HHV8/KSHV-reIated diseases: From Cellular Proliferation to Hyperinflammation
Kaposi sarcoma (KS):
• Target organs: Ubiquitous (cutaneous and mucosal predominance)
• Clinical features: Blue-violet macules or nodules (+/- according to visceral involvement)
• Histology: LANA+ fusiform cells (*) from endothelium
• Association HIV/EBV: Yes/No
• Clonality: Polyclonal (except nodular forms)
• Light chain: -
HHV8+ Multicentric Castleman Disease (MCD):
• Target organs: Lymph nodes and spleen
• Clinical features: Fever, adenopathies (ADP), splenomegaly, effusion, hemophagocytosis
• Histology: LANA+ plasmablasts from pre-GC B cells
• Association HIV/EBV: Yes/Rare
• Clonality: Polyclonal
• Light chain: Constant λ Monotype
Primary effusion lymphoma (PEL):
• Target organs: Pleural, pericardial, peritoneal cavities
• Clinical features: Effusion, B symptoms
• Histology: LANA+ immunoblastic/anaplasic cells from differentiated B cells
• Association HIV/EBV: Yes / Yes
• Clonality: Monoclonal
• Light chain: Monotype
By Dr. Gregoire Martin de Fremont
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