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Pediatr Blood Cancer. 2013 Jul;60(7):E49-51. doi: 10.1002/pbc.24444. Epub 2012 Dec 31.

Massive splenic infarction in an adolescent with hemoglobin S-HPFH.

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Yale University School of Medicine, New Haven, Connecticut, USA.


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.

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