Acute splenic sequestration in hemoglobin sickle O-Arab disease.
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.
D000208:Acute Disease; D000755:Anemia, Sickle Cell / Q000150:complications*; D002675:Child, Preschool; D005260:Female; D006451:Hemoglobin, Sickle; D006455:Hemoglobins, Abnormal / Q000235:genetics; D006801:Humans; D013158:Splenic Diseases / Q000209:etiology*
D006451:Hemoglobin, Sickle; D006455:Hemoglobins, Abnormal