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Hemoglobin
international journal for hemoglobin research
Volume 28, 2004 - Issue 4
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Review Article

Hb Hope [β136(H14)Gly→Asp (GGT→GAT)]: Interactions with Hb S [β6(A3)Glu→Val (GAG→GTG)], Other Variant Hemoglobins and Thalassemia

John Ingle Department of Medicine and Pathology, Division of Hematology/Oncology and the Center of Excellence in Sickle Cell Disease, Boston University School of Medicine, Boston, MassachusettsUSA; Hemoglobin Diagnostic Reference Laboratory, Boston Medical Center, Boston, MassachusettsUSA
,
Adeboye Adewoye Department of Medicine and Pathology, Division of Hematology/Oncology and the Center of Excellence in Sickle Cell Disease, Boston University School of Medicine, Boston, MassachusettsUSA; Hemoglobin Diagnostic Reference Laboratory, Boston Medical Center, Boston, MassachusettsUSA
,
Robert Dewan Department of Medicine and Pathology, Division of Hematology/Oncology and the Center of Excellence in Sickle Cell Disease, Boston University School of Medicine, Boston, MassachusettsUSA; Hemoglobin Diagnostic Reference Laboratory, Boston Medical Center, Boston, MassachusettsUSA
,
Michael Okoli Department of Medicine and Pathology, Division of Hematology/Oncology and the Center of Excellence in Sickle Cell Disease, Boston University School of Medicine, Boston, MassachusettsUSA; Hemoglobin Diagnostic Reference Laboratory, Boston Medical Center, Boston, MassachusettsUSA
,
Lamarr Rollins Department of Medicine and Pathology, Division of Hematology/Oncology and the Center of Excellence in Sickle Cell Disease, Boston University School of Medicine, Boston, MassachusettsUSA; Hemoglobin Diagnostic Reference Laboratory, Boston Medical Center, Boston, MassachusettsUSA
,
Shawn H. Eung Department of Medicine and Pathology, Division of Hematology/Oncology and the Center of Excellence in Sickle Cell Disease, Boston University School of Medicine, Boston, MassachusettsUSA; Hemoglobin Diagnostic Reference Laboratory, Boston Medical Center, Boston, MassachusettsUSA
,
Hong‐yuan Luo Department of Medicine and Pathology, Division of Hematology/Oncology and the Center of Excellence in Sickle Cell Disease, Boston University School of Medicine, Boston, MassachusettsUSA; Hemoglobin Diagnostic Reference Laboratory, Boston Medical Center, Boston, MassachusettsUSA
,
David H. K. Chui Department of Medicine and Pathology, Division of Hematology/Oncology and the Center of Excellence in Sickle Cell Disease, Boston University School of Medicine, Boston, MassachusettsUSA; Hemoglobin Diagnostic Reference Laboratory, Boston Medical Center, Boston, MassachusettsUSA
&
Martin H. Steinberg Department of Medicine and Pathology, Division of Hematology/Oncology and the Center of Excellence in Sickle Cell Disease, Boston University School of Medicine, Boston, MassachusettsUSA; Hemoglobin Diagnostic Reference Laboratory, Boston Medical Center, Boston, MassachusettsUSA; Center of Excellence in Sickle Cell Disease E248, Boston Medical Center, 88 E. Newton St., Boston, Massachusetts, 02118, USA
, M.D. show all
Pages 277-285 | Received 14 Jan 2004, Accepted 15 Jun 2004, Published online: 24 Aug 2009
 
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Abstract

Hb Hope [β136(H14)Gly→Asp (GGT→GAT)] was first described in an African–American family in 1965. Since then, it has been found in combination with several different globin gene mutations in many other families of divergent ethnic backgrounds. The basis for its relatively frequent occurrences remains unexplained. This variant hemoglobin (Hb) is mildly unstable and has reduced oxygen affinity, but is generally innocuous clinically. This variant Hb can present as a confounding factor in arriving at a correct diagnosis by either electrophoresis or high performance liquid chromatography (HPLC), particularly during the neonatal period. DNA‐based diagnostics can help solve this potential problem.

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