International, evidence-based consensus diagnostic criteria for HHV-8-negative/idiopathic multicentric Castleman disease.
Level 5 - mechanism / opinion, no new human data
Expert consensus guideline and retrospective case review
PubMed 28087540 · doi:10.1182/blood-2016-10-746933
What was done
An international working group of 34 pediatric and adult pathology and clinical experts from eight countries reviewed clinical data from 244 cases to establish consensus diagnostic criteria for HHV-8-negative/idiopathic multicentric Castleman disease over a 15-month period.
What was found
The consensus criteria require both major criteria (characteristic lymph node histopathology and multicentric lymphadenopathy), at least two of 11 minor criteria with at least one laboratory abnormality, and exclusion of infectious, malignant, and autoimmune mimics. Minor laboratory criteria include elevated C-reactive protein or erythrocyte sedimentation rate, anemia, thrombocytopenia or thrombocytosis, hypoalbuminemia, renal dysfunction or proteinuria, and polyclonal hypergammaglobulinemia. Minor clinical criteria include constitutional symptoms, hepatosplenomegaly, effusions or edema, eruptive cherry hemangiomatosis or violaceous papules, and lymphocytic interstitial pneumonitis.
Why it matters
This establishes the first unified international diagnostic definition for idiopathic multicentric Castleman disease, facilitating accurate diagnosis and consistent patient enrollment in clinical trials.
Limits
The criteria were formulated via expert consensus and retrospective case review without prospective validation described in the abstract. Diagnostic accuracy statistics such as sensitivity, specificity, and predictive values are not reported.
Cited by
- supports Castleman disease is an atypical lymphoproliferative disorder where hyperactivated immune cells produce cytokines that cause vital organ failure.