[PAGID] fulminant EBV?

[Ashish Kumar]

Dear Colleagues,

I need help for a 23 year old woman who developed a febrile illness in
September. She rapidly developed multi-organ failure needing artificial
ventilation and renal dialysis. She was found to have EBV viremia (60,000
copies/mL blood). Further, she was found to have pancytopenia,
hypofibrinogenemia; her Ferritin was 75,000, and her CD25 level was very
high too (I don't have the result in front of me). Her bone marrow did not
show Hemophagocytosis, but given this constellation of findings, we
diagnosed her with HLH and began treatment with Dexamethasone and Etoposide.
Within days, she responded, and 2 weeks later was discharged to home. She
also received a few doses of Ganciclovir but it was causing neutropenia, so
it was stopped. She came back a week later with pain in her thighs and once
again rapidly developed multi-organ failure (in a matter of hours). This
time she also had gram-negative bacilli in her blood culture (Pseudomonas).
She is currently on the ventilator, in renal failure and in DIC. Her
Ferritin is 150,000 and the EBV copy number is 900,000 copies/mL. She is
persistently febrile to 102-104F. Her liver enzymes had gone up to 3000 but
have settled down at 1000, and her white count is 6.9 with 70% neutrophils,
20% lymphocytes. She is still on Dexamethasone, but given the gram negative
septicemia, Etoposide was withheld. My question is: what are we dealing with
- fulminant EBV in a previously healthy 23 year old female with
relapsed/refractory HLH? The blood cultures have been negative since the
first positive one a week ago. Would you treat her Etoposide and
Cyclosprine? CSA was omitted the first time because of renal failure and the
good response to 2 drugs. We are giving one dose of Infliximab today and
have resumed Ganciclovir, for whatever its worth. Her NK cell function
studies done soon after the first diagnosis were normal. Any suggestions
would be extremely helpful.

Thank you!


Ashish Kumar M.D., Ph. D.
Assistant Professor
Pediatric Hematology/Oncology/Blood and Marrow Transplantation
University of Minnesota