T-gamma delta large granular lymphocyte leukemia preceded by pure red cell aplasia and complicated with hemophagocytic syndrome caused by Epstein-Barr virus infection.
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 gamma delta phenotype evolved after 6 months showing CD2+, CD3+, CD8- and CD56- with the T-cell receptor beta gene rearrangement, clonalities of gamma and delta 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, gammadelta LGL leukemia evolved, and finally fatal HPS become complicated, presumably caused by the EBV reactivation in the immunodeficiency state with the administration of immunosuppressants.
Tanaka
Yoshinori
Y
Division of Hematology, Department of Medicine, Yamaguchi Prefectural Medical Center, Hofu.
Matsui
Kumiko
K
Yamashita
Koji
K
Matsuda
Kazuhiro
K
Shinohara
Kenji
K
Matsutani
Akira
A
D020031:Epstein-Barr Virus Infections / Q000150:complications*; D017809:Fatal Outcome; D006801:Humans; D007945:Leukemia, Lymphoid / Q000150:complications* / Q000235:genetics / Q000378:metabolism; D008214:Lymphocytes / Q000378:metabolism*; D051359:Lymphohistiocytosis, Hemophagocytic / Q000150:complications* / Q000188:drug therapy / Q000821:virology*; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D008875:Middle Aged; D010641:Phenotype; D016692:Receptors, Antigen, T-Cell, gamma-delta / Q000378:metabolism*; D012010:Red-Cell Aplasia, Pure / Q000150:complications*; D017211:Treatment Failure
D016692:Receptors, Antigen, T-Cell, gamma-delta; D008775:Methylprednisolone