Table 1. Clinical, histologic,... | American Society of Hematology

Table 1.

Clinical, histologic, immunophenotypic, and genotypic features of KSHV-associated GLD

	Case 1	Case 2	Case 3
Age, y	41	61	63
Sex	Male	Male	Female
Clinical presentation	Axillary (6 × 6 × 6 cm) and cervical lymph node enlargement for 6 y; staging revealed right perirenal lymphadenopathy; bone marrow trephine and aspirate were normal	Submandibular and inguinal lymph node enlargement for 4 y; 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)
Viral serology	KSHV⁺, EBV⁺, HCV⁺, HIV⁻	KSHV⁺, EBV⁺, HIV⁻	Not available
Treatment and outcome	6 cycles of CHOP, complete remission for 7 y	Surgical excision and radiotherapy, complete remission for 15 y	New case
B-cell markers	CD20⁻ CD27⁻	CD20⁻ CD27⁻	CD20⁻ CD27⁻
	CD30⁻ CD38⁻	CD30⁺ CD38⁺	CD30⁻ CD38⁺
	CD79a⁻ CD138⁻	CD79a⁻ CD138⁻	CD79a⁻ CD138⁻
	BCL6⁻ CD10⁻	BCL6⁻ CD10⁻	BCL6⁻ CD10⁻
		EMA⁻	BCL2⁻
Ig light chain	λ	λ	κ
Ig heavy chain	IgM⁺D⁺	IgA⁺	IgM⁻, IgD⁻, IgG⁻, IgA⁻
Clonality
Whole tissue section	Weak dominant band in a polyclonal background	PCR failed	Polyclonal
KSHV-positive aggregate	Oligoclonal	PCR failed	Polyclonal
V_H gene mutation	Yes	Not done	Not done

	Case 1	Case 2	Case 3
Age, y	41	61	63
Sex	Male	Male	Female
Clinical presentation	Axillary (6 × 6 × 6 cm) and cervical lymph node enlargement for 6 y; staging revealed right perirenal lymphadenopathy; bone marrow trephine and aspirate were normal	Submandibular and inguinal lymph node enlargement for 4 y; 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)
Viral serology	KSHV⁺, EBV⁺, HCV⁺, HIV⁻	KSHV⁺, EBV⁺, HIV⁻	Not available
Treatment and outcome	6 cycles of CHOP, complete remission for 7 y	Surgical excision and radiotherapy, complete remission for 15 y	New case
B-cell markers	CD20⁻ CD27⁻	CD20⁻ CD27⁻	CD20⁻ CD27⁻
	CD30⁻ CD38⁻	CD30⁺ CD38⁺	CD30⁻ CD38⁺
	CD79a⁻ CD138⁻	CD79a⁻ CD138⁻	CD79a⁻ CD138⁻
	BCL6⁻ CD10⁻	BCL6⁻ CD10⁻	BCL6⁻ CD10⁻
		EMA⁻	BCL2⁻
Ig light chain	λ	λ	κ
Ig heavy chain	IgM⁺D⁺	IgA⁺	IgM⁻, IgD⁻, IgG⁻, IgA⁻
Clonality
Whole tissue section	Weak dominant band in a polyclonal background	PCR failed	Polyclonal
KSHV-positive aggregate	Oligoclonal	PCR failed	Polyclonal
V_H gene mutation	Yes	Not done	Not done

CHOP indicates cyclophosphamide, hydroxydoxorubicin, oncovin, prednisone.