Winkelmann R K, Buechner S A, Diaz-Perez J L
J Am Acad Dermatol. 1984 Jun;10(6):992-9. doi: 10.1016/s0190-9622(84)80321-7.
Eighteen patients with erythroderma, recurrent cycles of circulating Sézary cells of less than 1,000 cells/mm3, and a chronic course were followed for a mean time of nearly 5 years and were diagnosed as having pre-Sézary syndrome. Only one patient died, and none developed lymphoproliferative disease. All ten patients who underwent patch testing showed positive results. The elevation of IgE was striking when this group was compared with a group with Sézary syndrome. Most patients achieved partial or complete remission on low-dose chlorambucil and prednisone therapy. Some patients had lymphocytic or lymphomatoid bands on skin biopsy specimens and were like previously reported patients with pre-Sézary syndrome whose condition progressed to Sézary syndrome. A nontoxic chemotherapy or an anti-T cell treatment program can control this chronic erythroderma state.
18例患有红皮病、循环Sezary细胞反复出现周期且细胞数少于1000个/mm³、病程呈慢性的患者被随访了近5年，被诊断为Sezary综合征前期。仅1例患者死亡，且无人发展为淋巴增殖性疾病。所有接受斑贴试验的10例患者结果均为阳性。与Sezary综合征组相比，该组患者IgE升高显著。大多数患者在低剂量苯丁酸氮芥和泼尼松治疗下实现了部分或完全缓解。一些患者的皮肤活检标本上有淋巴细胞或淋巴瘤样带，类似于先前报道的病情进展为Sezary综合征的Sezary综合征前期患者。一种无毒化疗或抗T细胞治疗方案可以控制这种慢性红皮病状态。
