Table 1.

Clinical and Biological Features of GLPD

	Current Case 1	Current Case 2	Lit Case 1	Lit Case 2	Lit Case 3	Lit Case 4	Lit Case 5	Lit Case 6
Reported by			Du et al., 2002	Du et al., 2002	Du et al., 2002	D’antonio Antonio et al., 2007	Oh et al., 2012	Taris et al., 2014
Age (years)/sex	84/Female	58/ Male	41/Male	61/Male	63/Female	60/Male	75/Male	49/Female
Clinical presentation	Multifocal bulky lymphadenopathy involving left common femoral (largest node, 5 × 3.7 cm), common, external, and internal iliac chain lymph nodes and right axillary lymph nodes	10-year-history of localized right axillary mass, extending to chest wall, slowly growing (26 ×16 × 11 cm). Mild splenomegaly, no B symptoms	Axillary (6 × 6 × 6 cm) and cervical lymph node enlargement for 6 years; staging revealed right perirenal lymphadenopathy; bone marrow trephine and aspirate were normal	Submandibular and inguinal lymph node enlargement for 4 years; staging showed slightly enlarged spleen and right “paratracheal” lymphadenopathy	Presented with left leg swelling and paraesthesia; enlarged paraaortic lymph node (5 × 4 × 3.5 cm)	Cervical lymphadenopathy (2.8 ×1.2 cm); no other lymphadenopathy; post-operative radiological studies and bilateral bone marrow biopsies were normal	Incidental (3 × 2.5 × 1.5 cm) mass in the neck; enlarged, solid, enhancing lymph node with an increased FDG uptake in left submandibular area; 1.5 cm partially cystic lymph node in left cervical level II area; bilateral bone marrow trephine and aspirate were normal	Right jugulo-cervical isolated nodal mass, slowly evolving for 4 months; no systemic symptoms; 9 nodes including two nodes (greater than 3.5 cm × 3 cm)
Viral serology	KSHV+ EBV+ HCV− HIV−	KSHV+ EBV+ HIV+	KSHV+ EBV+ HCV+ HIV−	KSHV+ EBV+ HIV−	Not available	KSHV+ EBV+ HIV−	KSHV+ EBV+ HIV−	KSHV+ EBV+ HIV− HBV− HCV− HTLV1− HTLV2−
Treatment	6 cycles of CHOP	Resection	6 cycles of CHOP	Surgical excision and radiotherapy	Not available		4 cycles of CHOP	Surgical excision and radiotherapy
Outcome	Complete remission after 6 months	Subsequent EBV+ DLBCL (HHV8−) Patient expired, exact date unknown.	Complete remission for 7 years	Complete remission for 15 years	NA	No clinical evidence of relapse after 2 years	Remained disease- free for 19 months	Complete remission for more than 1 year
B-cell antigens	CD20− PAX5− CD79a− CD138− BCL6− CD10−, MUM1+, EMA+ focal, CD30−	CD20− PAX5− BCL6− MUM1+, CD10− CD30−	CD20− CD79a− CD138− CD38−BCL6− CD10− CD27− CD30−	CD20− CD79a− CD138− CD38+ BCL6− CD10− EMA− CD27− CD30+	CD20− CD79a− CD138− CD38+ BCL6− CD10− EMA− CD27− BCL2− CD30−	CD20− CD79a− CD138− BCL6− CD10− CD22− BCL2− CD19− CD45− CD30−	CD20− CD79a− CD138+ CD10− EMA+ CD30−	CD20− PAX5− CD79a− CD138− CD38+ weak, EMA+ MUM1+ BOB1+ OCT2+ BCL2+ CD30−
T/NK cell & other antigens	CD56− ALK1− CD3+ partial, CD5− CD43+ partial, CD7− CD4− CD8−	CD3+ partial, CD5− CD4− CD8− CD2−	ND	ND	ND	ND	CD3− CD5− CD43+ CD15− CD21− CD23− CD45− CD56− CD68− S100−	ALK1− CD3− CD15− CD21− CD23− CD45− CD56− CD68− S100−
Viral Antigens	vIL-6+ LANA+ EBER+ LMP1− EBNA2− BZLF1−	vIL-6+ LANA+ EBER+ LMP1− EBNA2− BZLF1−	largely vIL-6+ LANA+ EBER+	largely vIL-6+ LANA+ EBER+	largely vIL-6+ LANA+ EBER+	LANA+ EBER+	HHV8+ EBER+	HHV8+ EBER+
Ig light chain	None	None	lambda	lambda	kappa	kappa	kappa	lambda
Ig heavy chain	Not done	Not done	IgM+ D+	IgA+	IgM− IgD− IgG− IgA−	IgM+	Not done	Not done
IG Clonality by PCR	Polyclonal for both B and T cell gene rearrangements	No amplification	Weak dominant band in a polyclonal background	PCR failed	Polyclonal	Polyclonal	Not done	Polyclonal