Immunoblastic transformation of a Sezary syndrome in a black Caribbean patient without evidence of HTLV-I.
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.
Academic Department of Haematology and Cytogenetics, Royal Marsden Hospital, London, U.K.
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E
Matutes
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Schulz
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Dyer
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Ellis
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Hedges
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Catovsky
D001483:Base Sequence; D002869:Chromosome Aberrations / Q000473:pathology; D002871:Chromosome Banding; D025063:Chromosome Disorders; D017931:DNA Primers / Q000737:chemistry; D004279:DNA, Viral / Q000032:analysis; D015329:Gene Rearrangement, T-Lymphocyte; D016259:Genes, myc; D015368:Human T-lymphotropic virus 1 / Q000235:genetics; D006801:Humans; D016130:Immunophenotyping; D007563:Jamaica / Q000208:ethnology; D016399:Lymphoma, T-Cell / Q000382:microbiology* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D008969:Molecular Sequence Data; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000382:microbiology* / Q000473:pathology; D012751:Sezary Syndrome / Q000473:pathology*
D017931:DNA Primers; D004279:DNA, Viral