Table 1

HHV8-related neoplastic and non-neoplastic diseases in posttransplantation patients

HHV8-related posttreatment diseasesClinical presentations and histopathologic featuresMonitoring testsTherapeutic options*
Neoplastic    
    KS Cutaneous or mucosal lesions; visceral involvement; hematologic manifestations. HHV8+ spindle cells; inflammatory infiltrates HHV8 viral load (Q-PCR): useful; HHV8 T-cell quantitation (ELISPOT): useful Reduction of IS or switch to SRL (II-3); CHT (liposomal anthracyclines; II-3); antivirals (III) 
    MCD Lymphadenopathy; systemic inflammatory symptoms. HHV8+ plasmablasts in mantle zone of follicles; vascular hyperplasia HHV8 viral load (Q-PCR): useful; HHV8 T-cell quantitation (ELISPOT): investigational Reduction of IS or switch to SRL (III); rituximab (II-3); CHT (CVP, CHOP, R-CHOP) (II-3); antivirals (III) 
    PEL Pleural, peritoneal, pericardial effusions; HHV8+ plasmablasts with immunoblastic and anaplastic features HHV8 viral load (Q-PCR): useful; HHV8 T-cell quantitation (ELISPOT): investigational Reduction of IS (III); CHT (CHOP) (II-3); antivirals (III) 
Non-neoplastic    
    Plasmacytic B-cell proliferation Polyclonal HHV8+ B-cell proliferations, in lymph nodes and visceral organs; systemic inflammatory symptoms HHV8 viral load (Q-PCR): useful (also for diagnosis) Reduction of IS (III); rituximab (III); antivirals (III) 
    BM failure Acute bone marrow failure, often with plasmacytosis and signs of HPS; hepatosplenomegaly and severe pancytopenia; systemic inflammatory symptoms; skin maculopapular rash HHV8 viral load (Q-PCR): useful (also for diagnosis) Reduction of IS (III); rituximab (III); antivirals (III) 
    Hepatitis Elevated liver enzymes; systemic inflammatory symptoms; skin maculopapular rash HHV8 viral load (Q-PCR): useful (also for diagnosis) Reduction of IS (III); antivirals (III) 
HHV8-related posttreatment diseasesClinical presentations and histopathologic featuresMonitoring testsTherapeutic options*
Neoplastic    
    KS Cutaneous or mucosal lesions; visceral involvement; hematologic manifestations. HHV8+ spindle cells; inflammatory infiltrates HHV8 viral load (Q-PCR): useful; HHV8 T-cell quantitation (ELISPOT): useful Reduction of IS or switch to SRL (II-3); CHT (liposomal anthracyclines; II-3); antivirals (III) 
    MCD Lymphadenopathy; systemic inflammatory symptoms. HHV8+ plasmablasts in mantle zone of follicles; vascular hyperplasia HHV8 viral load (Q-PCR): useful; HHV8 T-cell quantitation (ELISPOT): investigational Reduction of IS or switch to SRL (III); rituximab (II-3); CHT (CVP, CHOP, R-CHOP) (II-3); antivirals (III) 
    PEL Pleural, peritoneal, pericardial effusions; HHV8+ plasmablasts with immunoblastic and anaplastic features HHV8 viral load (Q-PCR): useful; HHV8 T-cell quantitation (ELISPOT): investigational Reduction of IS (III); CHT (CHOP) (II-3); antivirals (III) 
Non-neoplastic    
    Plasmacytic B-cell proliferation Polyclonal HHV8+ B-cell proliferations, in lymph nodes and visceral organs; systemic inflammatory symptoms HHV8 viral load (Q-PCR): useful (also for diagnosis) Reduction of IS (III); rituximab (III); antivirals (III) 
    BM failure Acute bone marrow failure, often with plasmacytosis and signs of HPS; hepatosplenomegaly and severe pancytopenia; systemic inflammatory symptoms; skin maculopapular rash HHV8 viral load (Q-PCR): useful (also for diagnosis) Reduction of IS (III); rituximab (III); antivirals (III) 
    Hepatitis Elevated liver enzymes; systemic inflammatory symptoms; skin maculopapular rash HHV8 viral load (Q-PCR): useful (also for diagnosis) Reduction of IS (III); antivirals (III) 

Q-PCR indicates quantitative polymerase chain reaction; ELISPOT, enzyme-linked immunospot; IS, immunosuppression; SRL, sirolimus; and CHT, chemotherapy.

*

Levels of evidence (according to the US Preventive Services Task Force system) for therapeutic options are reported in parentheses.

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