Pathology of Abnormal Hemoglobin Diseases Seen in Thailand
ชื่อเรื่อง
Pathology of Abnormal Hemoglobin Diseases Seen in Thailand
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ผู้สร้าง/เจ้าของผลงาน
Bhamarapravati, Natth
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Na-Nakorn, Supa
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Wasi, Prawase
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Tuchinda, Soodsakorn
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หัวเรื่อง
Hemoglobin
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บทคัดย่อ
The clinical picture of β-thalassaemia haemoglobin E disease resembles that of thalassaemia major. The present paper is based on post-mortem protocols and tissue sections from 20 patients who had died from β-thalassaemia haemoglobin E disease seen at the Siriraj Hospital Medical College in Bangkok. The post mortems were performed between 1961 and 1966. 2 cases of thalassaemia major and 4 of haemoglobin H disease were also available for comparison. In each case the final diagnosis was made before death. The patients were aged between 1 and 38 years, 12 were female and 8 male. The pathological findings could be divided into two categories, those which could be associated with haemolytic anaemia as such, and those which needed further clarification. To the first type belonged the increased deposits of iron in the tissues, the increase in intra- and extramedullary haematopoietic tissue and other non-specific changes which could be related to prolonged tissue anoxia. The increased deposits of iron in the tissues were most frequent in the parenchymatous organs rather than in the reticulo-endothelial tissue and the pattern of distribution resembled therefore that of haemochromatosis and not that found in the haemosiderosis following frequent blood transfusion. There was no evidence of tissue damage due to iron deposits. The presence and the degree of extramedullary haematopoiesis in liver, spleen and lymph nodes could not be correlated with the degree of anaemia, the age of the patients, or even the extent of tissue damage. A significant observation in all patients was a progressive liver damage, however, this has to be assessed in the light of a high incidence of cirrhosis and carcinoma of the liver in the same population. Nevertheless, whereas in a general analysis in Thailand the type of cirrhosis is postnecrotic and the average age at death is between 40 and 50, in the present series the cirrhosis was monolobular and of the septal type, and the average age of the patients in whom this cirrhosis was fully developed was only 20 years. Liver cell carcinoma occurred in one patient aged 9 years, whereas the average age of death from liver cell carcinoma in the general Thai population is 47 years. There was also an increase in lipochrome pigment in the visceral organs and in smooth muscle. The histological appearances are illustrated in 20 photomicrographs. H. Lehmann.
ผู้ร่วมงาน
Departments of Pathology, Medicine, and Pediatrics, Siriraj Hospital Medical College, Bangkok, Thailand
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ปีที่ผลิตเอกสาร
วันที่บันทึกข้อมูล
2023-04-27
ประเภท
Article
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รูปแบบ
ขนาดหรือจำนวนของเอกสาร
14 Pages
ต้นฉบับ/แหล่งที่มาของเอกสาร
American Journal of Clinical Pathology, Volume 47, Issue 6, 1 June 1967, Pages 745–758
ภาษา
รหัส หรือตัวบ่งชี้เอกสาร
ขอบเขต
เจ้าของลิขสิทธิ์
Oxford University Press
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