[Five adult cases of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis].
Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) is more common in children, and is characterized by pancytopenia, liver dysfunction and coagulopathy caused by interactions between EBV-infected T cells and activated macrophages. We describe here five adults with EBV-HLH. The median age was 17 years (range 16 approximately 40). HLH developed in 4 patients within 2 months after the primary infection, and in the other one during the reactivation. All patients had a high EBV viral load in peripheral blood (2 x 10(2)-3 x 10(6) copies/ml) and monoclonal proliferation of EBV-infected T cells. All patients received immunosuppressive therapy with or without etoposide, and two patients required plasmapheresis due to the severity. Three patients are alive in complete remission (follow up periods; 13, 19, 30 months), while two patients became refractory to chemo-immunotherapy and died despite multidrug chemotherapy. EBV-HLH should be more widely recognized in adults in order to achieve early diagnosis and appropriate treatment.
Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University.
Yasunobu
Y
Abe
Motoaki
M
Shiratsuchi
Eriko
E
Nagasawa
Rie
R
Ohtsuka
Junichi
J
Kiyasu
Eriko
E
Sada
Kensaku
K
Idutsu
Kazuhiro
K
Kotoh
Junji
J
Nishimura
Shouichi
S
Ohga
Ryoichi
R
Takayanagi
D000293:Adolescent; D000328:Adult; D049109:Cell Proliferation; D042241:Early Diagnosis; D020031:Epstein-Barr Virus Infections; D005047:Etoposide / Q000008:administration & dosage; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D051359:Lymphohistiocytosis, Hemophagocytic / Q000175:diagnosis / Q000628:therapy* / Q000821:virology*; D008262:Macrophage Activation; D008297:Male; D010956:Plasmapheresis; D012074:Remission Induction; D013601:T-Lymphocytes / Q000473:pathology / Q000821:virology; D055815:Young Adult
D007166:Immunosuppressive Agents; D005047:Etoposide