       Document 0461
 DOCN  M9640461
 TI    Immunoblastic transformation of a Sezary syndrome in a black Caribbean
       patient without evidence of HTLV-I.
 DT    9604
 AU    Matutes E; Schulz T; Dyer M; Ellis J; Hedges M; Catovsky D; Academic
       Department of Haematology and Cytogenetics, Royal; Marsden Hospital,
       London, U.K.
 SO    Leuk Lymphoma. 1995 Aug;18(5-6):521-7. Unique Identifier : AIDSLINE
       MED/96116896
 AB    We describe an unusual case of Sezary syndrome which transformed into a
       large T-cell non Hodgkin's lymphoma (immunoblastic) in a black man of
       Caribbean descent with negative HTLV-I serology and no evidence of
       HTLV-I infection by DNA analysis using sensitive techniques. The disease
       presented as a small-cell Sezary syndrome and transformed in an inguinal
       lymph node one year from diagnosis. Immunological markers in the small
       and large cells showed a mature T-cell phenotype CD4+, CD8- with
       expression of T-cell activation markers and a high proliferative rate.
       Ultrastructural analysis confirmed small Sezary cells with serpentine
       nucleus in the peripheral blood and immunoblasts in the lymph node.
       Cytogenetics demonstrated complex clonal chromosome abnormalities with
       involvement of 7q35, the locus for the beta chain of the T-cell receptor
       (TCR). Southern-blot analysis showed the same rearrangement of the TCR
       beta, gamma, delta chain genes in lymph node and peripheral blood cells.
       Antibodies to HTLV-I were not detected in the serum by ELISA and
       particle agglutination (PA) nor HTLV-I specific sequences were
       demonstrated by nested polymerase chain reaction with primers to the
       envelope proteins, LTR and tax/rex of HTLV-I in both tissues, blood and
       lymph node. The disease had an aggressive course and was refractory to
       therapy; the patient died of progressive disease 28 months from
       presentation. Two unusual features characterised this patient's illness:
       immunoblastic transformation of a Sezary syndrome in a patient of
       Afro-Caribbean origin without evidence of HTLV-I DNA sequences and
       negative HTLV-I serology and the atypical lymph node histology
       resembling ATLL.
 DE    Base Sequence  Case Report  Chromosome Abnormalities/PATHOLOGY
       Chromosome Banding  DNA Primers/CHEMISTRY  DNA, Viral/ANALYSIS  Gene
       Rearrangement, T-Lymphocyte  Genes, myc  Human  HTLV-I/GENETICS
       Immunophenotyping  Jamaica/ETHNOLOGY  Lymphoma,
       Lymphoblastic/*MICROBIOLOGY/PATHOLOGY  Lymphoma,
       T-Cell/*MICROBIOLOGY/PATHOLOGY  Male  Middle Age  Molecular Sequence
       Data  Sezary Syndrome/*PATHOLOGY  JOURNAL ARTICLE

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

