Diagnosis of lupus anticoagulant in the lupus anticoagulant‐hypoprothrombinemia syndrome: Report of two cases and review of the literature

Baca, Vicente; Montiel, Guadalupe; Meillón, Luis; Pizzuto, J; Catalán, Teresa; Juan-Shum, Luis; Nieva, Beatriz

doi:10.1002/ajh.10194

Cited by 52 publications

(47 citation statements)

References 39 publications

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“…The bleeding complications observed in patients with the Lupus anticoagulant-hypoprothrombinemia syndrome are usually seen with FII activity levels <30% [18]. The lowest FII activity level reported in our patient, who had a long history of recurrent bleeding, was 34%.…”

Section: Discussionmentioning

confidence: 86%

Approach to the treatment, characterization and diagnosis of an acquired auto‐antibody directed against factors prothrombin, factor X and factor IX: a case report and review of the literature

et al. 2011

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Bleeding disorders secondary to acquired non-inhibitory antibodies directed against vitamin K-dependent coagulation proteins are rare. In this report, the authors describe a patient with a low grade lymphoma who presented with a fatal acquired bleeding manifestation and abnormal hemostatic studies resulting from deficiencies in both prothrombin and factor X. Patient plasma samples were collected and studied for the presence of an acquired inhibitor. Levels of plasma coagulation proteins were measured using immunoassay. Patient anti-prothrombin immunoglobulin G was isolated and binding to prothrombin, prothrombin F1.2, factors IX and X was evaluated using immunoblots and competition immunoassay. Prolongation in the prothrombin time and activated partial thromboplastin time suggested a factor deficiency in the common pathway of coagulation. Functional and antigenic levels of both prothrombin and factor X were decreased. An IgG subtype-4 antibody was isolated from patient plasma using affinity chromatography on prothrombin-sepharose. This antibody was found to bind to a common metal-ion-dependent conformational epitope found on the γ-carboxyglutamic acid (Gla) domain of prothrombin, factor X and factor IX. This report represents the first description of an acquired bleeding disorder resulting from a unique cross-reactive auto-antibody against a common metal-ion-dependent antigenic structure on the Gla-domain of the vitamin K-dependent proteins.

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Section: Discussionmentioning

confidence: 86%

Approach to the treatment, characterization and diagnosis of an acquired auto‐antibody directed against factors prothrombin, factor X and factor IX: a case report and review of the literature

et al. 2011

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“…Significant prolongation of the PT or aPTT requires further evaluation for autoantibodies against coagulation factors10). Management may require the use of intravenous steroids, immunoglobulin and immunosuppressive agents1,6,9).…”

Section: Discussionmentioning

confidence: 99%

Pulmonary hemorrhage in pediatric lupus anticoagulant hypoprothrombinemia syndrome

Kim

Bae

et al. 2014

Korean J Pediatr

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Lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS), a very rare disease that is caused by the presence of antifactor II antibodies, is usually counterbalanced by the prothrombotic effect of lupus anticoagulant (LAC). Patients with LAHPS are treated using fresh frozen plasma, steroids, immunosuppressive agents, and immunoglobulins for managing the disease and controlling hemorrhages. Notably, steroids are the important treatment for treating hypoprothrombinemia and controlling the bleeding. However, some patients suffer from severe, life-threatening hemorrhages, when factor II levels remain very low in spite of treatment with steroids. Here, we report a case of LAHPS in a 15-year-old girl who experienced pulmonary hemorrhage with rapid progression. She was referred to our hospital owing to easy bruising and prolonged bleeding. She was diagnosed with LAHPS that presented with pancytopenia, positive antinuclear antibody, proloned prothrombin time, activated partial thromboplastin time, positive LAC antibody, and factor II deficiency. Her treatment included massive blood transfusion, high-dose methylprednisolone, vitamin K, and immunoglobulin. However, she died due to uncontrolled pulmonary hemorrhage.

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“…Prothrombin antibodies are classically associated with lupus inhibitors [3][4][5][6] but antibodies to other clotting factors such as factors VII, VIII and X are described [7][8][9].…”

Section: Discussionmentioning

confidence: 99%

Life-threatening bleeding in a patient with a lupus inhibitor and probable acquired factor VII deficiency

Lim¹,

Zuha²,

Burt³

et al. 2006

Blood Coagulation &Amp; Fibrinolysis

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We report the case of a 71-year-old man on warfarin for chronic atrial fibrillation presenting with a massive spontaneous soft tissue bleed. Despite reversing the effects of warfarin with large doses of intravenous vitamin K and fresh frozen plasma, bleeding continued, and his prothrombin time and activated partial thromboplastin time remained prolonged. The prothrombin time and activated partial thromboplastin time failed to correct with 50% normal plasma. Further investigations confirmed a lupus inhibitor with low levels of factors II, V, VII and XI. Factor II, V and XI levels normalized, however, when the patient's plasma was diluted 1:16 in buffer, suggesting the lupus inhibitor may have been interfering with these factor assays causing artefactual low results. Factor VII levels remained consistently low at all dilutions. The patient subsequently died following a massive left haemothorax despite surgical intervention and treatment with activated recombinant factor VII concentrate. We presumed the primary problem was bleeding from a local vascular lesion but the patient was never well enough to undergo confirmatory angiography. This case highlights the fact that patients with lupus inhibitors can develop severe haemorrhagic complications, and illustrates the complexities involved in both the investigation and treatment of abnormal bleeding in these patients.

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Diagnosis of lupus anticoagulant in the lupus anticoagulant‐hypoprothrombinemia syndrome: Report of two cases and review of the literature

Cited by 52 publications

References 39 publications

Approach to the treatment, characterization and diagnosis of an acquired auto‐antibody directed against factors prothrombin, factor X and factor IX: a case report and review of the literature

Approach to the treatment, characterization and diagnosis of an acquired auto‐antibody directed against factors prothrombin, factor X and factor IX: a case report and review of the literature

Pulmonary hemorrhage in pediatric lupus anticoagulant hypoprothrombinemia syndrome

Life-threatening bleeding in a patient with a lupus inhibitor and probable acquired factor VII deficiency

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