Question

\A 5-year-old African-American male with a known history of sickle cell disease presented to
an outside hospital with 3 days of intermittent fevers and headache. The mother reported
that he had had one episode of vomiting, decreased oral intake, and fatigue. The patient also
complained of occasional abdominal and foot pain. At the outside hospital, his complete
blood count showed numerous nucleated red blood cells (RBCs), a corrected white blood cell
(WBC) count of 75,000/ul (normal, 5,000 to 14,500), a hemoglobin level of 3.1 g/dl (normal,
11.5 to 13.5), and a platelet count of 962,000/ul (normal, 150,000 to 440,000). The patient's
baseline hemoglobin was 6 to 8 g/a. After blood cultures were obtained, he was given
ceftriaxone before transfer to our institution. He had had three prior hospitalizations, all for
fever and upper respiratory viral syndromes. The patient had been on penicillin since early
infancy and was up to date on vaccinations, including pneumococcus and Haemophilus
influenzae type b vaccines. Upon admission at our institution, the patient continued to be
anemic, with a hemoglobin level of 2.5 g/dl and a hematocrit of 7.4% (normal, 34 to 40%).
He subsequently received two transfusions of packed RBCs, which brought his hemoglobin
up to 5.3 g/dl. Although blood cultures were negative to date, he was continued on
ceftriaxone and vancomycin was added. A positive PCR result was obtained, which confi
rmed this patient's infection.
1. What syndrome did this patient have? What is the etiology of his infection? Why were his
WBC and platelet counts so high?
2. How does infection with this organism normally present in childhood? Are there any
differences in presentation when primary infection occurs in an adult?
3. This patient had sickle cell disease, which predisposed him to this infection as well as
other infections. What infections are patients with sickle cell disease more susceptible to,
and why? Why had he received life-long penicillin G therapy?
4. What other patient populations are at risk for severe infections due to this organism?
5. What methods are available to diagnose this infection? Include a discussion of their
strengths and weaknesses.​

Answer 1

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Answer 2

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