Publication Cover
Hemoglobin
international journal for hemoglobin research
Volume 26, 2002 - Issue 2
108
Views
11
CrossRef citations to date
0
Altmetric
Original

COMPOUND HETEROZYGOSITY FOR α0-THALASSEMIA (−−THAI) AND Hb CONSTANT SPRING CAUSES SEVERE Hb H DISEASE

&
Pages 155-162 | Received 09 Nov 2001, Published online: 07 Jul 2009
 

Abstract

We report two unrelated cases of Hb H-Constant Spring (Hb H-CS) disease caused by a compound heterozygosity for α0-thalassemia (−−THAI deletion) and Hb CS (α142, TAA→CAA) in Thai patients. Hematological and clinical observations in the probands are more severe than those of deletional type of Hb H disease (−−/–α), owing to an early onset of anemia and recurrent episode of anemic crises. These cases also address the importance of the −−THAI deletion which causes a severe clinical phenotype, and that could be missed by routine screening. We suggest that testing for this mutation should be included in the screening program for the prevention and control of thalassemia in Thailand, and possibly in other countries in Southeast Asia, where α0-thalassemias are highly prevalent.

Log in via your institution

Log in to Taylor & Francis Online

PDF download + Online access

  • 48 hours access to article PDF & online version
  • Article PDF can be downloaded
  • Article PDF can be printed
USD 65.00 Add to cart

Issue Purchase

  • 30 days online access to complete issue
  • Article PDFs can be downloaded
  • Article PDFs can be printed
USD 1,628.00 Add to cart

* Local tax will be added as applicable

Related Research

People also read lists articles that other readers of this article have read.

Recommended articles lists articles that we recommend and is powered by our AI driven recommendation engine.

Cited by lists all citing articles based on Crossref citations.
Articles with the Crossref icon will open in a new tab.