TY - JOUR
T1 - Five adult cases of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis
AU - Abe, Yasunobu
AU - Shiratsuchi, Motoaki
AU - Nagasawa, Eriko
AU - Ohtsuka, Rie
AU - Kiyasu, Junichi
AU - Sada, Eriko
AU - Idutsu, Kensaku
AU - Kotoh, Kazuhiro
AU - Nishimura, Junji
AU - Ohga, Shouichi
AU - Takayanagi, Ryoichi
PY - 2010/1
Y1 - 2010/1
N2 - 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.
AB - 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.
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M3 - Article
C2 - 20134144
AN - SCOPUS:77950349750
SN - 0485-1439
VL - 51
SP - 74
EP - 79
JO - [Rinshō ketsueki] The Japanese journal of clinical hematology
JF - [Rinshō ketsueki] The Japanese journal of clinical hematology
IS - 1
ER -