HELLP Syndrome and Its Relationship with Antiphospholipid Syndrome and Antiphospholipid Antibodies

• Published in: Seminars in arthritis and rheumatism Vol. 41; no. 3; pp. 517 - 523
• Main Authors: Appenzeller, Simone, Souza, Fernando H.C., Wagner Silva de Souza, Alexandre, Shoenfeld, Yehuda, de Carvalho, Jozélio Freire
• Format: Journal Article
• Language: English
• Published: New York, NY Elsevier Inc 01.12.2011; Elsevier
• Subjects: Antibodies, Antiphospholipid - immunology; antiphospholipid syndrome; Antiphospholipid Syndrome - complications; Antiphospholipid Syndrome - immunology; Biological and medical sciences; Diseases of mother, fetus and pregnancy; Diseases of the osteoarticular system; eclampsia; Female; Gynecology. Andrology. Obstetrics; HELLP syndrome; HELLP Syndrome - etiology; HELLP Syndrome - immunology; Hematologic and hematopoietic diseases; Humans; Medical sciences; placental insufficiency; Platelet diseases and coagulopathies; Pregnancy; pregnancy loss; Pregnancy. Fetus. Placenta; Rheumatology; antiphospholipid syndrome; eclampsia; pregnancy loss; placental insufficiency; HELLP syndrome; Immunopathology; Antiphospholipid antibody syndrome; Pregnancy disorders; Rheumatology; Autoimmune disease; Cardiovascular disease; Placental insufficiency; Hemopathy; Placenta diseases; Pregnancy toxemia; Vascular disease; Pregnancy; Platelet; Eclampsia
• ISSN: 0049-0172; 1532-866X; 1532-866X
• DOI: 10.1016/j.semarthrit.2011.05.007

To perform a systematic review of the association between antiphospholipid antibodies, antiphospholipid syndrome (APS), and HELLP syndrome (Hemolysis; Elevated Liver enzymes; Low Platelet count), describing clinical features, outcome, pathophysiological findings, and treatment. We performed a literature search in PubMed using the following MeSH entry terms: HELLP syndrome, anticardiolipin antibodies, lupus anticoagulant, antiphospholipid antibodies, and antiphospholipid syndrome. We limited our search to articles published in the English literature from 1994 to 2010. We identified 29 case reports/studies including a total of 51 pregnancies with HELLP syndrome in 50 patients. The majority of the cases occurred during the 28 to 36 weeks of pregnancy. Nausea, vomiting, epigastric, or right upper quadrant pain was the most frequently reported symptoms at disease onset. Elevated liver enzymes and low platelet count were reported in all studies. Concomitant hypertension and proteinuria were reported in 2/3 of the patients. Hepatic infarctions were observed in 33.3% pregnancies. However, thrombosis was also reported in the central nervous system, 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 the use of plasma exchange and fresh frozen plasma administration, intravenous immunoglobulins and plasmapheresis have been described. 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.