A New Abnormal Hemoglobin O Padova, α 30 (B11) Glu → Lys, and a Dyserythropoietic Anemia With Erythroblastic Multinuclearity Coexisting in the Same Patient

A patient with a not previously described abnormal hemoglobin (α30Glu → Lys) and dyserythropoietic anemia with erythroblastic multinuclearity is reported. The heat stability and the functional properties of the new abnormal hemoglobin, named hemoglobin O Padova, are normal, although the replacement...

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Veröffentlicht in:Blood 1974-12, Vol.44 (6), p.869-877
Hauptverfasser: Vettore, L., Sandre, G. De, Iorio, E. E. Di, Winterhalter, K.H., Lang, A., Lehmann, H.
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container_end_page 877
container_issue 6
container_start_page 869
container_title Blood
container_volume 44
creator Vettore, L.
Sandre, G. De
Iorio, E. E. Di
Winterhalter, K.H.
Lang, A.
Lehmann, H.
description A patient with a not previously described abnormal hemoglobin (α30Glu → Lys) and dyserythropoietic anemia with erythroblastic multinuclearity is reported. The heat stability and the functional properties of the new abnormal hemoglobin, named hemoglobin O Padova, are normal, although the replacement lies in the α1ß1 interchain contact. The hemolytic condition, which was much improved by splenectomy, therefore appears to be linked to the hereditary erythroblastic multinuclearity similar to hereditary erythroblastic multinuclearity with positive acidified serum test (HEMPAS). In addition to the leading features observed in published cases of this entity, our case exhibited some immunologic peculiarities.
doi_str_mv 10.1182/blood.V44.6.869.869
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The hemolytic condition, which was much improved by splenectomy, therefore appears to be linked to the hereditary erythroblastic multinuclearity similar to hereditary erythroblastic multinuclearity with positive acidified serum test (HEMPAS). In addition to the leading features observed in published cases of this entity, our case exhibited some immunologic peculiarities.</abstract><cop>United States</cop><pub>Elsevier Inc</pub><pmid>4429803</pmid><doi>10.1182/blood.V44.6.869.869</doi><tpages>9</tpages><oa>free_for_read</oa></addata></record>
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source MEDLINE; EZB-FREE-00999 freely available EZB journals; Alma/SFX Local Collection
subjects Adult
Amino Acids - analysis
Anemia - complications
Bilirubin - blood
Bone Marrow Cells
Cell Nucleus
Cell Survival
Complement System Proteins
Electrophoresis, Starch Gel
Erythrocytes, Abnormal
Female
Fetal Hemoglobin - analysis
Globins - isolation & purification
Heinz Bodies
Hemagglutination Tests
Hemoglobinopathies - complications
Hemoglobins, Abnormal
Hemolysis
Humans
Italy
Osmotic Fragility
Oxygen - analysis
Peptides - analysis
Potassium - blood
Pregnancy
Sodium - blood
Temperature
title A New Abnormal Hemoglobin O Padova, α 30 (B11) Glu → Lys, and a Dyserythropoietic Anemia With Erythroblastic Multinuclearity Coexisting in the Same Patient
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