A correlation of erythrokinetics, ineffective erythropoiesis, and erythroid precursor apoptosis in Thai patients with thalassemia

ชื่อเรื่อง

A correlation of erythrokinetics, ineffective erythropoiesis, and erythroid precursor apoptosis in Thai patients with thalassemia See all items with this value

ผู้สร้าง/เจ้าของผลงาน

Pootrakul, Pensri
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Sirankapracha, Pornpan
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Hemsorach, Surai
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Moungsub, Wanna
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Kumbunlue, Rawiprapa
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Piangitjagum, Anong
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Wasi, Prawase
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Ma, Lisa
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Schrier, Stanley L. See all items with this value

หัวเรื่อง

Red Cells See all items with this value

Thalassemia See all items with this value

คำอธิบายรายละเอียดของเอกสาร

This article was presented in part at the XXI Congress of the International Society of Hematology, Sydney, Australia, May 1985, and the Annual Meeting of the American Society of Hematology, San Diego, CA, December 1997.

บทคัดย่อ

The variety of patients with thalassemia in Thailand offers an opportunity to fully characterize the kinetic causes of the anemia and to study apoptosis of marrow erythroid precursors as a possible factor contributing to its severity. Kinetic studies showed that in hemoglobin H (HbH) disease, the extent of hemolysis, as well as the minimally ineffective erythropoiesis, usually falls within the compensatory capacity of normal erythropoiesis; therefore, anemia in patients with HbH partly represents a failure to expand erythropoiesis adequately. Hemoglobin Constant Spring (HbCS), a common variant of α thalassemia in Bangkok, causes more severe hemolysis and a distinct increase in ineffective erythropoiesis. Ineffective erythropoiesis plays a much more prominent role in β thalassemia/hemoglobin E (β-thal/HbE) disease, in which the variability of the anemia is puzzling. We compared mild and severe cases and found that patients with severe disease had a maximal marrow erythropoietic response that failed to compensate for very short survival of red blood cells and a marked quantitative increase in ineffective erythropoiesis. Analysis of apoptosis of marrow erythroid precursors done both on shipped samples and in Bangkok showed a moderate increase in HbH disease, consistent with the small increase in ineffective erythropoiesis. In patients with homozygous HbCS, there was a further increase in apoptosis, consistent with the additional increase in ineffective erythropoiesis. Patients with β-thal/HbE disease had the most ineffective erythropoiesis and the most erythroid apoptosis. Thus, it appears that α-chain deposition in erythroid precursors, either αA or αcs, leads to accelerated apoptosis and ineffective erythropoiesis.

ผู้ร่วมงาน

Thalassemia Research Center, Institute of Sciences and Technology for Research and Development, Mahidol University See all items with this value

Faculty of Medicine, Siriraj Hospital, Mahidol University See all items with this value

Faculty of Tropical Medicine, Mahidol University See all items with this value

Division of Hematology, Department of Medicine, Stanford University School of Medicine, Stanford, CA. See all items with this value

ปีที่ผลิตเอกสาร

2000 See all items with this value

วันที่บันทึกข้อมูล

2023-05-03

ประเภท

Article See all items with this value

รูปแบบ

pdf See all items with this value

ขนาดหรือจำนวนของเอกสาร

7 pages

ต้นฉบับ/แหล่งที่มาของเอกสาร

Blood, Vol. 96, No. 7, Page 2606-2612

ภาษา

en See all items with this value

รหัส หรือตัวบ่งชี้เอกสาร

https://doi.org/10.1182/blood.V96.7.2606 See all items with this value

ขอบเขต

Health See all items with this value

เจ้าของลิขสิทธิ์

American Society of Hematology See all items with this value

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