       Document 0132
 DOCN  M95A0132
 TI    Hepatosinusoidal leukaemia/lymphoma consisting of Epstein-Barr
       virus-containing natural killer cell leukaemia/lymphoma and T-cell
       lymphoma; mimicking malignant histiocytosis.
 DT    9510
 AU    Ohshima K; Kikuchi M; Mizuno S; Akashi K; Moriyama K; Yoneda S;
       Takeshita M; Shibata T; Department of Pathology, School of Medicine,
       Fukuoka University,; Japan.
 SO    Hematol Oncol. 1995 Mar-Apr;13(2):83-97. Unique Identifier : AIDSLINE
       MED/95317736
 AB    Previously diagnosed cases of hepatosinusoidal T-cell lymphoma and
       malignant histiocytosis (MH) may include lymphoid neoplasms of natural
       killer (NK) cell lineage associated with Epstein-Barr virus (EBV). Such
       hepatosinusoidal neoplasms were found to demonstrate hepatomegaly but
       not lymphadenopathy, and all were diagnosed by a liver biopsy. Sixteen
       adult patients diagnosed with hepatosinusoidal leukaemia/lymphoma (six
       NK-cell leukaemia/lymphomas [NKLLs], five instances of MH, three T-cell
       malignant lymphomas [T-MLs], and two adult T-cell leukaemia/lymphomas
       [ATLLs] were examined for EBV by in situ hybridization, then were
       studied immunohistochemically and subjected to a DNA analysis. Among our
       five patients with MH, neoplastic cells showed T-cells, but no
       histiocytic markers, and they were considered to have either a T-cell or
       NK-cell lineage. All NKLLs, MHs and T-MLs, except for ATLLs accompanied
       by reactive hemophagocytic histiocytes, varied in number in each case.
       In situ hybridization revealed the presence of EBV in the nuclei of
       atypical cells in all of the six lymphoid neoplasms of NK-cell lineage.
       Each case of MH and each T-ML which represented EBV demonstrated no
       definite T-cell or histiocytic markers. Patients with ATLL did not
       reveal EBV. In all patients with hemophagocytosis, EBV was present in
       the nuclei of the neoplastic lymphocytes, but not in the hemophagocytic
       cells. Finally, the 16 cases were reclassified into eight cases with
       EBV-containing NKLLs, six T-MLs, and two ATLLs. In addition, no true
       histiocytic neoplasms were observed. The mechanism of hemophagocytosis
       may be therefore the production of lymphokines (macrophage-activating
       factors) by neoplastic lymphocytes. EBV-associated hepatosinusoidal
       leukaemia/lymphoma may thus contain a lymphoid neoplasm of NK-cell
       lineage, which made it difficult to be distinguished from the previously
       designated malignant histiocytosis.
 DE    Adult  Base Sequence  Biopsy  Diagnosis, Differential  Female  Genotype
       Herpesviridae Infections/PATHOLOGY/*VIROLOGY  *Herpesvirus 4, Human
       Histiocytosis, Malignant/*DIAGNOSIS/*PATHOLOGY  Human
       Immunohistochemistry  Killer Cells, Natural/*PATHOLOGY/*VIROLOGY
       Leukemia-Lymphoma, T-Cell, Acute, HTLV-I-Associated/*DIAGNOSIS/
       PATHOLOGY/*VIROLOGY  Liver Neoplasms/*DIAGNOSIS/*PATHOLOGY  Lymphoma,
       T-Cell/*DIAGNOSIS/*PATHOLOGY  Male  Molecular Sequence Data  Phenotype
       Support, Non-U.S. Gov't  Tumor Virus Infections/PATHOLOGY/*VIROLOGY
       JOURNAL ARTICLE

       SOURCE: National Library of Medicine.  NOTICE: This material may be
       protected by Copyright Law (Title 17, U.S.Code).

