Sickle cell anemia: conclusions from a forensic case report of a young African woman who died after anesthesia.
A 20-year old African woman underwent anesthesia for interruption of an unwanted pregnancy. As a consequence of the anesthesia, she went into coma because of an as yet unknown and untested homozygotic state of sickle cell anemia. Her vital functions were maintained for more than 1 year by intensive medicine, but she died finally in multiorgan failure and aspiration pneumonia. Because of the complications under anesthesia and the missing preanesthetic test for hemoglobinopathy, autopsy was conducted in the forensic medicine department and not in the department of pathology. The sickle cell disease was diagnosed by electrophoresis of the blood, by molecular detection of mutation in the hemoglobin gene, as well as by postmortem light and electron microscopy. Sickle cells were found in capillaries of brain, liver, lung, bone marrow, and spleen. Electrophoretic analysis revealed 80.2% HbS in addition to 3.2% HbA2 and 16.6% HbF, whereas no HbA0 could be detected in blood, confirming the homozygosity of sickle cell anemia. Because of sickle cell crisis, occluded blood vessels, and severe brain cortex necrosis, the patient died in spite of reanimation and intensive medicine. This case demonstrates that it is still important to realize the possibility of this disease and diagnostic obstacles even in regions where its manifestation is not endemic, as in Northern and Central Europe.
Hammer
Ulrich
U
Department of Forensic Medicine, Medical Faculty, University of Rostock, Rostock, Germany.
Wegener
Rudolf
R
Nizze
Horst
H
Wöhlke
Gunther
G
Kruse
Christine
C
Dworniczak
Bernd
B
Kühn-Velten
W Nikolaus
WN
Nöldge-Schomburg
Gabriele
G
Hofmockel
Rainer
R
Jonas
Ludwig
L
D000028:Abortion, Induced; D000328:Adult; D000349:Africa; D000755:Anemia, Sickle Cell / Q000150:complications*; D000758:Anesthesia / Q000009:adverse effects*; D004586:Electrophoresis; D017809:Fatal Outcome; D005260:Female; D005554:Forensic Medicine; D006451:Hemoglobin, Sickle; D006454:Hemoglobins / Q000235:genetics; D006801:Humans; D046529:Microscopy, Electron, Transmission; D009154:Mutation; D011247:Pregnancy
D006451:Hemoglobin, Sickle; D006454:Hemoglobins