-Case Reports-

Epstein-Barr Virus-positive T-cell Lymphoproliferative Disease Following Umbilical Cord Blood Transplantation for Acute Myeloid Leukemia

Shunsuke Yui¹, Hiroki Yamaguchi¹, Ken-ichi Imadome², Ayako Arai³, Mikiko Takahashi⁴, Ryuji Ohashi⁴, Hayato Tamai¹, Keiichi Moriya¹, Kazutaka Nakayama¹, Akira Shimizu⁴ and Koiti Inokuchi¹

¹Department of Hematology, Nippon Medical School
²Division of Advanced Medicine for Virus Infections, National Research Institute for Child Health and Development
³Department of Hematology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University
⁴Division of Pathology, Department of Internal Medicine, Nippon Medical School

We report a case of the extremely rare condition Epstein-Barr virus (EBV)-positive T-cell lymphoproliferative disease (LPD) which occurred after umbilical cord blood transplantation. A 25-year-old Japanese man underwent cord blood transplantation from a male human leukocyte antigen 4/6-matched donor due to acute myeloid leukemia with trisomy 8. Bone marrow examination on day 30 showed chimerism with at least 90% donor cells and complete hematological response. Chronic symptoms of graft-versus-host disease appeared only on the skin and were successfully treated with cyclosporine alone. Three years later, however, the patient experienced repeated cold-like symptoms and was hospitalized with liver dysfunction. A high fever developed and was followed by significant edema of the right side of the face. The EBV DNA copy number in whole peripheral blood was 2×10⁴/mL. Liver biopsy showed invasion of EBV-infected CD8-positive T cells. Southern blotting analysis of the whole peripheral blood showed that the T-cell receptor Cβ1 rearrangement was positive. On the basis of these results, EBV-positive T-cell LPD was diagnosed and treated with prednisolone, cyclosporine, and etoposide, followed by cyclophosphamide, doxorubicin, vincristine, and prednisone. However, the patient died of cardiac function failure, pneumonia, and pulmonary hemorrhage, all of unidentified cause. Most cases of EBV-related LPD after hematopoietic stem cell transplantation consist of EBV-positive B-cell LPD, and, to our knowledge, de novo EBV-positive T-cell LPD subsequent to transplantation has not been previously reported.

J Nippon Med Sch 2016; 83: 35-42

Keywords
Epstein-Barr virus-positive T/natural killer-cell lymphoproliferative disease, CD8-positive T cells, infectious mononucleosis, umbilical cord blood transplantation, multiple organ failure

Correspondence to
Hiroki Yamaguchi, MD, PhD, Department of Hematology, Nippon Medical School, 1-1-5 Sendagi, Bunkyo-ku, Tokyo 113-8603, Japan
y-hiroki@fd6.so-net.ne.jp

Received, June 17, 2015
Accepted, September 25, 2015