PowerPoint Slideshow about ' Splenic Sequestration' - kieve
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A 3 year old girl known to have sickle cell disease (Hb SS) presents to the Emergency Room with a 2 day history of weakness and abdominal pain and recent onset of fever. Examination reveals HR140, RR40 T37.5C, pallor and tender splenomegaly. CBC show Hb4, WBC20, Plt 120. A diagnosis of acute splenic sequestration crisis is made.
There is a risk of hypovolemic shock and death. Splenic sequestration crisis is associated with a 10-15% mortality rate, occurring before transfusions can be given.
Treatment adulthood, but most first attacks occur between 3 months and 5 years.
Immediate treatment involves urgent RBC transfusion. This corrects the hypovolemia and causes mobilization of the blood cells trapped in the spleen. Splenomegaly begins to resolve and the hemoglobin level begins to rise (autotransfusion). The decrease in the size of the spleen and relief of abdominal pain may occur within hours to days after the transfusion.