Cartier L, Castillo J L, Cabrera M E, Araya F, Matutes E, Ford A M, Greaves M F
Department of Neurology, Faculty of Medicine, University of Chile, Santiago.
Leuk Lymphoma. 1995 May;17(5-6):459-64. doi: 10.3109/10428199509056858.
We describe the clinical and laboratory features in three Caucasian Chilean patients with tropical spastic paraparesis (TSP) associated with/or preceded by a lymphoproliferative disorder involving cutaneous lesions and localised lymphadenopathy. The neurological symptoms and signs were characteristic of TSP and CSF examination revealed the presence of oligoclonal bands. All three patients had a moderate leucocytosis (10-14 x 10(9)/l) with eosinophilia and a minority (2-4%) of circulating atypical polylobed or ATLL-like lymphocytes. Lymph node histology showed a diffuse pattern of infiltration (1 case) and marked expansion of the paracortical zone with convoluted lymphocytes and immunoblasts (2 cases). Skin biopsy demonstrated a dermal lymphoid infiltration with epidermotropism. Antibodies to HTLV-I were detected in the serum and CSF in the three patients and Southern blot analysis of peripheral blood mononuclear cells showed a monoclonal integration of HTLV-I proviral DNA in one case whereas in the two others the pattern was indicative of low level polyclonal integration. All three patients were treated with prednisolone and one with PUVA with transient partial response on the skin and neurological manifestations. Two patients died months to 5 years from presentation and the other is alive 12 years from diagnosis with active neurological and skin disease. The simultaneous occurrence of HTLV-I associated TSP with smouldering ATLL and a cutaneous ATLL or pre-leukaemic form is discussed.
我们描述了三名患有热带痉挛性截瘫（TSP）的智利白种人患者的临床和实验室特征，这些患者伴有/或在出现涉及皮肤病变和局部淋巴结病的淋巴增殖性疾病之前发病。神经症状和体征具有TSP的特征，脑脊液检查显示存在寡克隆带。所有三名患者均有中度白细胞增多（10 - 14×10⁹/L）伴嗜酸性粒细胞增多，以及少数（2 - 4%）循环中的非典型多叶核或成人T细胞白血病样淋巴细胞。淋巴结组织学显示弥漫性浸润模式（1例），以及副皮质区明显扩张，伴有卷曲淋巴细胞和免疫母细胞（2例）。皮肤活检显示真皮淋巴细胞浸润伴亲表皮现象。在三名患者的血清和脑脊液中均检测到抗HTLV - I抗体，外周血单个核细胞的Southern印迹分析显示，1例患者中HTLV - I前病毒DNA呈单克隆整合，而另外2例的模式表明为低水平多克隆整合。所有三名患者均接受泼尼松龙治疗，1例接受补骨脂素加紫外线A光疗，皮肤和神经表现有短暂部分缓解。两名患者在出现症状后数月至5年死亡，另一名患者自诊断后12年仍存活，有活动性神经和皮肤疾病。本文讨论了HTLV - I相关TSP与隐匿性成人T细胞白血病和皮肤成人T细胞白血病或白血病前期形式同时发生的情况。
