The clinical picture of Castleman disease: a systematic review and meta-analysis
Christian Hoffmann1,2, Eric Oksenhendler3, Sarah Littler4
1Infektionsmedizinisches Centrum Hamburg Study Center, Hamburg, Germany.
Insights
Castleman disease (CD) subtypes, unicentric (UCD), idiopathic multicentric (iMCD), and HHV8-associated MCD, show overlapping symptoms but distinct features. HHV8+ MCD cases present more constitutional symptoms and splenomegaly than iMCD.
Area of Science:
- Hematology
- Rare Diseases
- Oncology
Background:
- Castleman disease (CD) is a rare lymphoproliferative disorder with diverse subtypes.
- Key subtypes include unicentric CD (UCD), idiopathic multicentric CD (iMCD), and human herpesvirus 8-associated MCD (HHV8+ MCD).
- Understanding subtype-specific clinical presentations is crucial for diagnosis and management.
Purpose of the Study:
- To systematically review and compare the clinical symptoms and laboratory parameters across UCD, iMCD, and HHV8+ MCD subtypes.
- To identify key differences and similarities in disease manifestations to aid in differential diagnosis.
- To estimate the frequency of diagnostic criteria for iMCD using meta-analyses.
Main Methods:
- Systematic review of publications reporting ≥5 CD cases between 1995 and 2021.
- Adherence to Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines.
- Data extraction on clinical symptoms and laboratory parameters, with meta-analyses for iMCD criteria frequency.
Main Results:
- Analysis of 32 studies involving 559 UCD, 1023 iMCD, and 416 HHV8+ MCD cases.
- HHV8+ MCD cases showed significantly higher rates of constitutional symptoms (98.6%) and splenomegaly (89.2%) compared to iMCD (46.6% and 48.2%, respectively).
- Renal dysfunction was more frequent in iMCD (36.9%) than HHV8+ MCD (17.4%) pre-adjustment; UCD cases had fewer symptoms, but pediatric UCD showed pronounced abnormalities.
Conclusions:
- iMCD and HHV8+ MCD share many overlapping symptoms, including constitutional symptoms and organ dysfunction, suggesting shared pathophysiological pathways.
- Distinctive features like higher rates of constitutional symptoms and splenomegaly in HHV8+ MCD, and higher renal dysfunction in iMCD, aid in subtype differentiation.
- UCD, particularly in pediatric patients, can present with significant symptoms and laboratory abnormalities, warranting careful evaluation.
Abstract:
Castleman disease (CD) encompasses a spectrum of rare disorders, including unicentric CD (UCD), idiopathic multicentric CD (iMCD), and human herpesvirus 8-associated MCD (HHV8+ MCD). We performed a systematic review of publications reporting ≥5 cases of CD between 1995 and 2021, following preferred reporting items for systematic reviews and meta-analyses guidelines, to describe and compare subtypes. We extracted data on clinical symptoms and laboratory parameters as stated in international consensus diagnostic criteria for iMCD and estimated the frequency of each criterion using meta-analyses. We analyzed 32 studies describing 559 UCD, 1023 iMCD, and 416 HHV8+ MCD cases. Although many symptoms and laboratory abnormalities occurred at similar rates in patients with iMCD and HHV8+ MCD, patients with HHV8+ MCD had significantly higher rates of constitutional symptoms (46.6% vs 98.6%; P = .038) and splenomegaly (48.2% vs 89.2%; P = .031). Renal dysfunction was significantly more common in patients with iMCD than in patients with HHV8+ MCD before adjustment (36.9% vs 17.4%; P = .04; adjusted P = .1). Patients with UCD had lower rates of symptoms and laboratory abnormalities, although these were present in 20% of patients and were particularly pronounced in pediatric UCD. There are many similarities in the symptomatology of iMCD and HHV8+ MCD; many patients experience constitutional symptoms and organ dysfunction. Differences between these subtypes likely reflect differences in pathophysiology and/or comorbidity burdens.
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