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
- Diagnostic guidelines
- Pathology services
Idiopathic MCD (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 will 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, you should start to consider whether your patient has iMCD.
Abbreviations: CD, Castleman disease; iMCD,
idiopathic Multicentric
Castleman Disease.
References: 1. Fajgenbaum DC, et al. Blood. 2017; 129: 1646–57.
Patients can often present with a mix of symptoms, including...
Flu-like fever
Arthritis
Neuropathy
Oedema
Enlarged organs
Weight loss
Skin changes/lesions
Anaemia
Please note, not all iMCD patients will exhibit all / any of the symptoms described here. See the diagnostic guidelines (Fajgenbaum DC, et al. Blood. 2017) for more information.
Idiopathic MCD (iMCD) – International diagnostic guidelines1
International diagnostic guidelines for iMCD exist.
These guidelines are based on the principle of establishing the two major criteria, adding two or more minor criteria, 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
- Histopathologic lymph node features consistent with the iMCD spectrum
- Requires lymph node biopsy (excisional biopsy preferred)
If you suspect CD / iMCD, involve your pathology colleagues and provide
them
with an excisional lymph node biopsy
Abbreviations: CD, Castleman disease; iMCD,
idiopathic Multicentric
Castleman Disease.
References: 1. Fajgenbaum DC, et al. Blood. 2017; 129: 1646–57.
To confirm a diagnosis of iMCD:1
Idiopathic MCD (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 you suspect a patient has CD / iMCD, it is important to involve pathology services in an assessment of the lymph node (excisional biopsy recommended where possible), and highlight to your pathology colleagues that you have a suspicion of CD / iMCD so that they can perform the appropriate tests.
Expert-led guidance for pathology colleagues can be found here.
Abbreviations: CD, Castleman disease; iMCD,
idiopathic Multicentric
Castleman Disease.
References: 1. Fajgenbaum DC, et al. Blood. 2017; 129: 1646–57. 2. Lomas OC,
et al. Br J Haematol. 2021; 195(3): 328-37.
Why are lymph node excisional biopsies important?1,2
A comprehensive assessment of the lymph node for CD/iMCD features requires sufficient tissue. Core needle biopsies (which are often favoured) provide limited tissue, which increases the risk of false negatives being returned by pathology services.
Remember to tell pathology colleagues that you “suspect Castleman disease”
