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Semin Thromb Hemost
DOI: 10.1055/a-2657-6291
Review Article

Lupus Anticoagulant-hypoprothrombinemia Syndrome: A Review Enriched by a New Particular Patient

Doris Barcellona
1   Azienda Ospedaliero-Universitaria of Cagliari, Cagliari, Italy
2   Department of Medical Science and Public Health, University of Cagliari, Cagliari, Italy
3   Fondazione Arianna, Anticoagulazione.it, Bologna, Italy
,
Antonella Mameli
1   Azienda Ospedaliero-Universitaria of Cagliari, Cagliari, Italy
,
Roberta Montisci
1   Azienda Ospedaliero-Universitaria of Cagliari, Cagliari, Italy
,
Patol Maria Filomena Clin Ruberto
1   Azienda Ospedaliero-Universitaria of Cagliari, Cagliari, Italy
,
Patol Lara Clin Fenu
1   Azienda Ospedaliero-Universitaria of Cagliari, Cagliari, Italy
,
Francesco Marongiu
1   Azienda Ospedaliero-Universitaria of Cagliari, Cagliari, Italy
4   Department of Medical Science and Public Health, Fondazione Arianna, Anticoagulazione.it, Bologna, Italy
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Abstract

Lupus anticoagulant-hypoprothrombinemia syndrome (LAHS) is a rare hemorrhagic disorder that should be differentiated from classical antiphospholipid syndrome. Literature review shows that LAHS may affect people at any age but approximately 40% are children younger than 10 years. Autoimmune and infectious diseases are the most frequent triggering causes, and the laboratory profile is characterized by a prolongation of prothrombin time (PT) and activated partial thromboplastin time (aPTT) with a mild to severe reduction in factor II levels. In more than half the patients, the other coagulation factors are normal, while anti-cardiolipin and anti-β2-glycoprotein I antibodies show a high titer. Lupus anticoagulant (LA) is positive in 100% of cases, as this represents a defining feature. The majority of patients have mucocutaneous bleeding events (44%); cerebral bleeding can occur in 10% of patients and other common bleeding sites are the gastrointestinal and genitourinary tracts. There is no standard treatment for LAHS. Supportive measures, such as fresh frozen plasma, packed red blood, and platelet transfusion, are frequently administered in association with steroids alone or in combination with intravenous immunoglobulin or cyclophosphamide, azathioprine, and rituximab. Death, recurrent bleeding, and thrombosis can occur in approximately 3, 13, and 14% of patients, respectively. Our patient was an old man with a myocardial infarction and a systemic infection from Candida parapsilosis. Thrombin generation and clot waveform analysis were performed before and after treatment. Thrombin generation better reflected the role of prothrombin, revealing that a factor II value of below around 15% can represent a risk for major bleeding. Treatment with methylprednisolone and three-factor human prothrombin complex concentrate allowed the patient to reach a complete recovery 1 month after initial diagnosis.

Keywords

LA-hypoprothrombinemia syndrome - bleeding management - thrombin generation - clot waveform analysis


Publication History

Received: 21 April 2025

Accepted: 16 July 2025

Article published online:
25 July 2025

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