The Patient with Sickle Cell Anemia

  • Gary Hartstein


A 31-year-old black man, an intravenous cocaine abuser with a history of sickle cell anemia (SCA), presented to the hospital with headache, stiff neck, and left hemiparesis of 5 days’ duration.

The patient was last admitted to the hospital 7 years earlier for painful sickle cell crisis, and was reportedly transfused at that time. He last used cocaine intravenously 2 weeks before this admission. He had no allergies, took no medication, and had only occasional sickle crises (2–3 per year, requiring only analgesics).

The patient was thin, drowsy but arousable, and in no acute distress. Vital signs were: temperature 100.3°F, pulse 48/min, blood pressure 130/80 mmHg, respiratory rate 18/ min. Mild scleral icterus, nuchal rigidity, and positive Kernig’s and Brudzinski’s signs were noted, as was a right carotid bruit, a III /VI systolic murmur, and digital clubbing. A left hemiparesis with decreased appreciation of light touch and pinprick sensation on the left were also present.

Laboratory studies showed white blood cell count 24,000, hemoglobin 8.3 gm/dl, hematocrit 25.3%, and SMA-6 within normal limits. SMA-12 was significant for total: direct bilirubin 6.3:1.5, LDH 404. Lumbar puncture showed elevated opening pressure, xanthochromia, and many red cells. CT scan of the head showed multiple right parietooccipital infarcts. Carotid angiography revealed right carotid occlusion and a left posterior cerebral artery aneurysm. Hemoglobin electrophoresis was not performed.

The patient was scheduled for urgent clipping of a posterior cerebral artery aneurysm.


Sickle Cell Sickle Cell Trait Sickle Cell Anemia Patient Mean Corpuscular Hemoglobin Concentration Carotid Bruit 
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© Birkhäuser Boston Inc. 1989

Authors and Affiliations

  • Gary Hartstein

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