title: Massive splenic infarction in an adolescent with hemoglobin S-HPFH.
abstract: Hemoglobin sickle-hereditary persistence of fetal hemoglobin (S-HPFH) is a condition in which there is compound heterozygosity for the Hb S mutation and the HPFH deletion. These patients have no anemia, little evidence of hemolysis and generally have a benign clinical course compared to other types of sickle cell anemia. We describe a 19-year-old male with HbS-HPFH who had no history of anemia or vaso-occlusive crisis, who presented with a massive splenic infarct. We conclude that patients with HbS-HPFH can occasionally present with severe complications and require a high level of clinical suspicion for complications when presenting to the hospital.
authors.affiliation: Yale University School of Medicine, New Haven, Connecticut, USA.
authors.forename: Donna
authors.initials: D
authors.lastname: Whyte
authors.forename: Bernard
authors.initials: B
authors.lastname: Forget
authors.forename: David H K
authors.initials: DH
authors.lastname: Chui
authors.forename: Hong-yuan
authors.initials: HY
authors.lastname: Luo
authors.forename: Farzana
authors.initials: F
authors.lastname: Pashankar
mesh_terms: D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000150:complications* / Q000503:physiopathology*; D005319:Fetal Hemoglobin; D006451:Hemoglobin, Sickle; D006801:Humans; D008297:Male; D013159:Splenic Infarction / Q000209:etiology* / Q000503:physiopathology*
chemical_list: D006451:Hemoglobin, Sickle; D005319:Fetal Hemoglobin