Hb S/Hb Lepore with mild sickling symptoms: a hemoglobin variant with mostly delta-chain sequences ameliorates sickle-cell disease.
Three cases are reported of Hb S/Hb Lepore combination with very mild sickling manifestations. The presence of a nonalpha-chain variant with a high proportion of delta chain sequences, including 22 ala, appears to ameliorate sickle-cell disease. Efforts to increase the proportion of Hb A2 may be beneficial in sickle-cell disease.
Fairbanks
V F
VF
Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota 55905, USA.
McCormick
D J
DJ
Kubik
K S
KS
Rezuke
W N
WN
Black
D
D
Ochaney
M S
MS
Schwartz
D
D
D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000097:blood* / Q000503:physiopathology*; D005260:Female; D014644:Genetic Variation; D006451:Hemoglobin, Sickle / Q000032:analysis*; D006454:Hemoglobins / Q000235:genetics*; D006455:Hemoglobins, Abnormal / Q000032:analysis*; D006801:Humans; D008297:Male
D006451:Hemoglobin, Sickle; D006454:Hemoglobins; D006455:Hemoglobins, Abnormal; C037659:hemoglobin Lepore