[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.
Abe
Yasunobu
Y
Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University.
Shiratsuchi
Motoaki
M
Nagasawa
Eriko
E
Ohtsuka
Rie
R
Kiyasu
Junichi
J
Sada
Eriko
E
Idutsu
Kensaku
K
Kotoh
Kazuhiro
K
Nishimura
Junji
J
Ohga
Shouichi
S
Takayanagi
Ryoichi
R
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