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Massive splenic infarction in an adolescent with hemoglobin S‐HPFH
Author(s) -
Whyte Donna,
Forget Bernard,
Chui David H.K.,
Luo Hongyuan,
Pashankar Farzana
Publication year - 2013
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.24444
Subject(s) - medicine , hemolysis , hemoglobin , fetal hemoglobin , splenic infarction , anemia , hemolytic anemia , blood cancer , compound heterozygosity , hemoglobinopathy , pediatrics , mutation , fetus , pregnancy , cancer , spleen , gene , genetics , biology
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. Pediatr Blood Cancer 2013; 60: E49–E51. © 2012 Wiley Periodicals, Inc.