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HELLP syndrome and its relationship with antiphospholipid syndrome and antiphospholipid antibodies
Seminars in Arthritis and Rheumatism, 08/30/2011  Clinical Article

Appenzeller S et al. – The incidence of obstetric events in patients with APS is a matter of great interest among rheumatology and gynecology and obstetrics professionals. The current knowledge that antiphospholipid antibodies/APS is not only a thrombotic disease, but also associated with microangiopathic features, can explain the greater prevalence of HELLP syndrome in these patients.

Methods

  • Literature search in PubMed using following MeSH entry terms: HELLP syndrome, anticardiolipin antibodies, lupus anticoagulant, antiphospholipid antibodies, and antiphospholipid syndrome
  • Limited search to articles published in English literature from 1994 to 2010

Results
  • Identified 29 case reports/studies including total of 51 pregnancies with HELLP syndrome in 50 patients
  • Majority of cases occurred during 28 to 36 weeks of pregnancy
  • Nausea, vomiting, epigastric, or right upper quadrant pain was most frequently reported symptoms at disease onset
  • Elevated liver enzymes and low platelet count reported in all studies
  • Concomitant hypertension and proteinuria reported in 2/3 of the patients
  • Hepatic infarctions observed in 33.3% pregnancies
  • Thrombosis was also reported in CNS, deep or superficial vein thrombosis, skin, intestine, bone, spleen, and adrenal glands
  • Treatment is still a matter of debate in HELLP syndrome
  • Aspirin, subcutaneous, intravenous, and oral anticoagulation, and prednisone have been used
  • In addition to use of plasma exchange and fresh frozen plasma administration, intravenous immunoglobulins and plasmapheresis have been described

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