高危家庭阴燃型ATLL及家族性乙型肝炎病毒双重感染1例

Y Sawada, Y Nitta, T Ikeya, O Daimaru
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摘要

鹿儿岛县一名35岁男性于1986年4月在双上眼睑、双手和右腿上出现结节并无症状红斑。1987年4月10日,皮肤病变活检显示大量非典型CD4阳性淋巴细胞侵入血管周围的真皮层。血清分析显示人T淋巴嗜病毒I型(HTLV-I)抗体效价(X40)阳性,外周血可见花细胞;但未见骨髓或淋巴结浸润。来自外周血和右腿皮肤病变的淋巴细胞显示HTLV-I前病毒DNA的单克隆整合。慢性活动性乙型肝炎病毒(HBV)也是一个复杂因素。家族性研究显示,所有三名家庭成员都感染了HTLV-1和HBV。HTLV-I的前病毒整合在3例中有2例为中间型。根据结果,诊断为阴燃成人T细胞白血病淋巴瘤(ATLL),该患者对家庭成员具有高风险。在此,我们讨论该病例ATLL的发展和家庭成员之间的感染。
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[A case of smoldering ATLL in high risk family and familial double infection of HTLV-1 and HBV].

A 35-year-old male from Kagoshima prefecture developed nodules with asymptomatic erythema on both upper eyelids, both hands and upper right leg in April 1986. On April 10, 1987, biopsy of skin lesions revealed numerous atypical CD4 positive lymphocytes which had invaded the dermis around the vessels. Analysis of serum showed positive antibody titer (X40) against the human T lymphotropic virus type I (HTLV-I) with some flower cells evident in peripheral blood; however, no infiltration into bone marrow or lymph nodes could be seen. Lymphocytes from peripheral blood and from the skin lesion of the right leg showed monoclonal integration of HTLV-I proviral DNA. Chronic active hepatitis B virus (HBV) was also a complicating factor. Familial study revealed all three family members to be infected with HTLV-1 as well as HBV. Proviral integration of HTLV-I was the intermediate type in two out of the three. From the results, a diagnosis of smoldering adult T cell leukemia-lymphoma (ATLL) was made in this patient with high risk to family members. Herein we discuss the development of ATLL in this case and infection among family members.

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