Rheumatology International

, Volume 38, Issue 10, pp 1933–1940 | Cite as

Lupus anticoagulant hypoprothrombinemia syndrome associated with systemic lupus erythematosus in children: report of two cases and systematic review of the literature

  • Rakesh Kumar Pilania
  • Deepti SuriEmail author
  • Ankur Kumar Jindal
  • Narender Kumar
  • Avinash Sharma
  • Praveen Sharma
  • Sandesh Guleria
  • Amit Rawat
  • Jasmina Ahluwalia
  • Surjit Singh
Case Based Review


We report two children with systemic lupus erythematosus (SLE) having severe bleeding manifestations and lupus anticoagulant hypoprothrombinemia syndrome (LAHPS) along with a review of published cases of childhood SLE and LAHPS. We report clinical and laboratory profile of two children diagnosed with childhood SLE and LAHPS. We also conducted literature search to identify similar published cases and a review was performed. An 8-year-old girl had presented with fever, arthralgia, alopecia, anasarca and bleeding from multiple sites. She was diagnosed to have SLE based on laboratory investigations which showed anemia, thrombocytopenia, low complements, positive anti-nuclear antibody (ANA) and double standard DNA (dsDNA) antibodies. She was also found to have prolonged prothrombin time (PT) and activated partial thromboplastin time (aPTT), positive lupus anticoagulant (LA) and low factor II levels. She was diagnosed to have SLE with LAHPS and treated with intravenous methylprednisolone, intravenous immunoglobulin and cyclophosphamide with good outcome. Patient 2 was a 7-year-old-boy who was diagnosed to have SLE when he presented with fever, anasarca, malar rash, arthritis and bleeding from skin and mucosa. Laboratory investigations revealed anemia, proteinuria, low complements, positive ANA and anti-dsDNA titre. Coagulation studies showed deranged PT and aPTT, positive LA and low factor II levels. He was diagnosed to have SLE with LAHPS and was treated with intravenous methylprednisolone and oral mycophenolate mofetil. Review of literature of cases with childhood SLE and LAHPS showed that there are 32 cases have been reported till date which have been summarized. LAHPS is an uncommonly identified cause of bleeding in patients with SLE and must be suspected while evaluating these children.


Child Systemic lupus erythematosus Factor II deficiency Hypoprothrombinemia Lupus anticoagulant hypoprothrombinemia syndrome 


Author contributions

RKP: writing of initial draft of manuscript, editing and revision of manuscript at all stages of its production, review of literature and management of patients. AKJ/AS/PS/SG: contributed to editing of manuscript, review of literature and management of patients. NK/AR/JA/SS: critically revised the manuscript, review of literature and management of patients. DS: critically revised the manuscript at all stages of its production, final approval of manuscript, review of literature and management of patients.

Compliance with ethical standards

Conflict of interest

All authors declare no conflict of interest.

Ethical standards

The study complies with the Declaration of Helsinki. The study was approved by Department Review Board (DRB), Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

Informed consent

An informed consent was obtained from parents of the patients for publication.


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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Pediatric Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics CentrePostgraduate Institute of Medical Education and ResearchChandigarhIndia
  2. 2.Department of HematologyPostgraduate Institute of Medical Education and ResearchChandigarhIndia

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