T-.GAMMA. .DELTA. 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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- 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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- Yamashita Koji
- 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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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.<br>
収録刊行物
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- Internal Medicine
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Internal Medicine 45 (9), 631-635, 2006
一般社団法人 日本内科学会
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詳細情報 詳細情報について
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- CRID
- 1390001204873340032
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- NII論文ID
- 130000076774
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- ISSN
- 13497235
- 09182918
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- 本文言語コード
- en
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- データソース種別
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- JaLC
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- 使用不可