Case Study

A 3-year-old boy of Ghanaian ethnicity came to the emergency department acutely ill, with fever, chest pain, and a heavy cough. He was accompanied by his parents, who said that he seemed to have a mild cold and slight fever. However, his condition had become more serious in the last 24 hours. His temperature was 103°F. His parent informed the emergency department staff that he has a diagnosis of sickle cell anemia, but they thought that this episode was different from his previous crisis episodes. A CBC was ordered and, because of his cough, he was helped to cough up a sputum sample for culture. He was given ibuprofen for pain and fever. Four hours from the time he was seen in the emergency department, he was admitted and put in the critical care unit, in grave condition. His breathing was compromised and he was placed on mechanical ventilation and lapsed into a coma. He developed disseminated intravascular coagulation (DIC), using 10 units of fresh frozen plasma, 10 units of platelets, and 20 units of packed cells to control the bleeding. Twenty-four hours after admission, he died from overwhelming sepsis. Initial results are as follows (see cover for normal values). What role does splenic function play in the management of sickle cell patients?

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124 Part II • Red Cell Disorders

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