[Two young adult cases of Epstein-Barr virus associated-hemophagocytic lymphohistiocytosis with monoclonal proliferation of virus-infected cells within a short period after primary infection].
Hemophagocytic lymphohistiocytosis (HLH) is a rare but severe complication of Epstein-Barr virus (EBV) infection. Interactions between EBV-infected T cells and activated macrophages cause several conditions such as pancytopenia, liver dysfunction and coagulopathy. We describe here two young adults with EBV-associated HLH with monoclonal proliferation of EBV-infected T cells within a short period after infectious mononucleosis as a primary infection. One patient was a 16-year-old man who developed severe pancytopenia and liver dysfunction two months after infectious mononucleosis. Bone marrow examination showed hemophagocytosis, and laboratory data demonstrated monoclonal proliferation of EBV-infected T cells. Several treatments such as immunosuppressive therapy, chemotherapy and hematopoietic stem cell transplantation were not effective, and the patient died of progressive disease. The other patient was a 19-year-old woman who developed thrombocytopenia and liver dysfunction two months after infectious mononucleosis. Findings of hemophagocytosis and monoclonal proliferation of EBV-infected T cells were similar to those in the first case. Clinical signs and symptoms were resolved completely by immunosuppressive therapy containing methyl-prednisolone and cyclosporine. Since these two cases each demonstrated a distinct clinical course, an investigation of the prognostic factors and treatment strategies for EBV-HLH is warranted.
Idutsu
Kensaku
K
Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University.
Abe
Yasunobu
Y
Matsushima
Takamitsu
T
Sada
Eriko
E
Ohtsuka
Rie
R
Kiyasu
Junichi
J
Shiratsuchi
Motoaki
M
Kotoh
Kazuhiro
K
Nishimura
Junji
J
Ohga
Shouichi
S
Takayanagi
Ryoichi
R
D000293:Adolescent; D049109:Cell Proliferation; D017809:Fatal Outcome; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation; D004854:Herpesvirus 4, Human; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007244:Infectious Mononucleosis / Q000150:complications*; D051359:Lymphohistiocytosis, Hemophagocytic / Q000209:etiology* / Q000628:therapy; D008297:Male; D013601:T-Lymphocytes / Q000473:pathology / Q000821:virology*; D016896:Treatment Outcome; D055815:Young Adult
D007166:Immunosuppressive Agents