Shape-changing agents produce abnormally large platelets in a hereditary "giant platelets syndrome (MPS)".
Shape-changing agents produce abnormally large platelets in a hereditary "giant platelets syndrome (MPS)".
复制标题
变形剂会在遗传性“巨血小板综合症（MPS）”中产生异常大的血小板。
DOI：
10.5555/uri:pii:0022214379901203
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发表时间：
1979
期刊：
影响因子：
--
通讯作者：
M. Frojmovic
中科院分区：
文献类型：
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作者：
J. Milton;M. Frojmovic
比较从正常供体（17）和患有遗传性显性巨血小板综合征（本文称为“蒙特利尔血小板综合征”（MPS））的供体（5）获得的柠檬酸化PRP中血小板的几何形状。测量的几何轴比（rp =厚度/直径）用于将血小板形态分为三组：盘状细胞（rp小于0.5）、盘状棘细胞（rp = 0.5至0.9）、球形棘细胞（rp大于0.9）。MPS椎间盘细胞大小正常;然而，MPS球形棘细胞和椎间盘棘细胞的平均体积约为正常的两倍。研究表明，这些大于正常尺寸的MPS血小板可以通过用已知诱导血小板形状变化的试剂（二磷酸腺苷、凝血酶和4 ℃孵育）处理直接从MPS盘细胞产生。将从正常供体获得的血小板重新悬浮在MPS PPP和ADP中或在4 ℃下孵育，处理这些血小板可导致正常大小的盘状棘细胞和球状棘细胞的形成。MPS盘状棘细胞的直径与外周血涂片上观察到的MPS血小板直径相同，而MPS球形棘细胞的直径约低20%。这表明MPS中异常大血小板的出现与在形状改变期间调节血小板大小和形状的机制中的缺陷有关。
Geometries of platelets in citrated PRP obtained from normal donors (17) and donors (5) with a hereditary dominant giant platelet syndrome, herein referred to as "Montreal platelet syndrome" (MPS), are compared. The measured geometric axial ratio (rp = thickness/diameter) is used to classify platelet morphologies into three groups: discocytes (rp less than 0.5), disco-echinocytes (rp = 0.5 to 0.9), sphero-echinocytes (rp greater than 0.9). MPS discocytes are normal sized; however, MPS sphero-echinocytes and disco-echinocytes have mean volumes approximately two times larger than normal. It is demonstrated that these larger-than-normal sized MPS platelets can be produced directly from MPS discocytes by treatment with agents known to induce platelet shape change (adenosine diphosphate, thrombin, and incubation at 4 degrees C). Treatment of platelets obtained from normal donors which have been resuspended in MPS PPP and ADP or incubation at 4 degrees C causes the formation of normal-sized disco-echinocytes and sphero-echinocytes. The diameters of MPS disco-echinocytes are identical to the diameters of MPS platelets observed on peripheral blood smear, whereas those of MPS sphero-echinocytes are approximately 20% lower. It is suggested that the appearance of abnormally large platelets in MPS is related to a defect in the mechanism which regulates platelet size and shape during shape change.