Slappendel R J, Renooij W, de Bruijne J J
Department of Clinical Sciences of Companion Animals, Faculty of Veterinary Medicine, University of Utrecht, The Netherlands.
Blood. 1994 Aug 1;84(3):904-9.
Examination of the red blood cells (RBCs) of eight dogs with familial stomatocytosis-hypertrophic gastritis (FS-HG), a multiorgan disease associated with hemolytic anemia, hereditary stomatocytosis (HSt), and hypertrophic gastritis resembling Ménétrier's disease in man, showed abnormal osmotic fragility, normal mean corpuscular volume, slightly increased cell water, and normal cation content and cation fluxes. Cholesterol was decreased in RBC and increased in plasma. In both RBCs and plasma, total phospholipid (PL) was normal, phosphatidylcholine (PC) decreased, and sphingomyelin increased. The palmitic acid content of PC was increased, and the stearic acid content of PC was decreased. Sodium dodecyl sulfate electrophoresis of RBC membrane proteins was normal. These findings have not been described previously in HSt. They suggest that in FS-HG, abnormal composition of the PL in RBCs secondary to abnormal PL in plasma causes defective membrane function and stomatocytic shape-change. This conclusion was supported by a shortened half-life of 51Cr-labeled RBCs from normal dogs after transfusion in dogs with FS-HG. It was concluded (1) that not all hereditary forms of stomatocytosis are necessarily associated with an intrinsic structural defect of the RBC membrane, but that the change in shape of RBC may also be induced by abnormal composition of the plasma; (2) that stomatocytosis may be caused by loss of membrane surface area rather than by the increased cation uptake such as has been shown in some human kindreds with HSt, (3) that FS-HG is a disorder of lipid metabolism, and by consequence, (4) that abnormal lipid metabolism might be involved in the pathogenesis of Ménétrier's disease.
对八只患有家族性口形细胞增多症 - 肥厚性胃炎（FS - HG）的犬进行红细胞（RBC）检查，该多器官疾病与溶血性贫血、遗传性口形细胞增多症（HSt）以及类似人类梅内特里耶病的肥厚性胃炎相关。检查发现红细胞渗透脆性异常、平均红细胞体积正常、细胞内水分略有增加、阳离子含量及阳离子通量正常。红细胞中胆固醇降低，血浆中胆固醇升高。红细胞和血浆中的总磷脂（PL）均正常，磷脂酰胆碱（PC）降低，鞘磷脂增加。PC中的棕榈酸含量增加，硬脂酸含量降低。红细胞膜蛋白的十二烷基硫酸钠电泳正常。这些发现此前在HSt中未曾描述过。它们表明，在FS - HG中，血浆中PL异常导致红细胞中PL组成异常，进而引起膜功能缺陷和口形细胞形态改变。将正常犬的51Cr标记红细胞输给患有FS - HG的犬后，其半衰期缩短，这支持了该结论。得出的结论是：（1）并非所有遗传性口形细胞增多症都必然与红细胞膜的内在结构缺陷相关，红细胞形态的改变也可能由血浆成分异常引起；（2）口形细胞增多症可能是由于膜表面积减少所致，而非像某些人类HSt家系中所显示的那样由阳离子摄取增加引起；（3）FS - HG是一种脂质代谢紊乱疾病，因此，（4）异常脂质代谢可能参与了梅内特里耶病的发病机制。
