Hydrops fetalis–associated congenital dyserythropoietic anemia treated with intrauterine transfusions and bone marrow transplantation
Hydrops fetalis is rarely caused by congenital dyserythropoietic anemia (CDA). We report a patient with hydrops fetalis as a result of severe anemia. This patient needed intrauterine transfusions from 21 weeks of gestation until birth. The hematologic study showed an atypical CDA (hydrops fetalis–as...
Gespeichert in:
| Veröffentlicht in: | Blood 2002-07, Vol.100 (1), p.356-358 |
|---|---|
| Hauptverfasser: | , , , , , , , , , |
| Format: | Artikel |
| Sprache: | eng |
| Schlagworte: | |
| Online-Zugang: | Volltext |
| Tags: |
Tag hinzufügen
Keine Tags, Fügen Sie den ersten Tag hinzu!
|
| container_end_page | 358 |
|---|---|
| container_issue | 1 |
| container_start_page | 356 |
| container_title | Blood |
| container_volume | 100 |
| creator | Remacha, Angel F. Badell, Isabel Pujol-Moix, Núria Parra, Juan Muñiz-Diaz, Eduardo Ginovart, Gemma Sardà, M. Pilar Hernández, Angel Moliner, Elisenda Torrent, Montserrat |
| description | Hydrops fetalis is rarely caused by congenital dyserythropoietic anemia (CDA). We report a patient with hydrops fetalis as a result of severe anemia. This patient needed intrauterine transfusions from 21 weeks of gestation until birth. The hematologic study showed an atypical CDA (hydrops fetalis–associated CDA) characterized by features resembling CDA type II, but negative acidified serum lysis test (HEMPAS negative). The patient was regularly transfused for a year, after which an allogeneic bone marrow transplantation (BMT) from an HLA-identical sibling was successfully carried out. His actual hemoglobin is 127 g/L, and he has not received transfusions for more than a year. In conclusion, intrauterine transfusions and BMT could cure an otherwise lethal atypical CDA. |
| doi_str_mv | 10.1182/blood-2001-12-0351 |
| format | Article |
| fullrecord | <record><control><sourceid>proquest_cross</sourceid><recordid>TN_cdi_proquest_miscellaneous_71830265</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><els_id>S0006497120600155</els_id><sourcerecordid>71830265</sourcerecordid><originalsourceid>FETCH-LOGICAL-c426t-133237eb9da92623b91c9b53f21d85ee1dfba47328b96b40b751db0cd130e11c3</originalsourceid><addsrcrecordid>eNp9kE1rFEEQhhtRzCb6BzzIXPQ22lU9n-BFgiZCwIuem_6oMS2z02tXj2Fv3vwB_kN_ib3Zhdw8FdT7VPHyCPEC5BuAAd_aOUZfo5RQA9ZStfBIbKDFoZYS5WOxkVJ2dTP2cCbOmb8XsFHYPhVngLKXsoWN-H299ynuuJoomznw319_DHN0wWTylYvLN1pCSSq_Z0r7fFvgGCgHV5mFtsFUOdE9exfybRWWnMyaKYWFSmIWnlYOceFC-8rGst2alOLdMdzNZskmF-CZeDKZmen5aV6Irx8_fLm8rm8-X326fH9Tuwa7XINSqHqyozcjdqjsCG60rZoQ_NASgZ-saXqFgx0720jbt-CtdB6UJACnLsTr499dij9W4qy3gR3NpQjFlXUPg5LYtQXEI-hSZE406V0Kpfteg9QH_fpevz7o14D6oL8cvTx9X-2W_MPJyXcBXp0Aw87MU5HgAj9wqm8RGyzcuyNHxcXPQEmzC7Q48iGRy9rH8L8e_wBA16hs</addsrcrecordid><sourcetype>Aggregation Database</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>71830265</pqid></control><display><type>article</type><title>Hydrops fetalis–associated congenital dyserythropoietic anemia treated with intrauterine transfusions and bone marrow transplantation</title><source>MEDLINE</source><source>EZB-FREE-00999 freely available EZB journals</source><source>Alma/SFX Local Collection</source><creator>Remacha, Angel F. ; Badell, Isabel ; Pujol-Moix, Núria ; Parra, Juan ; Muñiz-Diaz, Eduardo ; Ginovart, Gemma ; Sardà, M. Pilar ; Hernández, Angel ; Moliner, Elisenda ; Torrent, Montserrat</creator><creatorcontrib>Remacha, Angel F. ; Badell, Isabel ; Pujol-Moix, Núria ; Parra, Juan ; Muñiz-Diaz, Eduardo ; Ginovart, Gemma ; Sardà, M. Pilar ; Hernández, Angel ; Moliner, Elisenda ; Torrent, Montserrat</creatorcontrib><description>Hydrops fetalis is rarely caused by congenital dyserythropoietic anemia (CDA). We report a patient with hydrops fetalis as a result of severe anemia. This patient needed intrauterine transfusions from 21 weeks of gestation until birth. The hematologic study showed an atypical CDA (hydrops fetalis–associated CDA) characterized by features resembling CDA type II, but negative acidified serum lysis test (HEMPAS negative). The patient was regularly transfused for a year, after which an allogeneic bone marrow transplantation (BMT) from an HLA-identical sibling was successfully carried out. His actual hemoglobin is 127 g/L, and he has not received transfusions for more than a year. In conclusion, intrauterine transfusions and BMT could cure an otherwise lethal atypical CDA.</description><identifier>ISSN: 0006-4971</identifier><identifier>EISSN: 1528-0020</identifier><identifier>DOI: 10.1182/blood-2001-12-0351</identifier><identifier>PMID: 12070051</identifier><language>eng</language><publisher>Washington, DC: Elsevier Inc</publisher><subject>Adult ; Anemia, Dyserythropoietic, Congenital - complications ; Anemia, Dyserythropoietic, Congenital - therapy ; Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy ; Biological and medical sciences ; Blood Transfusion, Intrauterine ; Bone Marrow Transplantation ; Bone marrow, stem cells transplantation. Graft versus host reaction ; Diseases of mother, fetus and pregnancy ; Family Health ; Female ; Fetal Diseases - therapy ; Gynecology. Andrology. Obstetrics ; Humans ; Hydrops Fetalis - diagnosis ; Hydrops Fetalis - etiology ; Hydrops Fetalis - therapy ; Infant, Newborn ; Male ; Medical sciences ; Pregnancy ; Pregnancy. Fetus. Placenta ; Prenatal Diagnosis ; Transfusions. Complications. Transfusion reactions. Cell and gene therapy ; Transplantation, Homologous ; Treatment Outcome</subject><ispartof>Blood, 2002-07, Vol.100 (1), p.356-358</ispartof><rights>2002 American Society of Hematology</rights><rights>2002 INIST-CNRS</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c426t-133237eb9da92623b91c9b53f21d85ee1dfba47328b96b40b751db0cd130e11c3</citedby><cites>FETCH-LOGICAL-c426t-133237eb9da92623b91c9b53f21d85ee1dfba47328b96b40b751db0cd130e11c3</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,780,784,27923,27924</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=13752242$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/12070051$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Remacha, Angel F.</creatorcontrib><creatorcontrib>Badell, Isabel</creatorcontrib><creatorcontrib>Pujol-Moix, Núria</creatorcontrib><creatorcontrib>Parra, Juan</creatorcontrib><creatorcontrib>Muñiz-Diaz, Eduardo</creatorcontrib><creatorcontrib>Ginovart, Gemma</creatorcontrib><creatorcontrib>Sardà, M. Pilar</creatorcontrib><creatorcontrib>Hernández, Angel</creatorcontrib><creatorcontrib>Moliner, Elisenda</creatorcontrib><creatorcontrib>Torrent, Montserrat</creatorcontrib><title>Hydrops fetalis–associated congenital dyserythropoietic anemia treated with intrauterine transfusions and bone marrow transplantation</title><title>Blood</title><addtitle>Blood</addtitle><description>Hydrops fetalis is rarely caused by congenital dyserythropoietic anemia (CDA). We report a patient with hydrops fetalis as a result of severe anemia. This patient needed intrauterine transfusions from 21 weeks of gestation until birth. The hematologic study showed an atypical CDA (hydrops fetalis–associated CDA) characterized by features resembling CDA type II, but negative acidified serum lysis test (HEMPAS negative). The patient was regularly transfused for a year, after which an allogeneic bone marrow transplantation (BMT) from an HLA-identical sibling was successfully carried out. His actual hemoglobin is 127 g/L, and he has not received transfusions for more than a year. In conclusion, intrauterine transfusions and BMT could cure an otherwise lethal atypical CDA.</description><subject>Adult</subject><subject>Anemia, Dyserythropoietic, Congenital - complications</subject><subject>Anemia, Dyserythropoietic, Congenital - therapy</subject><subject>Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy</subject><subject>Biological and medical sciences</subject><subject>Blood Transfusion, Intrauterine</subject><subject>Bone Marrow Transplantation</subject><subject>Bone marrow, stem cells transplantation. Graft versus host reaction</subject><subject>Diseases of mother, fetus and pregnancy</subject><subject>Family Health</subject><subject>Female</subject><subject>Fetal Diseases - therapy</subject><subject>Gynecology. Andrology. Obstetrics</subject><subject>Humans</subject><subject>Hydrops Fetalis - diagnosis</subject><subject>Hydrops Fetalis - etiology</subject><subject>Hydrops Fetalis - therapy</subject><subject>Infant, Newborn</subject><subject>Male</subject><subject>Medical sciences</subject><subject>Pregnancy</subject><subject>Pregnancy. Fetus. Placenta</subject><subject>Prenatal Diagnosis</subject><subject>Transfusions. Complications. Transfusion reactions. Cell and gene therapy</subject><subject>Transplantation, Homologous</subject><subject>Treatment Outcome</subject><issn>0006-4971</issn><issn>1528-0020</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2002</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp9kE1rFEEQhhtRzCb6BzzIXPQ22lU9n-BFgiZCwIuem_6oMS2z02tXj2Fv3vwB_kN_ib3Zhdw8FdT7VPHyCPEC5BuAAd_aOUZfo5RQA9ZStfBIbKDFoZYS5WOxkVJ2dTP2cCbOmb8XsFHYPhVngLKXsoWN-H299ynuuJoomznw319_DHN0wWTylYvLN1pCSSq_Z0r7fFvgGCgHV5mFtsFUOdE9exfybRWWnMyaKYWFSmIWnlYOceFC-8rGst2alOLdMdzNZskmF-CZeDKZmen5aV6Irx8_fLm8rm8-X326fH9Tuwa7XINSqHqyozcjdqjsCG60rZoQ_NASgZ-saXqFgx0720jbt-CtdB6UJACnLsTr499dij9W4qy3gR3NpQjFlXUPg5LYtQXEI-hSZE406V0Kpfteg9QH_fpevz7o14D6oL8cvTx9X-2W_MPJyXcBXp0Aw87MU5HgAj9wqm8RGyzcuyNHxcXPQEmzC7Q48iGRy9rH8L8e_wBA16hs</recordid><startdate>20020701</startdate><enddate>20020701</enddate><creator>Remacha, Angel F.</creator><creator>Badell, Isabel</creator><creator>Pujol-Moix, Núria</creator><creator>Parra, Juan</creator><creator>Muñiz-Diaz, Eduardo</creator><creator>Ginovart, Gemma</creator><creator>Sardà, M. Pilar</creator><creator>Hernández, Angel</creator><creator>Moliner, Elisenda</creator><creator>Torrent, Montserrat</creator><general>Elsevier Inc</general><general>The Americain Society of Hematology</general><scope>6I.</scope><scope>AAFTH</scope><scope>IQODW</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>20020701</creationdate><title>Hydrops fetalis–associated congenital dyserythropoietic anemia treated with intrauterine transfusions and bone marrow transplantation</title><author>Remacha, Angel F. ; Badell, Isabel ; Pujol-Moix, Núria ; Parra, Juan ; Muñiz-Diaz, Eduardo ; Ginovart, Gemma ; Sardà, M. Pilar ; Hernández, Angel ; Moliner, Elisenda ; Torrent, Montserrat</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c426t-133237eb9da92623b91c9b53f21d85ee1dfba47328b96b40b751db0cd130e11c3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2002</creationdate><topic>Adult</topic><topic>Anemia, Dyserythropoietic, Congenital - complications</topic><topic>Anemia, Dyserythropoietic, Congenital - therapy</topic><topic>Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy</topic><topic>Biological and medical sciences</topic><topic>Blood Transfusion, Intrauterine</topic><topic>Bone Marrow Transplantation</topic><topic>Bone marrow, stem cells transplantation. Graft versus host reaction</topic><topic>Diseases of mother, fetus and pregnancy</topic><topic>Family Health</topic><topic>Female</topic><topic>Fetal Diseases - therapy</topic><topic>Gynecology. Andrology. Obstetrics</topic><topic>Humans</topic><topic>Hydrops Fetalis - diagnosis</topic><topic>Hydrops Fetalis - etiology</topic><topic>Hydrops Fetalis - therapy</topic><topic>Infant, Newborn</topic><topic>Male</topic><topic>Medical sciences</topic><topic>Pregnancy</topic><topic>Pregnancy. Fetus. Placenta</topic><topic>Prenatal Diagnosis</topic><topic>Transfusions. Complications. Transfusion reactions. Cell and gene therapy</topic><topic>Transplantation, Homologous</topic><topic>Treatment Outcome</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Remacha, Angel F.</creatorcontrib><creatorcontrib>Badell, Isabel</creatorcontrib><creatorcontrib>Pujol-Moix, Núria</creatorcontrib><creatorcontrib>Parra, Juan</creatorcontrib><creatorcontrib>Muñiz-Diaz, Eduardo</creatorcontrib><creatorcontrib>Ginovart, Gemma</creatorcontrib><creatorcontrib>Sardà, M. Pilar</creatorcontrib><creatorcontrib>Hernández, Angel</creatorcontrib><creatorcontrib>Moliner, Elisenda</creatorcontrib><creatorcontrib>Torrent, Montserrat</creatorcontrib><collection>ScienceDirect Open Access Titles</collection><collection>Elsevier:ScienceDirect:Open Access</collection><collection>Pascal-Francis</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>Remacha, Angel F.</au><au>Badell, Isabel</au><au>Pujol-Moix, Núria</au><au>Parra, Juan</au><au>Muñiz-Diaz, Eduardo</au><au>Ginovart, Gemma</au><au>Sardà, M. Pilar</au><au>Hernández, Angel</au><au>Moliner, Elisenda</au><au>Torrent, Montserrat</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Hydrops fetalis–associated congenital dyserythropoietic anemia treated with intrauterine transfusions and bone marrow transplantation</atitle><jtitle>Blood</jtitle><addtitle>Blood</addtitle><date>2002-07-01</date><risdate>2002</risdate><volume>100</volume><issue>1</issue><spage>356</spage><epage>358</epage><pages>356-358</pages><issn>0006-4971</issn><eissn>1528-0020</eissn><abstract>Hydrops fetalis is rarely caused by congenital dyserythropoietic anemia (CDA). We report a patient with hydrops fetalis as a result of severe anemia. This patient needed intrauterine transfusions from 21 weeks of gestation until birth. The hematologic study showed an atypical CDA (hydrops fetalis–associated CDA) characterized by features resembling CDA type II, but negative acidified serum lysis test (HEMPAS negative). The patient was regularly transfused for a year, after which an allogeneic bone marrow transplantation (BMT) from an HLA-identical sibling was successfully carried out. His actual hemoglobin is 127 g/L, and he has not received transfusions for more than a year. In conclusion, intrauterine transfusions and BMT could cure an otherwise lethal atypical CDA.</abstract><cop>Washington, DC</cop><pub>Elsevier Inc</pub><pmid>12070051</pmid><doi>10.1182/blood-2001-12-0351</doi><tpages>3</tpages><oa>free_for_read</oa></addata></record> |
| fulltext | fulltext |
| identifier | ISSN: 0006-4971 |
| ispartof | Blood, 2002-07, Vol.100 (1), p.356-358 |
| issn | 0006-4971 1528-0020 |
| language | eng |
| recordid | cdi_proquest_miscellaneous_71830265 |
| source | MEDLINE; EZB-FREE-00999 freely available EZB journals; Alma/SFX Local Collection |
| subjects | Adult Anemia, Dyserythropoietic, Congenital - complications Anemia, Dyserythropoietic, Congenital - therapy Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy Biological and medical sciences Blood Transfusion, Intrauterine Bone Marrow Transplantation Bone marrow, stem cells transplantation. Graft versus host reaction Diseases of mother, fetus and pregnancy Family Health Female Fetal Diseases - therapy Gynecology. Andrology. Obstetrics Humans Hydrops Fetalis - diagnosis Hydrops Fetalis - etiology Hydrops Fetalis - therapy Infant, Newborn Male Medical sciences Pregnancy Pregnancy. Fetus. Placenta Prenatal Diagnosis Transfusions. Complications. Transfusion reactions. Cell and gene therapy Transplantation, Homologous Treatment Outcome |
| title | Hydrops fetalis–associated congenital dyserythropoietic anemia treated with intrauterine transfusions and bone marrow transplantation |
| url | https://sfx.bib-bvb.de/sfx_tum?ctx_ver=Z39.88-2004&ctx_enc=info:ofi/enc:UTF-8&ctx_tim=2025-01-10T11%3A24%3A25IST&url_ver=Z39.88-2004&url_ctx_fmt=infofi/fmt:kev:mtx:ctx&rfr_id=info:sid/primo.exlibrisgroup.com:primo3-Article-proquest_cross&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.genre=article&rft.atitle=Hydrops%20fetalis%E2%80%93associated%20congenital%20dyserythropoietic%20anemia%20treated%20with%20intrauterine%20transfusions%20and%20bone%20marrow%20transplantation&rft.jtitle=Blood&rft.au=Remacha,%20Angel%20F.&rft.date=2002-07-01&rft.volume=100&rft.issue=1&rft.spage=356&rft.epage=358&rft.pages=356-358&rft.issn=0006-4971&rft.eissn=1528-0020&rft_id=info:doi/10.1182/blood-2001-12-0351&rft_dat=%3Cproquest_cross%3E71830265%3C/proquest_cross%3E%3Curl%3E%3C/url%3E&disable_directlink=true&sfx.directlink=off&sfx.report_link=0&rft_id=info:oai/&rft_pqid=71830265&rft_id=info:pmid/12070051&rft_els_id=S0006497120600155&rfr_iscdi=true |