Matutes E, Spittle M F, Smith N P, Eady R A, Catovsky D
Academic Department of Haematology and Cytogenetics, Royal Marsden Hospital, London.
Br J Haematol. 1995 Mar;89(3):615-9. doi: 10.1111/j.1365-2141.1995.tb08371.x.
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 Caribbean only once. Both patients were HTLV-1 seropositive, as was their mother; their father and brother were negative. The older sibling had the lymphoma form of ATLL, whilst the younger had chronic ATLL. The former was unresponsive to chemotherapy and died of progressive disease; the 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 from mother to child and documents the development of ATLL in second-generation Caribbean immigrants.
我们描述了两名在4年内患上成人T细胞白血病淋巴瘤(ATLL)的兄弟姐妹。两人都是非洲加勒比裔黑人,但其中一人出生在英国,只去过一次加勒比地区。两名患者均为HTLV-1血清阳性,他们的母亲也是如此;他们的父亲和兄弟为阴性。年长的兄弟姐妹患的是ATLL淋巴瘤型,而年幼的患的是慢性ATLL。前者对化疗无反应,死于疾病进展;后者对各种治疗有短暂反应,就诊5年后仍存活。免疫表型分析显示为CD4+、CD25+表型;Southern印迹法证明HTLV-I在受累组织中存在单克隆整合。这份关于英国首例家族性ATLL的报告支持了HTLV-I从母亲传给孩子的观点,并记录了第二代加勒比移民中ATLL的发病情况。