Acute splenic sequestration in hemoglobin sickle O-Arab disease
Gilman, P.A.; Abel, A.S.
Johns Hopkins Medical Journal 146(6): 285-288
1980
ISSN/ISBN: 0021-7263 PMID: 7382253 Document Number: 156123
A white girl with sickle O-Arab disease (Hb S/O-Arab) had three separate episodes of acute splenic sequestration at 8, 12, and 15 months of age with hemoglobin levels falling to 3.9, 2.8, and 4.2 g/dl, respectively. Functional hyposplenism was suggested by the radionucleotide spleen scan. Following splenectomy her hemoglobin stabilized in the range of 0.7--8.5 g/dl. Life-threatening episodes of splenic sequestration, pneumococcal infections and osteomyelitis may occur in both Hb S/O-Arab and homozygous sickle cell diseases.