Successful Treatment of a Child with Hemoglobin Hammersmith with Hematopoietic Stem Cell Transplantation

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dc.contributor.authorYıldırım A.T.
dc.contributor.authorGülen H.
dc.contributor.authorTürkmen H.
dc.contributor.authorÖzek G.
dc.contributor.authorOymak Y.
dc.contributor.authorDurmaz B.
dc.contributor.authorKaraca E.
dc.date.accessioned2024-07-22T08:03:24Z
dc.date.available2024-07-22T08:03:24Z
dc.date.issued2023
dc.description.abstractHemoglobin (Hb) Hammersmith, formed by serine substitution for phenylalanine at residue 42 in the beta-globin chain, is a very rare variant of unstable hemoglobin with low oxygen affinity. For patients with hemoglobinopathies, it is well-established that hematopoietic stem cell transplantation provides a complete cure, but the literature on its role for those with Hb Hammersmith is limited. A seven-month-old girl who was examined for anemia and splenomegaly was followed up for congenital hemolytic anemia. The patient with visible cyanosis of the lips and whose p50 was low in blood gas was diagnosed with Hb Hammersmith through the DNA sequence analysis. During the follow-up, frequent blood transfusions had to be given due to anemia aggravated by infections. Following a successful hematopoietic stem cell transplant from an HLA-matched sibling, the patient completely recovered from Hb Hammersmith. The case is presented because of its rarity. © 2023 Informa UK Limited, trading as Taylor & Francis Group.
dc.identifier.DOI-ID10.1080/03630269.2023.2219007
dc.identifier.issn03630269
dc.identifier.urihttp://akademikarsiv.cbu.edu.tr:4000/handle/123456789/12269
dc.language.isoEnglish
dc.publisherTaylor and Francis Ltd.
dc.subjectAnemia, Hemolytic
dc.subjectChild
dc.subjectFemale
dc.subjectHematopoietic Stem Cell Transplantation
dc.subjectHemoglobinopathies
dc.subjectHemoglobins, Abnormal
dc.subjectHumans
dc.subjectInfant
dc.subjectbilirubin
dc.subjectcyclosporine
dc.subjectfolic acid
dc.subjecthemoglobin
dc.subjecthemoglobin A
dc.subjecthemoglobin A2
dc.subjecthemoglobin F
dc.subjectmethotrexate
dc.subjectoxygen
dc.subjectsteroid
dc.subjecttreosulfan
dc.subjectzinc
dc.subjecthemoglobin Hammersmith
dc.subjecthemoglobin variant
dc.subjectacute graft versus host disease
dc.subjectacute skin graft versus host disease
dc.subjectacute skin graft versus host disease
dc.subjectallele
dc.subjectallogeneic hematopoietic stem cell transplantation
dc.subjectanemia
dc.subjectArticle
dc.subjectbasophilic stippling
dc.subjectcase report
dc.subjectchimera
dc.subjectclinical article
dc.subjectcyanosis
dc.subjecterythrocyte transfusion
dc.subjecterythropoiesis
dc.subjectfemale
dc.subjectfollow up
dc.subjectgraft failure
dc.subjectgraft versus host reaction
dc.subjectgrowth retardation
dc.subjecthemoglobin blood level
dc.subjecthemoglobin hammersmith
dc.subjecthemoglobinopathy
dc.subjecthemolytic anemia
dc.subjecthigh performance liquid chromatography
dc.subjecthigh throughput sequencing
dc.subjecthuman
dc.subjectinfant
dc.subjectlung alveolus oxygen tension
dc.subjectmean corpuscular hemoglobin
dc.subjectmean corpuscular hemoglobin concentration
dc.subjectmean corpuscular volume
dc.subjectmyeloablative conditioning
dc.subjectneutrophil
dc.subjectparental consent
dc.subjectpulse oximetry
dc.subjectred blood cell distribution width
dc.subjectreticulocyte
dc.subjectrisk reduction
dc.subjectspleen
dc.subjectsplenectomy
dc.subjectsplenomegaly
dc.subjectsteroid therapy
dc.subjectchild
dc.subjectgenetics
dc.subjecthematopoietic stem cell transplantation
dc.subjecthemoglobinopathy
dc.subjecthemolytic anemia
dc.titleSuccessful Treatment of a Child with Hemoglobin Hammersmith with Hematopoietic Stem Cell Transplantation
dc.typeArticle

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