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Castleman Disease (CD) - Rare immune dysregulation disorder that causes…
Castleman Disease (CD) - Rare immune dysregulation disorder that causes enlarged lymph nodes and chronic inflammation.
Etiology (Causes)
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Non-modifiable: HHV-8, HIV, age (30-60), weakened immune system
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Diagnosis
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Blood tests (CBC, CRP & ESR, Kidney function, HIV & HVV-8 testing
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Prognosis
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Multicentric CD
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Risk of infection, organ failure, lymphoma
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ETIOLOGY > SYMPTOMS > SIGNS
Immune dysregulation → Increased IL-6 production → Chronic inflammation → Enlarged lymph nodes + Fever + Fatigue + Weight loss → Multicentric Castleman disease symptoms
ETIOLOGY > DIAGNOSIS Persistent enlarged lymph nodes → Physical examination → Blood tests (CRP, ESR, CBC, HIV, HHV-8) → CT/PET scan → Lymph node biopsy (Gold Standard) → Castleman disease diagnosis
UNICENTRIC DISEASE Single enlarged lymph node → Unicentric Castleman disease → Surgical removal of lymph node → Inflammation resolved → Normal life expectancy
MULTICENTRIC DISEASE HHV-8 infection/HIV or idiopathic immune dysregulation → Multicentric Castleman disease → Immunotherapy (Siltuximab) + Corticosteroids ± Rituximab/Chemotherapy → Reduced IL-6 activity → Decreased inflammation → Improved quality of life
SYMPTOMS > DIAGNOSIS > TREATMENT Fever + Night sweats + Weight loss + Enlarged lymph nodes → Medical evaluation → CT/PET scan + Lymph node biopsy → Determine UCD vs. MCD → Appropriate treatment