Abstract
Hemoglobin (Hb) Zürich-Albisrieden (ZA) [α2 59(E8) Gly>Arg; HBA2:c.178G>C] is a rare and highly unstable α-chain variant. A few simple and compound heterozygotes (αZA α/αα and -/αZA α, respectively) have been described so far in Switzerland and China. We describe here a case of homozygosity for the Hb ZA mutation (αZA α/αZA α) in a Brazilian child with severe congenital hemolytic anemia and ineffective erythropoiesis.
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