E. Matutes et al., THE FIRST REPORT OF FAMILIAL ADULT T-CELL LEUKEMIA-LYMPHOMA IN THE UNITED-KINGDOM, British Journal of Haematology, 89(3), 1995, pp. 615-619
We describe two siblings who developed adult T-cell leukaemia lymphoma
(ATLL) within 4 years. Both were black of Afro-Caribbean extraction,
but one had been born in the United Kingdom and had visited the Caribb
ean only once. Both patients were HTLV-1 seropositive, as was their mo
ther; their father and brother were negative. The older sibling had th
e lymphoma form of ATLL, whilst the younger had chronic ATLL. The form
er was unresponsive to chemotherapy and died of progressive disease; t
he latter experienced transient responses to various treatments and is
alive 5 years after presentation. Immunophenotyping showed a CD4(+),
CD25(+) phenotype; Southern blot demonstrated a monoclonal integration
of HTLV-I in the tissues involved. This report, of the first familial
ATLL in the U.K., supports the suggestion of transmission of HTLV-I f
rom mother to child and documents the development of ATLL in second-ge
neration Caribbean immigrants.