An overview of the iMCD diagnostic process and the role of pathology
A diagnosis of iMCD requires close collaboration between pathology services and treating physicians.
It is typical for pathologists to raise a suspicion of iMCD or other forms of Castleman Disease when evaluating (excisional) lymph node biopsies.
Given the spectrum of symptoms seen in iMCD, treating physicians may have suspicions of autoimmune disorders, infections, or malignancies prior to the pathologist receiving a sample.
If you suspect iMCD or other forms of Castleman Disease, it is important to recommend that the patient be referred to the care of a haematologist or similar specialist (if not already), as this is the specialty best placed to manage iMCD. In some countries, expert Castleman Disease centres may be established and can provide support.
MAJOR DIAGNOSTIC CRITERIA
A diagnosis of iMCD requires the confirmation of two major criteria, one of which must be assessed by pathology services.
MAJOR CLINICAL CRITERIA
- Multiple enlarged lymph nodes
- Measuring ≥1cm in short-axis diameter in multiple lymph node stations
MAJOR PATHOLOGY CRITERIA
- Histopathologic lymph node features consistent with the iMCD spectrum
-
Requires lymph node biopsy
(excisional biopsy preferred)
In addition to these, two or more minor criteria must be present (including at least one laboratory abnormality), and a number of additional conditions excluded before diagnosis can be confirmed.
References
- Fajgenbaum DC, et al. Blood. 2017; 129: 1646–57.
Abbreviations
iMCD, idiopathic Multicentric Castleman Disease.
