Premium
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
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.
Accelerating Research
Robert Robinson Avenue,
Oxford Science Park, Oxford
OX4 4GP, United Kingdom
Address
John Eccles HouseRobert Robinson Avenue,
Oxford Science Park, Oxford
OX4 4GP, United Kingdom