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Article: Sibling HLA-matched cord blood transplant for β-thalassemia: Report of two cases, expression of fetal hemoglobin, and review of the literature

TitleSibling HLA-matched cord blood transplant for β-thalassemia: Report of two cases, expression of fetal hemoglobin, and review of the literature
Authors
Keywordsβ-thalassemia major
Cord blood stem cell
Fetal hemoglobin
Transplantation
Issue Date1998
PublisherLippincott Williams & Wilkins. The Journal's web site is located at http://www.jpho-online.com
Citation
Journal Of Pediatric Hematology/Oncology, 1998, v. 20 n. 5, p. 477-481 How to Cite?
AbstractPurpose: A program of cord blood stem cell (CBSC) transplants for patients with β-thalassemia major was initiated in conjunction with the prenatal diagnostic service in 1994. Two patients who received HLA-matched related CBSC transplants with posttransplant fetal hemoglobin (HbF) expression are described and the literature is reviewed. Patients and Methods: After screening 12 pregnancies, matched sibling CBSC transplants were performed for 2 girls with β-thalassemia major when they were 3.8 and 2.2 years old, respectively. Their HbF was assayed serially. Results: The nucleated cell counts/kg were 11.4 x 10 7 and 6.2 x 10 7, which engrafted on days 19 and 24, respectively. The children are now transfusion-independent at 3 years and 1.2 years posttransplant. Their HbF levels showed a rapid rise posttransplant and reached peak levels of 37.2% and 42.2% on day 83 and day 88, respectively. The HbF levels declined to 1.0% and 3.8% on day 581 and day 305, respectively. Nine other sibling CBSC transplants for thalassemias have been reported with an engraftment rate of approximately 50%. Graft rejection was related to insufficient CBSC number in one. Conclusions: HbF levels in patients with β-thalassemia major after CBSC transplants could be influenced by many factors, including reactivation of HbF synthesis, intrinsic rate of Hb switching of CBSC, and mixed chimerism.
Persistent Identifierhttp://hdl.handle.net/10722/170293
ISSN
2015 Impact Factor: 1.146
2015 SCImago Journal Rankings: 0.452
ISI Accession Number ID
References

 

DC FieldValueLanguage
dc.contributor.authorLau, YLen_HK
dc.contributor.authorMa, ESKen_HK
dc.contributor.authorHa, SYen_HK
dc.contributor.authorChan, GCFen_HK
dc.contributor.authorChiu, Den_HK
dc.contributor.authorTang, Men_HK
dc.contributor.authorHawkins, BRen_HK
dc.contributor.authorChan, Ven_HK
dc.contributor.authorLiang, RHSen_HK
dc.date.accessioned2012-10-30T06:07:17Z-
dc.date.available2012-10-30T06:07:17Z-
dc.date.issued1998en_HK
dc.identifier.citationJournal Of Pediatric Hematology/Oncology, 1998, v. 20 n. 5, p. 477-481en_HK
dc.identifier.issn1077-4114en_HK
dc.identifier.urihttp://hdl.handle.net/10722/170293-
dc.description.abstractPurpose: A program of cord blood stem cell (CBSC) transplants for patients with β-thalassemia major was initiated in conjunction with the prenatal diagnostic service in 1994. Two patients who received HLA-matched related CBSC transplants with posttransplant fetal hemoglobin (HbF) expression are described and the literature is reviewed. Patients and Methods: After screening 12 pregnancies, matched sibling CBSC transplants were performed for 2 girls with β-thalassemia major when they were 3.8 and 2.2 years old, respectively. Their HbF was assayed serially. Results: The nucleated cell counts/kg were 11.4 x 10 7 and 6.2 x 10 7, which engrafted on days 19 and 24, respectively. The children are now transfusion-independent at 3 years and 1.2 years posttransplant. Their HbF levels showed a rapid rise posttransplant and reached peak levels of 37.2% and 42.2% on day 83 and day 88, respectively. The HbF levels declined to 1.0% and 3.8% on day 581 and day 305, respectively. Nine other sibling CBSC transplants for thalassemias have been reported with an engraftment rate of approximately 50%. Graft rejection was related to insufficient CBSC number in one. Conclusions: HbF levels in patients with β-thalassemia major after CBSC transplants could be influenced by many factors, including reactivation of HbF synthesis, intrinsic rate of Hb switching of CBSC, and mixed chimerism.en_HK
dc.languageengen_US
dc.publisherLippincott Williams & Wilkins. The Journal's web site is located at http://www.jpho-online.comen_HK
dc.relation.ispartofJournal of Pediatric Hematology/Oncologyen_HK
dc.rightsJournal of Pediatric Hematology / Oncology. Copyright © Lippincott Williams & Wilkins.-
dc.subjectβ-thalassemia majoren_HK
dc.subjectCord blood stem cellen_HK
dc.subjectFetal hemoglobinen_HK
dc.subjectTransplantationen_HK
dc.subject.meshFemaleen_US
dc.subject.meshFetal Blooden_US
dc.subject.meshFetal Hemoglobin - Biosynthesisen_US
dc.subject.meshFetal Tissue Transplantationen_US
dc.subject.meshGraft Survivalen_US
dc.subject.meshHematopoietic Stem Cell Transplantationen_US
dc.subject.meshHistocompatibility Testingen_US
dc.subject.meshHumansen_US
dc.subject.meshInfanten_US
dc.subject.meshTransplantation, Homologousen_US
dc.subject.meshBeta-Thalassemia - Blood - Immunology - Therapyen_US
dc.titleSibling HLA-matched cord blood transplant for β-thalassemia: Report of two cases, expression of fetal hemoglobin, and review of the literatureen_HK
dc.typeArticleen_HK
dc.identifier.emailLau, YL: lauylung@hku.hken_HK
dc.identifier.emailChan, GCF: gcfchan@hku.hken_HK
dc.identifier.emailTang, M: mhytang@hkucc.hku.hken_HK
dc.identifier.emailChan, V: vnychana@hkucc.hku.hken_HK
dc.identifier.emailLiang, RHS: rliang@hku.hken_HK
dc.identifier.authorityLau, YL=rp00361en_HK
dc.identifier.authorityChan, GCF=rp00431en_HK
dc.identifier.authorityTang, M=rp01701en_HK
dc.identifier.authorityChan, V=rp00320en_HK
dc.identifier.authorityLiang, RHS=rp00345en_HK
dc.description.naturelink_to_subscribed_fulltexten_US
dc.identifier.doi10.1097/00043426-199809000-00014en_HK
dc.identifier.pmid9787324-
dc.identifier.scopuseid_2-s2.0-0032469442en_HK
dc.identifier.hkuros39302-
dc.relation.referenceshttp://www.scopus.com/mlt/select.url?eid=2-s2.0-0032469442&selection=ref&src=s&origin=recordpageen_HK
dc.identifier.volume20en_HK
dc.identifier.issue5en_HK
dc.identifier.spage477en_HK
dc.identifier.epage481en_HK
dc.identifier.isiWOS:000076358900014-
dc.publisher.placeUnited Statesen_HK
dc.identifier.scopusauthoridLau, YL=7201403380en_HK
dc.identifier.scopusauthoridMa, ESK=7202039934en_HK
dc.identifier.scopusauthoridHa, SY=7202501115en_HK
dc.identifier.scopusauthoridChan, GCF=16160154400en_HK
dc.identifier.scopusauthoridChiu, D=16749062300en_HK
dc.identifier.scopusauthoridTang, M=35362943900en_HK
dc.identifier.scopusauthoridHawkins, BR=35944486200en_HK
dc.identifier.scopusauthoridChan, V=7202654865en_HK
dc.identifier.scopusauthoridLiang, RHS=26643224900en_HK

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