[Double negative adult T-cell leukemia with hemophagocytic syndrome].
A 79-year-old woman was admitted with general fatigue, jaundice and hepatosplenomegaly. Perpheral blood examination showed 8.0 g/dl Hb, 15 x 10(3)/microliter platelet and 10,490/microliter leukocytes with 86% abnormal lymphocytes. Immunophenotypic analysis of abnormal lymphocytes demonstrated CD2(+), CD3(+/-), CD4(-), and CD8(-). Serum antibody for HTLV-1 was positive. In addition, the monoclonal integration of HTLV-1 proviral DNA into the genome of leukemic cells was demonstrated on Southern blot hybridization. Bone marrow revealed ATL cell in vasion with myelofibrosis and hemophagocytic cell proliferation. Therefore, adult T-cell leukemia with hemophagocytic syndrome was diagnosed. She was treated with methyl prednisolone pulse therapy and gammaglobulin. But she died of hepatic failure 14 days after hospitalization. On autopsy, EB virus LMP-1 was detected in ATL cells in bone marrow. ATL with hemophagocytosis is relatively rare. The association of both pathological states was discussed.
Masutani
K
K
Division of Internal Medicine, Kyushu Central Hospital.
Fujimaru
M
M
Tsubota
Y
Y
Matsui
N
N
Okamura
T
T
D000368:Aged; D000914:Antibodies, Viral / Q000032:analysis; D015704:CD4 Antigens / Q000032:analysis*; D016827:CD8 Antigens / Q000032:analysis*; D005260:Female; D004854:Herpesvirus 4, Human / Q000276:immunology; D015616:Histiocytosis, Non-Langerhans-Cell / Q000209:etiology*; D006801:Humans; D015458:Leukemia, T-Cell / Q000150:complications / Q000276:immunology*; D013601:T-Lymphocytes / Q000276:immunology*
D000914:Antibodies, Viral; D015704:CD4 Antigens; D016827:CD8 Antigens