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 |
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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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De ; Iorio, E. E. Di ; Winterhalter, K.H. ; Lang, A. ; Lehmann, H.</creator><creatorcontrib>Vettore, L. ; Sandre, G. De ; Iorio, E. E. Di ; Winterhalter, K.H. ; Lang, A. ; Lehmann, H.</creatorcontrib><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.</description><identifier>ISSN: 0006-4971</identifier><identifier>EISSN: 1528-0020</identifier><identifier>DOI: 10.1182/blood.V44.6.869.869</identifier><identifier>PMID: 4429803</identifier><language>eng</language><publisher>United States: Elsevier Inc</publisher><subject>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</subject><ispartof>Blood, 1974-12, Vol.44 (6), p.869-877</ispartof><rights>1974 American Society of Hematology</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c399t-fade1064639d74eeb890e32850254b133189e339c203131b8e5aeb322de69f593</citedby></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,780,784,27924,27925</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/4429803$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Vettore, L.</creatorcontrib><creatorcontrib>Sandre, G. De</creatorcontrib><creatorcontrib>Iorio, E. E. Di</creatorcontrib><creatorcontrib>Winterhalter, K.H.</creatorcontrib><creatorcontrib>Lang, A.</creatorcontrib><creatorcontrib>Lehmann, H.</creatorcontrib><title>A New Abnormal Hemoglobin O Padova, α 30 (B11) Glu → Lys, and a Dyserythropoietic Anemia With Erythroblastic Multinuclearity Coexisting in the Same Patient</title><title>Blood</title><addtitle>Blood</addtitle><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.</description><subject>Adult</subject><subject>Amino Acids - analysis</subject><subject>Anemia - complications</subject><subject>Bilirubin - blood</subject><subject>Bone Marrow Cells</subject><subject>Cell Nucleus</subject><subject>Cell Survival</subject><subject>Complement System Proteins</subject><subject>Electrophoresis, Starch Gel</subject><subject>Erythrocytes, Abnormal</subject><subject>Female</subject><subject>Fetal Hemoglobin - analysis</subject><subject>Globins - isolation & purification</subject><subject>Heinz Bodies</subject><subject>Hemagglutination Tests</subject><subject>Hemoglobinopathies - complications</subject><subject>Hemoglobins, Abnormal</subject><subject>Hemolysis</subject><subject>Humans</subject><subject>Italy</subject><subject>Osmotic Fragility</subject><subject>Oxygen - analysis</subject><subject>Peptides - analysis</subject><subject>Potassium - blood</subject><subject>Pregnancy</subject><subject>Sodium - blood</subject><subject>Temperature</subject><issn>0006-4971</issn><issn>1528-0020</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>1974</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp9kU1u1EAQhVuIKEwCJ0BItUJEiof-sT3uBYthCAnSQJD4W7badk2mUds9dLcDvgAH4AC5Qy6SQ3ASepgRSxalWryq9_T0EfKY0SljFX9eW-fa6ec8n5bTqpTbuUcmrOBVRimn98mEUlpmuZyxB-QohK-Uslzw4pAc5jmXFRUTcjOHd_gd5nXvfKctXGDnrqyrTQ-X8F637lqfwt0tCArPXjJ2Aud2gN8_f8FyDKeg-xY0vBoD-jGuvds4g9E0MO-xMxq-mLiGs51UWx220tvBRtMPjUXtTRxh4fCHSUp_BSkzrhE-6A5TdDTYx4fkYKVtwEf7fUw-vT77uLjIlpfnbxbzZdYIKWO20i0yWualkO0sR6wrSVHwqqC8yGsmBKskCiEbTgUTrK6w0FgLzlss5aqQ4pg83fluvPs2YIiqM6FBa3WPbgiq4sVMpoB0KHaHjXcheFypjTed9qNiVG2pqL9UVKKiSpWIbCd9PdnbD3WH7b-fPYakv9jpmDpeG_QqNKl-g63x2ETVOvNf_z-4a5_k</recordid><startdate>197412</startdate><enddate>197412</enddate><creator>Vettore, L.</creator><creator>Sandre, G. De</creator><creator>Iorio, E. E. Di</creator><creator>Winterhalter, K.H.</creator><creator>Lang, A.</creator><creator>Lehmann, H.</creator><general>Elsevier Inc</general><scope>6I.</scope><scope>AAFTH</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope></search><sort><creationdate>197412</creationdate><title>A New Abnormal Hemoglobin O Padova, α 30 (B11) Glu → Lys, and a Dyserythropoietic Anemia With Erythroblastic Multinuclearity Coexisting in the Same Patient</title><author>Vettore, L. ; Sandre, G. De ; Iorio, E. E. 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De</creatorcontrib><creatorcontrib>Iorio, E. E. Di</creatorcontrib><creatorcontrib>Winterhalter, K.H.</creatorcontrib><creatorcontrib>Lang, A.</creatorcontrib><creatorcontrib>Lehmann, H.</creatorcontrib><collection>ScienceDirect Open Access Titles</collection><collection>Elsevier:ScienceDirect:Open Access</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Blood</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Vettore, L.</au><au>Sandre, G. De</au><au>Iorio, E. E. Di</au><au>Winterhalter, K.H.</au><au>Lang, A.</au><au>Lehmann, H.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>A New Abnormal Hemoglobin O Padova, α 30 (B11) Glu → Lys, and a Dyserythropoietic Anemia With Erythroblastic Multinuclearity Coexisting in the Same Patient</atitle><jtitle>Blood</jtitle><addtitle>Blood</addtitle><date>1974-12</date><risdate>1974</risdate><volume>44</volume><issue>6</issue><spage>869</spage><epage>877</epage><pages>869-877</pages><issn>0006-4971</issn><eissn>1528-0020</eissn><abstract>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.</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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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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