Table 2.

Demographic features, presentation, management and outcome ANKL, EBV-

Case No.	Age/ Sex	Ethnicity	Clinical presentation	Fever	Night sweats	Splenomegaly	Lymphadenopathy	Cytopenias	Therapy	Outcome	Duration disease/ follow-up
1	22/F	White	Acute onset, skin rash, edema, ascites, hepatomegaly, jaundice, abnormal hepatic & kidney function, coagulopathy, high LDH	−	+	+	abdomen, chest	thrombocytopenia	CHOP, EPOCH, Campath + Cytosine arabinoside	DoD	8 wks
2	73/M	White	Fever and weakness, focal liver abnormalities	+	−	−	−	pancytopenia	N/A	DoD	7 wks
3	52/M	Asian	3–4 wk history of abdominal distension, chills, weakness, ascites, pleural effusion, hypoxemia, abnormal liver function	+	+	+	mediastinum	−	1xCHOP	DoD	8 wks
4	83/F	White	4 week history of fever, episodic confusion	+	−	−	−	pancytopenia	1xCHOP	DoD	8 wks
5	64/M	White	Acute onset, nausea, vomiting, diarrhea	+	+	+	diffuse	pancytopenia	6xSMILE, allogeneic MUD BMT	Alive, no disease	15 months (8 months after transplant)
6	63/M	Black	Acute onset, chest pain, skin plaques/nodules, edema, sinus changes, pericardium, pancreas, gastric wall. 1 yr history of lower extremity ulcers, Hep C, IVDA, CKD.	+	+	−	diffuse	anemia	Palliative Care	DoD	8 wks
7	60/M	White	Acute onset, skin rash/patches/plaques/nodules, edema, ascites, fever, chills, night sweats, weakness, weight loss, hepatomegaly, high LDH, abnormal kidney function	+	+	+	−	pancytopenia	2xHyper- CVAD, Bortezomib, intrathecal CHT	DoD	7 mo

Abbreviations: LPL - lymphoplasmacytic lymphoma, IVDA - intravenous drug abuse, Hep - hepatitis C, CKD – chronic kidney disease

CHT, chemotherapy, CHOP - cyclophosphamide, adriamycin, vincristine, prednisone

CVAD - cyclophosphamide, vincristine, doxorubicin, dexamethasone

EPOCH - etoposide, prednisolone, vincristine, cyclophosphamide, doxorubicin

SMILE - dexamethasone, methotrexate, ifosfamide, L-asparaginase, etoposide

UD BMT - matched unrelated donor bone marrow transplant

DoD - died of disease; wks - weeks; yrs -years, M - male; F - female