CASE PRESENTATION: A 27-year-old Caucasian woman who was 15+1 weeks pregnant reported to the emergency department twice due to stabbing right-upper-quadrant
abdominal pain. Initial laboratory testing revealed mild
leukocytosis and slightly elevated liver
enzymes. On second presentation, the patient was febrile and had an increased
C-reactive protein concentration. Over the course of the next days, nonhemolytic
anemia and
thrombocytopenia emerged with elevated liver
enzymes. Coagulation studies also revealed a prolongation of activated partial thromboplastin time. Magnetic resonance imaging showed nonspecific alterations in the right liver lobe, possibly corresponding to
infection or
infarction. A hepatic
viral infection was ruled out. At that time, the most likely diagnosis was
cholangitis with
liver abscess formation, and
antibiotic therapy was started. Further worsening of the
anemia and
thrombocytopenia, development of
proteinuria, together with a
miscarriage on the fourth day of hospitalization resulted in the tentative diagnosis of (triple-positive)
antiphospholipid antibody syndrome, which was confirmed 12 weeks after the initial investigation. Treatment consisted of prompt anticoagulation with
heparin and later on with a
vitamin K antagonist as well as high-dose
glucocorticoid therapy. There was no need for
intravenous immunoglobulin therapy or
plasma exchange, although we suspected a catastrophic form of
antiphospholipid antibody syndrome due to
infarctions of the liver, placenta, and possibly kidneys (
proteinuria). The outcome was favorable.
CONCLUSION: