HHV-8-Associated Lymphoproliferative Disorders and Pathogenesis in an HIV-Positive Patient

Table 1

Typical features of the HHV-8-associated lymphoproliferative diseases.

Feature	DLBCL, NOS	GLPD	MCD	PEL
Clinical presentation	Diffuse LAN, constitutional symptoms, splenomegaly; HHV-8 MCD usually present	Localized LAN	Diffuse LAN, constitutional symptoms, splenomegaly	Serous effusions, no LAN
Immunosuppression	+/–	–	+/–	+/–
Microscopy	Sheets of PB/IB, effacement of nodal architecture	PB/IB in germinal centers, nodal architecture intact	PB/IB in mantle zones Interfollicular plasmacytosis	PB/IB in serous effusions (esp. pleural, pericardial, peritoneal)
Phenotype	CD20 +/–,; CD138 –	CD20 –,; CD138 –	CD20 +/–,; CD138 –	CD20 –,; CD138 +/–
Clonality	Monoclonal	Poly- or oligoclonal	Polyclonal	Monoclonal
Ig	IgM ƛ +	Monotypic κ or ƛ, any heavy chain	IgM ƛ +	–
HHV-8 (LANA)	+	+	+	+
EBV (EBER)	–	+	–	+
Prognosis	Poor	Good	Poor, but improving	Poor
Treatment	EPOCH or CHOP plus rituximab if CD20+, ART if HIV+	Chemotherapy (e.g., CHOP) or surgical resection +/–RT, ART if HIV+	No organ failure: rituximab Organ failure: chemotherapy and/or rituximab ART if HIV+	EPOCH or CHOP plus rituximab if CD20+, ART if HIV+

CD: cluster of differentiation; CHOP: cyclophosphamide, doxorubicin, vincristine, prednisone; EPOCH: etoposide, prednisolone, vincristine, cyclophosphamide, doxorubicin; IB: immunoblast; Ig: immunoglobulin; LAN: lymphadenopathy; PB: plasmablast; RT: radiation therapy.