Skip to main content
Log in

Association of HELLP syndrome with primary antiphospholipid syndrome—a case report

  • Case Report
  • Published:
Clinical Rheumatology Aims and scope Submit manuscript

Abstract

Authors present the first Hungarian case of a young pregnant woman with the association of antiphospholipid syndrome (APS) and hemolysis, elevated liver enzymes, and low platelet count (HELLP) syndrome. After the onset of severe preeclampsia, the pregnancy was terminated but the patient’s condition continued to worsen. New symptoms of APS, including deep vein thrombosis and ischemic nervus opticus lesion, developed in the patient followed by the onset of acute respiratory distress syndrome, which required respiratory therapy. Intensive treatment with plasmapheresis, high-dose intravenous immunoglobulin, high-dose corticosteroids, cyclophosphamide, and anticoagulants eventually led to full recovery. There have been only few scattered reports in the literature on the association of HELLP syndrome and APS, which was successfully managed with the combination of various immunosuppressive and immunomodulatory treatment modalities.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

References

  1. Alsulyman OM, Castro MA, Zuckerman E, McGehee W, Goodwin TM (1996) Preeclampsia and liver infarction in early pregnancy associated with the antiphospholipid syndrome. Obstet Gynecol 88:644–646

    Article  PubMed  CAS  Google Scholar 

  2. Asherson RA (1992) The catastrophic antifoszfolipid syndrome. J Rheumatol 19:508–512

    PubMed  CAS  Google Scholar 

  3. Bick RL, Baker WF (1999) Antiphospholipid syndrome and thrombosis. Semin Thromb Haemost 25:333–350

    Article  CAS  Google Scholar 

  4. Fehr T, Cathomas G, Weber C, Fontana A, Schaffner A (2001) Fetal loss, liver necrosis and acute lupus erythematosus in a patient with antiphospholipid antibody syndrome. Lupus 10:576–579

    Article  PubMed  CAS  Google Scholar 

  5. Nagayama K, Izumi N, Miyasaka Y, Saito K, Ono K, Noguchi O (1997) Hemolysis, elevated liver enzymes, and low platelets syndrome associated with primary anti-phospholipid antibody syndrome. Intern Med 9:661–666

    Google Scholar 

  6. Neuwelt CM, Daikh DI, Linfoot JA, Pfister DA, Young RG, Webb RL (1997) Catastrophic antiphospholipid syndrome: response to repeated plasmapheresis over three years. Arthritis Rheum 40:1534–1539

    Article  PubMed  CAS  Google Scholar 

  7. Ornstein MH, Rand JH (1994) An association between refractory HELLP syndrome and antiphospholipid antibodies during pregnancy, a report of 2 cases. J Rheumatol 7:1360–1364

    Google Scholar 

  8. van Pampus MG, Wolf H, Mayruhu G, Treffers PE, Bleker OP (2001) Long-term follow-up in patients with a history of (H)ELLP syndrome. Hypertens Pregnancy 20:15–23

    Article  PubMed  Google Scholar 

  9. Segal S, Shenhav S, Segal O, Zohav E, Gemer O (1996) Budd–Chiari syndrome complicating severe preeclampsia in a parturient with primary antiphospholipid syndrome. Eur J Obstet Gynecol Reprod Biol 68:227–229

    Article  PubMed  CAS  Google Scholar 

  10. Sinha J, Chowdhry I, Sedan S, Barland P (2002) Bone marrow necrosis and refractory HELLP syndrome in a patient catastrophic antiphospholipid antibody syndrome. J Rheumatol 29:195–197

    PubMed  Google Scholar 

  11. Soltesz P, Szekanecz Z, Vegh J, Lakos G, Toth L, Szakall S (2000) Catastrophic antiphospholipid antibody syndrome in cancer. Haematologia 30:303–311

    Article  PubMed  CAS  Google Scholar 

  12. Tsokos M, Longauer F, Kardosova V, Gavel A, Anders S, Schulz F (2002) Maternal death in pregnancy from HELLP syndrome. A report of three medico-legal autopsy cases with special reference to distinctive histopathological alterations. Int J Legal Med 116:50–53

    Article  PubMed  CAS  Google Scholar 

  13. Veres K, Papp K, Lakos G, Szomjak E, Szegedi G, Soltesz P (2003) Primer antifoszfolipid szindróma és HELLP szindróma együttes előfordulása. Orv Hetil 144:1353–1356

    PubMed  Google Scholar 

  14. Wilson WA, Gharavi AE, Koike T, Lockshin MD, Branch DW, Piette JC (1999) International consensus statement on preliminary classification criteria for definite antiphospholipid syndrome: report of an international workshop. Arthritis Rheum 42:1309–1311

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Pál Soltész.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Veres, K., Papp, K., Lakos, G. et al. Association of HELLP syndrome with primary antiphospholipid syndrome—a case report. Clin Rheumatol 27, 111–113 (2008). https://doi.org/10.1007/s10067-007-0687-x

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10067-007-0687-x

Keywords

Navigation