Massive splenic infarction in an adolescent with hemoglobin S-HPFH

Donna Whyte; Bernard Forget; David H.K. Chui; Hong yuan Luo; Farzana Pashankar

doi:10.1002/pbc.24444

Massive splenic infarction in an adolescent with hemoglobin S-HPFH

Donna Whyte, Bernard Forget, David H.K. Chui, Hong yuan Luo, Farzana Pashankar

Research output: Contribution to journal › Article › peer-review

2 Scopus citations

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.

Original language	English (US)
Pages (from-to)	E49-E51
Journal	Pediatric Blood and Cancer
Volume	60
Issue number	7
DOIs	https://doi.org/10.1002/pbc.24444
State	Published - Jul 2013
Externally published	Yes

Keywords

Children
Sickle cell disease
Splenic infarction

ASJC Scopus subject areas

Pediatrics, Perinatology, and Child Health
Hematology
Oncology

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10.1002/pbc.24444

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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.",

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T1 - Massive splenic infarction in an adolescent with hemoglobin S-HPFH

AU - Whyte, Donna

AU - Forget, Bernard

AU - Chui, David H.K.

AU - Luo, Hong yuan

AU - Pashankar, Farzana

PY - 2013/7

Y1 - 2013/7

N2 - 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.

AB - 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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KW - Sickle cell disease

KW - Splenic infarction

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Massive splenic infarction in an adolescent with hemoglobin S-HPFH

Abstract

Keywords

ASJC Scopus subject areas

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