Postmortem diagnosis of hemoglobin SC disease complicated by fat embolism.
A case is reported of a previously healthy 52-year-old African American male who presented with acute onset of abdominal pain. Progressive increase in his abdominal symptoms led to an exploratory laparotomy; however, no pathology was discovered. Postoperatively, the patient became hypoxemic which progressed to diffuse infiltrates on chest x-ray, suggestive of adult respiratory distress syndrome. He had a rapidly fatal course. Autopsy showed bone marrow infarction, fat embolism, splenomegaly, and widespread congestion with sickle erythrocytes. Hemoglobin electrophoresis done postmortem showed hemoglobin (Hb) SC disease that was undiagnosed antemortem. To the best of our knowledge, it is unusual for Hb SC to be diagnosed postmortem in adults. This case suggests that sickle cell disorders should be ruled out in patients at risk for hemoglobinopathy in the presence of signs and symptoms compatible with the disease, irrespective of age.
Ballas
S K
SK
Cardeza Foundation for Hematologic Research, Department of Medicine, Jefferson Medical College, Philadelphia, PA 19107, USA.
Pindzola
A
A
Chang
C D
CD
Rubin
R
R
Weibel
S B
SB
Manci
E
E
D001853:Bone Marrow / Q000473:pathology; D004620:Embolism, Fat / Q000175:diagnosis / Q000209:etiology* / Q000473:pathology; D017809:Fatal Outcome; D006450:Hemoglobin SC Disease / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D009336:Necrosis; D013163:Splenomegaly