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具有不寻常临床、形态学、超微结构和膜表面标志物特征的慢性淋巴细胞增殖性疾病。

Chronic lymphoproliferative disorder with unusual clinical, morphologic, ultrastructural and membrane surface marker characteristics.

作者信息

McKenna R W, Parkin J, Kersey J H, Gajl-Peczalska K J, Peterson L, Brunning R D

出版信息

Am J Med. 1977 Apr;62(4):588-96. doi: 10.1016/0002-9343(77)90422-3.

DOI:10.1016/0002-9343(77)90422-3
PMID:192076
Abstract

Four of 105 patients with chronic lymphocytic leukemia (CLL) manifested clinical, morphologic, ultrastructural and membrane surface marker characteristics that differed from those found in patients with typical CLL of demonstrated B-lymphocyte origin. These four patients presented with moderate increases in absolute lymphocyte counts, absolute neutropenia, polyclonal hypergammaglobulinemia and hepatosplenomegaly without lymphadenopathy. Two of them were unusually young, 19 and 25 years old, at the time of diagnosis. The proliferating lymphocytes carried receptors for sheep erythrocytes, a T-lymphocyte marker. In the three patients tested, the lymphocytes also carried Fc receptors. Ultrastructurally the lymphocytes contained cytoplasmic inclusion bodies consisting of parallel tubular arrays. The parallel tubular arrays corresponded to prominent cytoplasmic azurophilic granules on light microscopy. Parallel tubular arrays were found in less than 1 per cent of the lymphocytes in eight patients with typical B-lymphocyte CLL. The process in these four patients may be a distinctive chronic lymphoproliferative disorder originating in T lymphocytes with Fc receptors found in small numbers in the blood of normal persons.

摘要

105例慢性淋巴细胞白血病(CLL)患者中有4例表现出临床、形态学、超微结构和膜表面标志物特征,这些特征与典型的、已证实起源于B淋巴细胞的CLL患者不同。这4例患者表现为绝对淋巴细胞计数中度增加、绝对中性粒细胞减少、多克隆高丙种球蛋白血症和肝脾肿大,但无淋巴结病。其中2例在诊断时异常年轻,分别为19岁和25岁。增殖的淋巴细胞带有绵羊红细胞受体,这是一种T淋巴细胞标志物。在接受检测的3例患者中,淋巴细胞还带有Fc受体。超微结构上,淋巴细胞含有由平行管状排列组成的胞质包涵体。在光学显微镜下,平行管状排列对应于显著的胞质嗜天青颗粒。在8例典型B淋巴细胞CLL患者中,不到1%的淋巴细胞中发现了平行管状排列。这4例患者的病情可能是一种独特的慢性淋巴细胞增殖性疾病,起源于T淋巴细胞,Fc受体在正常人血液中少量存在。

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