Table 1.

Salient clinical features of 6 cases with EBV+ T-cell LPD

Case no. Age/sex Ethnic origin Clinical presentation Original diagnosis Therapy and outcome
1 37/M  White  Fever, mental status changes of 1 wk duration; hepatosplenomegaly, jaundice, pancytopenia EBV IgG anti-VCA 1:640, IgM anti-VCA neg  TTP  Plasmapheresis, supportive therapy; dead at 2 wk  
2  17/M  Mexican  Symptoms of viral upper respiratory infection for 1 wk; hepatosplenomegaly, jaundice, pancytopenia EBV serology not done Viral hepatitis  Vincristine, prednisone; dead at 8 wk 
3  23/M  Asian  Fever, night sweats, weight loss of a few weeks' duration; hepatosplenomegaly, jaundice, generalized lymphadenopathy, pancytopenia monospot negative, EBV IgM anti-VCA negative, IgG anti-VCA not done  Infection Chemotherapy with CHOP, dead at 10 mo  
4  22/F Mexican  Fever, weight loss, jaundice, hepatosplenomegaly for 2 mo EBV serology not done  Viral/autoimmune hepatitis  Supportive therapy, lost to follow-up in serious condition at 3 mo  
5  27 mo/M  Mexican  Fever of 2 wk duration; skin rash, hepatosplenomegaly, pancytopenia EBV IgG anti-VCA 2.07 (nl .00-0.9), IgM anti-VCA negative HPS/leukemia  Antibiotics, etoposide; dead at 5 d  
6 15/F  White  Diagnosed with IM at 8 yr old, followed by persistent fever, fatigue, c/w CAEBV. Developed significant hepatosplenomegaly at 14 yr old, HPS 6 mo later EBV IgG anti-VCA 1:40,960, IgA anti-VCA 1:512, EA-D 1:10,240  Known CAEBV  Valacyclovir, supportive Rx; dead at 6 mo of developing HPS 
Case no. Age/sex Ethnic origin Clinical presentation Original diagnosis Therapy and outcome
1 37/M  White  Fever, mental status changes of 1 wk duration; hepatosplenomegaly, jaundice, pancytopenia EBV IgG anti-VCA 1:640, IgM anti-VCA neg  TTP  Plasmapheresis, supportive therapy; dead at 2 wk  
2  17/M  Mexican  Symptoms of viral upper respiratory infection for 1 wk; hepatosplenomegaly, jaundice, pancytopenia EBV serology not done Viral hepatitis  Vincristine, prednisone; dead at 8 wk 
3  23/M  Asian  Fever, night sweats, weight loss of a few weeks' duration; hepatosplenomegaly, jaundice, generalized lymphadenopathy, pancytopenia monospot negative, EBV IgM anti-VCA negative, IgG anti-VCA not done  Infection Chemotherapy with CHOP, dead at 10 mo  
4  22/F Mexican  Fever, weight loss, jaundice, hepatosplenomegaly for 2 mo EBV serology not done  Viral/autoimmune hepatitis  Supportive therapy, lost to follow-up in serious condition at 3 mo  
5  27 mo/M  Mexican  Fever of 2 wk duration; skin rash, hepatosplenomegaly, pancytopenia EBV IgG anti-VCA 2.07 (nl .00-0.9), IgM anti-VCA negative HPS/leukemia  Antibiotics, etoposide; dead at 5 d  
6 15/F  White  Diagnosed with IM at 8 yr old, followed by persistent fever, fatigue, c/w CAEBV. Developed significant hepatosplenomegaly at 14 yr old, HPS 6 mo later EBV IgG anti-VCA 1:40,960, IgA anti-VCA 1:512, EA-D 1:10,240  Known CAEBV  Valacyclovir, supportive Rx; dead at 6 mo of developing HPS 

IM indicates infectious mononucleosis; VCA, viral capsid antigen; TTP, thrombotic thrombocytopenic purpura; CAEBV, chronic active EBV infection; HPS, hemophagocytic syndrome; CHOP, cyclophosphamide, hydroxydaunomycin, Oncovin, and predinsone.

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