biopsy (excisional biopsy preferred)
To confirm a diagnosis of iMCD1:
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