Biometrically revealed splenic differences between thalassemia, hereditary spherocytosis and elliptocytosis.
Biometrically revealed splenic differences between thalassemia, hereditary spherocytosis and elliptocytosis.
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生物识别揭示了地中海贫血、遗传性球形红细胞增多症和椭圆形红细胞增多症之间的脾脏差异。
DOI：
10.1159/000161643
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发表时间：
1967
期刊：
影响因子：
--
通讯作者：
P. Cavaliere
中科院分区：
文献类型：
--
作者：
C. Bosman;P. Cavaliere
作者检查了9例地中海贫血、6例遗传性球形红细胞增多症和2例遗传性椭圆形红细胞增多症患者的17个脾脏。地中海贫血的患者在生活中接受过输血，但6例遗传性球形红细胞增多症患者中只有1例接受过输血。三种类型的脾脏在组织学上无差异，均较正常脾脏重。在所有三种贫血中，脾脏比重也高于正常。然而，只有在地中海贫血症中，这一点才很明显，因为它等于血液比重的上限。血液的高比重是由红细胞引起的，红细胞的含水量低，血红蛋白富含铁。由于球形红细胞性贫血和椭圆形红细胞增多症患者脾脏中的红细胞数量并不比地中海贫血脾脏中的红细胞数量低得多，因此认为地中海贫血脾脏的比重较高是由于沉积的铁含量高。这方面的证据是存在许多含铁血黄素的巨噬细胞。虽然组织学检查的三种类型的脾脏没有显着差异，红髓体积的绝对增加是地中海贫血症比球形红细胞增多症大得多。另一方面，在球形红细胞增多症中，血窦的体积比例以髓索为代价而增加。地中海贫血症的情况正好相反。在地中海贫血中发现的红髓增生被认为与脾脏分解小细胞和脆性细细胞的功能有关。另一方面，球形红细胞中的血窦有助于脾脏隔离球形红细胞;隔离后，球形红细胞变得更加脆弱，并在脾脏中破裂。莱曼
The authors examined 17 spleens which came from 9 patients with thalassaemia, 6 with hereditary spherocytosis, and 2 with hereditary elliptocytosis. The patients with thalassaemia had received blood transfusion during life but of the 6 patients with hereditary spherocytosis only 1 had received a blood transfusion. There were no differences histologically between the three types of spleen, and all were heavier than normal spleens. The specific spleen weight also was higher than normal in all three anaemias. This was significantly so, however, only in thalassaemia where it was equal to the upper limit of specific weight for blood. The high specific weight of blood is caused by the erythrocytes which have a low water content and the haemoglobin of which is rich in iron. As the numbers of red cells in the spleen in spherocytic anaemia and elliptocytosis are not much lower than those found in the thalassaemic spleen, the higher specific weight of the thalassaemic spleens is thought to be due to the high content of deposited iron. The evidence for this was the presence of many haemosiderin-containing macrophages. Though histological examination of the three types of spleen showed no significant difference, the absolute increases in the volume of red pulp were much greater in thalassaemia than in spherocytosis. On the other hand, in spherocytosis the proportional volume of the sinusoids was increased at the expense of pulp cords. The opposite was the case in thalassaemia. Hyperplasia of the red pulp found in thalassaemia is thought to be associated with the function of the spleen in breaking up microcytes and brittle leptocytes. On the other hand, the sinusoids in spherocytosis assist with the sequestration of spherocytes by the spleen; after sequestration, these become even more brittle and break up in the spleen.H. Lehmann.