Document Detail

Interaction of sickle cell trait with hereditary spherocytosis: splenic infarcts and sequestration.
MedLine Citation:
PMID:  12486324     Owner:  NLM     Status:  MEDLINE    
The association of sickle cell trait (SCT) and hereditary spherocytosis (HS) has been reported in only 18 patients. Three of these 18 patients experienced splenic infarct or acute splenic sequestration. We report here a 46-year-old African-American male, the oldest reported case to date, who experienced episodes of hemolysis and severe left upper quadrant pain for the past 26 years. The patient had compensated hemolysis with splenomegaly. A CT scan of the abdomen revealed a large infarct in the spleen. The diagnosis of SCT was confirmed with isoelectric focusing, cation exchange and reverse-phase HPLC. The presence of a silent, interacting globin variant as the cause of hemolysis and sickling in the spleen was ruled out by sequencing of the alpha1-, alpha2- and beta-globin genes. The diagnosis of HS was established by an osmotic fragility test. The interaction of HS and SCT leads to RBC dehydration with increased MCHC and intracellular Hb S concentration presumably favoring intrasplenic sickling and resultant splenic infarcts and sequestration as seen in this case.
Celatettin Ustun; Ferdane Kutlar; Leslie Holley; Maree Seigler; Russell Burgess; Abdullah Kutlar
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Publication Detail:
Type:  Case Reports; Journal Article; Review    
Journal Detail:
Title:  Acta haematologica     Volume:  109     ISSN:  0001-5792     ISO Abbreviation:  Acta Haematol.     Publication Date:  2003  
Date Detail:
Created Date:  2002-12-17     Completed Date:  2003-03-18     Revised Date:  2005-11-16    
Medline Journal Info:
Nlm Unique ID:  0141053     Medline TA:  Acta Haematol     Country:  Switzerland    
Other Details:
Languages:  eng     Pagination:  46-9     Citation Subset:  IM    
Copyright Information:
Copyright 2003 S. Karger AG, Basel
Department of Medicine, Section of Hematology/Oncology, Medical College of Georgia, Augusta, Ga. 30912, USA.
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MeSH Terms
Anemia, Sickle Cell / complications*,  diagnosis
Middle Aged
Osmotic Fragility
Spherocytosis, Hereditary / complications*,  diagnosis
Spleen / abnormalities
Splenic Infarction / etiology
Splenomegaly / etiology
Comment In:
Acta Haematol. 2003;110(4):223   [PMID:  14663174 ]

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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