Combe B, Andary M, Baldet P, Caraux J, Clot J, Sany J
Rev Rhum Mal Osteoartic. 1987 May;54(5):381-7.
The authors have studied the case of a female patient with rheumatoid polyarthritis, who developed a lymphocytic proliferation in the blood, the marrow, and the liver, associated with a neutropenia. Several similar cases have been recently reported in the literature. The cellular proliferation is made of large granulous lymphocytes and the study of membrane markers enables to find the following homogeneous phenotype: E rosette+, CD8+, HNK-1+, FcR+, CD4-luminal diameter "divided by degrees - -, IgS-, HLA class II-. This lymphocytic sub-population produces little interleukin-2, responds weakly to mitogens (PHA, CON A, PWM), and inhibits the response of normal lymphocytes to the same mitogens. These lymphocytes have a weak natural killer activity but, on the contrary, develop a very strong cytotoxic activity which is antibody-dependent. Clinically, splenomegaly, anemia and infections are frequent and hepatomegaly or thrombopenia more rare. Adenopathies are never present. The evolution is chronic in nature and not very aggressive, although the lymphocytic proliferation is monoclonal in origin, as demonstrated in molecular biology studies. The neutropenia might be secondary to an inhibiting effect of lymphocytes on the granular precursors of the bone marrow. There is a definite association between this lympho-proliferative syndrome and rheumatoid polyarthritis, and this association appears to be different from the Felty's syndrome.
作者研究了一名患有类风湿性多关节炎的女性患者的病例,该患者血液、骨髓和肝脏出现淋巴细胞增殖,并伴有中性粒细胞减少。最近文献中报道了几例类似病例。细胞增殖由大颗粒淋巴细胞构成,对膜标志物的研究发现其具有以下均一表型:E 玫瑰花结阳性、CD8 阳性、HNK - 1 阳性、FcR 阳性、CD4 阴性、管腔直径“除以度数 - -”、IgS 阴性、HLA 二类阴性。这种淋巴细胞亚群产生少量白细胞介素 - 2,对有丝分裂原(PHA、CON A、PWM)反应较弱,并抑制正常淋巴细胞对相同有丝分裂原的反应。这些淋巴细胞自然杀伤活性较弱,但相反,具有非常强的细胞毒性活性,且该活性依赖抗体。临床上,脾肿大、贫血和感染较为常见,肝肿大或血小板减少则较为少见。从不出现淋巴结病。尽管分子生物学研究表明淋巴细胞增殖起源于单克隆,但病程本质上是慢性的,且侵袭性不强。中性粒细胞减少可能继发于淋巴细胞对骨髓粒细胞前体的抑制作用。这种淋巴细胞增殖综合征与类风湿性多关节炎之间存在明确关联,且这种关联似乎与费尔蒂综合征不同。