Systemic Epstein-Barr virus positive T-cell lymphoproliferative disease of childhood with hemophagocytic syndrome.
Epstein-Barr virus (EBV) associated lymphoproliferative disease (LPD) are commonly derived from B-cells, however, it is becoming more and more apparently that EBV can also infect T-lymphocytes. Systemic EBV positive T-cell LPD of childhood is rare and characterized by an extremely aggressive course and poor prognosis. Here, we report a 22-year-old female of systemic EBV positive TLPD with acute EBV infection and review the clinical features of this disorder. A 22-year-old previously healthy female without immunocompromised status presented with persisting coach and fever resistant to conventional therapies. Physical examination showed hemorrhage and hepatosplenomegaly. Laboratory examinations revealed severe pancytopenia, disseminated intra-vascular coagulopathy (DIC), and anti-EBV-IgM positivity. Peripheral blood smears and bone marrow investigation identified a number of atypical lymphocytes. Flow cytometry (FCM) did not show any significant evidence of leukemia or lymphoma. The lymph node biopsy showed apparent infiltration of lymphocytes, which expressed CD2+, CD3+, CD7+ and TIA1+. There was no CD20+ or CD56+ cells. EBV early RNA (EBER) was positive. Cytogenetic analysis showed a normal karyotype. T-cell receptor (TCR) gene rearrangement revealed a polyclonal pattern. The patient received prednisolone and IVIG therapy with a transient good condition, and then died of multiorgan failure one week after diagnosis.
Department of Hematology, Huizhou Municipal Central Hospital 41 Eling Road North, Huizhou 516001, P. R. China.
Guoshu
G
Chen
Department of Hematology, Huizhou Municipal Central Hospital 41 Eling Road North, Huizhou 516001, P. R. China.
Li
L
Chen
Department of Hematology, Huizhou Municipal Central Hospital 41 Eling Road North, Huizhou 516001, P. R. China.
Xiaohua
X
Qin
Department of Pathology, Huizhou Municipal Central Hospital 41 Eling Road North, Huizhou 516001, P. R. China.
Zhuoya
Z
Huang
Department of Hematology, Huizhou Municipal Central Hospital 41 Eling Road North, Huizhou 516001, P. R. China.
Xiaoling
X
Xie
Department of Hematology, Huizhou Municipal Central Hospital 41 Eling Road North, Huizhou 516001, P. R. China.
Guowei
G
Li
Department of Hematology, Nanfang Hospital, Southern Medical University Guangzhou 510515, P. R. China.
Bing
B
Xu
D001706:Biopsy; D020031:Epstein-Barr Virus Infections / Q000175:diagnosis / Q000235:genetics / Q000276:immunology / Q000628:therapy / Q000821:virology*; D017809:Fatal Outcome; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D004854:Herpesvirus 4, Human / Q000235:genetics / Q000302:isolation & purification*; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007150:Immunohistochemistry; D017403:In Situ Hybridization; D008198:Lymph Nodes / Q000276:immunology / Q000821:virology*; D051359:Lymphohistiocytosis, Hemophagocytic / Q000175:diagnosis / Q000235:genetics / Q000276:immunology / Q000628:therapy / Q000821:virology*; D008232:Lymphoproliferative Disorders / Q000175:diagnosis / Q000235:genetics / Q000276:immunology / Q000628:therapy / Q000821:virology*; D009102:Multiple Organ Failure / Q000209:etiology; D010641:Phenotype; D011239:Prednisolone / Q000627:therapeutic use; D012367:RNA, Viral / Q000302:isolation & purification; D013601:T-Lymphocytes / Q000276:immunology / Q000821:virology*; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult
D005938:Glucocorticoids; D016756:Immunoglobulins, Intravenous; D012367:RNA, Viral; D011239:Prednisolone
Epstein-Barr virus; hemophagocytic syndrome; lymphoproliferative disease