T-γ δ Large Granular Lymphocyte Leukemia Preceded by Pure Red Cell Aplasia and Complicated with Hemophagocytic Syndrome Caused by Epstein-Barr Virus Infection
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概要
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A 51-year-old man developed anemia, and was diagnosed with pure red cell aplasia through the absence of erythroid progenitors. Initially, he was treated with cyclosporine and prednisolone for 6 months but they were ineffective. Large granular lymphocyte (LGL) leukemia with the T-cell γ δ phenotype evolved after 6 months showing CD2+, CD3+, CD8- and CD56- with the T-cell receptor β gene rearrangement, clonalities of γ and δ genes and complex chromosome abnormality simultaneously with hemophagocytic syndrome (HPS). Epstein-Barr virus (EBV) genomic DNA was detected in the bone marrow cells. Administration of bolus methylprednisolone was ineffective, and the patient died one month later. In the present patient, it seemed that lymphoproliferative disease of large granular lymphocytes (LDGL) manifested initially as PRCA, γδ LGL leukemia evolved, and finally fatal HPS become complicated, presumably caused by the EBV reactivation in the immunodeficiency state with the administration of immunosuppressants.
著者
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Yamashita Koji
Department of Vascular Biology, Institute of Brain Science, Hirosaki University Graduate School of M
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Tanaka Yoshinori
Department of Medicine, Yamaguchi Prefectural Medical Center
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Matsui Kumiko
Department of Medicine, Yamaguchi Prefectural Medical Center
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Matsuda Kazuhiro
Department of Medicine, Yamaguchi Prefectural Medical Center
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Shinohara Kenji
Department of Medicine, Yamaguchi Prefectural Medical Center
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Matsutani Akira
Department of Medicine, Shunan City Shinnanyo Hospital
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Matsui Kumiko
Department Of Anesthesiology Showa University Fujigaoka Hospital
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Tanaka Yoshinori
Department Of Bacteriology Faculty Of Medicine Tottori University
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Yamashita Koji
Department Of Clinical Radiology Graduate School Of Medical Sciences Kyushu University
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Shinohara Kenji
Department Of Applied Chemistry Graduate School Of Engineering Kyushu University
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Matsuda Kazuhiro
Department Of Agronomy
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Yamashita Koji
Department of Medicine, Yamaguchi Prefectural Medical Center
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