CASTLEMAN DISEASE Disorder of the immune system with excessive production of IL-6 Castleman’s disease is a rare, heterogeneous group of lymphoproliferative disorders characterized by distinctive histopathological features. CD is broadly classified into two main types: • Unicentric Castleman disease (UCD) • Multicentric Castleman disease (MCD) Histologically, CD can be classified into, hyaline vascular, plasma cell and mixed cellularity types. Hyaline vascular type is more common in UCD, while plasma cell type is more frequently seen in MCD. Localized - Unicentric Castleman Disease (UCD): • Affects a single lymph node region. • More common and generally less serious. • Often asymptomatic, but can cause symptoms due to compression of nearby structures (e.g., chest pain, cough). • Usually treated with surgical removal of the affected lymph node, which is often curative. Multicentric Castleman Disease (MCD): • Affects multiple lymph node regions and can involve other organs. • More serious and can resemble cancer or autoimmune disorders. • Associated with systemic symptoms such as: Fever, Night sweats, Fatigue, Weight loss, Enlarged liver or spleen • Often linked to infection with human herpesvirus-8 (HHV-8), especially in people with HIV. Lab Tests: 1. CBC: Anemia and thrombocytopenia, particularly in MCD 2. CRP: Elevated CRP levels are common in MCD due to systemic inflammation 3. ESR: Typically elevated in MCD, reflecting the inflammatory state 4. Serum IL-6: Elevated IL-6 levels are a hallmark of MCD, especially in HHV-8-associated cases 5. Serum Immunoglobulins: Hypergammaglobulinemia is frequently observed in MCD 6. Serum Albumin: Hypoalbuminemia is common in MCD due to systemic inflammation 7. Viral Serologies: Testing for HHV-8 and HIV is crucial, as HHV-8 is associated with a significant proportion of MCD cases 8. Soluble Interleukin-2 Receptor (sIL-2R): Elevated levels can be seen in MCD and may help in diagnosis 9. Vascular Endothelial Growth Factor (VEGF): Elevated VEGF levels can be indicative of MCD #Castleman #disease #diagnosis
