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Hemoglobin
international journal for hemoglobin research
Volume 2, 1978 - Issue 3
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Original Article

Hemoglobin Legnano (α2 141 (HC3) Arg → Leu β2): A New Abnormal Human Hemoglobin with High Oxygen Affinity

F. Mavilio Laboratorio di Patologia non Infettiva, Istituto Superiore di Sanità, Rome, Italy
,
M. Marinucci Laboratorio di Patologia non Infettiva, Istituto Superiore di Sanità, Rome, Italy
,
L. Tentori Laboratorio di Patologia non Infettiva, Istituto Superiore di Sanità, Rome, Italy
,
P. P. Fontanarosa Laboratorio di Patologia non Infettiva, Istituto Superiore di Sanità, Rome, Italy
,
U. Rossi Servizio di Ematologia e Centro Trasfusionale, Ospedale Generale Provinciale, Legnano, Italy
&
S. Biagiotti Servizio di Ematologia e Centro Trasfusionale, Ospedale Generale Provinciale, Legnano, Italy
Pages 249-259 | Received 06 Dec 1977, Accepted 28 Feb 1978, Published online: 07 Jul 2009
 
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Abstract

An abnormal, fast moving hemoglobin was noted in a 34-yr-old male patient living in Legnano (northern Italy) affected with renal failure and iron deficiency anemia, not related to the presence of the Hb variant. Structural studies have demonstrated a previously undescribed amino acid substitution, α 141 Arg → Leu. This new variant has been named Hb Legnano, and is characterized by an increased oxygen affinity and a low cooperativity, at least as far as preliminary functional studies carried out on whole blood have indicated. Family studies are reported: three other heterozygous carriers were observed among the relatives of the propositus, all showing a mild polycythemia which, however, does not seem to produce appreciable clinical consequences.

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