RESUMEN
BACKGROUND: Lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS) is a rare disease caused by acquired factor II (FII) deficiency and lupus anticoagulant. Patients with LAHPS typically present with thrombosis and bleeding. However, little information is available on the evaluation of coagulation potential in patients with LAHPS. We examined global coagulation potentials in patients with LAHPS during the clinical course in this study. METHODS: Coagulation potentials in two pediatric patients with LAHPS were assessed by measuring clotting time (CT) and clot formation time using Ca2+-triggered rotational thromboelastometry (ROTEM), CT and maximum coagulation velocity using clot waveform analysis (CWA), and lag time and peak thrombin using the thrombin generation assay (TGA). The day of admission was defined as day 0. RESULTS: In case 1, the bleeding symptoms disappeared by day 5. However, the TGA and CWA results were markedly lower than normal, although FII activity (FII:C) returned to within the normal range by day 14. In contrast, ROTEM revealed a recovery to near-normal levels (day 14). All coagulation parameters (day 80) were within normal ranges. In case 2, coagulation potential was severely depressed until day 12, although FII:C returned to normal levels. Bleeding symptoms disappeared on day 19, and the ROTEM data revealed that the parameters were close to the normal range. The coagulation parameters in all assays were normalized on day 75. CONCLUSIONS: Recovery of coagulation potential in patients with LAHPS was slower than the recovery of FII:C. Moreover, ROTEM appeared to be clinically useful for assessing coagulation potential in patients with LAHPS.
Asunto(s)
Hipoprotrombinemias , Inhibidor de Coagulación del Lupus , Tromboelastografía , Humanos , Hipoprotrombinemias/sangre , Hipoprotrombinemias/diagnóstico , Inhibidor de Coagulación del Lupus/sangre , Femenino , Tromboelastografía/métodos , Masculino , Niño , Pruebas de Coagulación Sanguínea/métodos , Coagulación Sanguínea/fisiología , Preescolar , Síndrome Antifosfolípido/sangre , Síndrome Antifosfolípido/complicaciones , Síndrome Antifosfolípido/diagnósticoAsunto(s)
Síndrome Antifosfolípido , Hipoprotrombinemias , Lupus Eritematoso Sistémico , Infecciones por Mycoplasma , Masculino , Humanos , Inhibidor de Coagulación del Lupus , Hipoprotrombinemias/complicaciones , Hipoprotrombinemias/diagnóstico , Síndrome Antifosfolípido/complicaciones , Síndrome Antifosfolípido/diagnóstico , Infecciones por Mycoplasma/complicaciones , Infecciones por Mycoplasma/diagnóstico , ProtrombinaRESUMEN
OBJECTIVE: Children with lupus anticoagulant hypoprothrombinemia syndrome (LAHPS) are characterized by prolonged activated partial thromboplastin time (APTT) and prothrombin time (PT), lupus anticoagulant positivity and low prothrombin (factor II, FII) levels. Bleeding or thrombosis tendencies related to LAHPS in children can occur due to the development of anti-prothrombin antibodies that are usually linked to autoimmune or infectious diseases. METHODS: We report three pediatric cases of LAHPS and describe details on their clinical symptoms, laboratory characteristics, treatment. PubMed, Medline, and Web of Science searches were conducted on LAHPS in children between 1960 and 2023; articles in English were included. RESULTS: The coagulation profile revealed prolonged PT and APTT, with low prothrombin levels (19.4%, 21.0% and 12.9%, respectively) and positive lupus anticoagulant in 3 pediatric cases. Fifty-nine relevant articles reported 93 pediatric LAHPS cases (mean age: 9 years (0.8-17 years)); 63 females and 30 males, 87 patients presented with minor to severe bleeding diathesis, and 3 patients presented with thrombosis events. Among 48 patients ≥9 years old, 36 had SLE; among 45 patients <9 years, 29 had viral infection. When all patients were divided into two groups based on age, associated disease, and factor II level, Pearson's χ2 tests were performed, p =.00, and there was clinical significance between autoimmune and infectious disease in patients ≥9 years old and <9 years old, and in patients FII level ≤10% and >10%. LAHPS patients with autoimmune disease had a protracted course and needed prolonged treatment with immune-modulating therapy, while those patients with infectious disease resolved spontaneously or needed short-term immune-modulating therapy. CONCLUSION: LAHPS caused by autoimmune disease are common in patients ≥9 years old, especially SLE, and FII level ≤10% is often reported in patients caused by autoimmune disease, suggesting that children ≥9 years old diagnosed with LAHPS-related autoimmune disease should pay special attention to the FII level. While LAHPS caused by infectious disease is more frequently observed in patients <9 years, especially viral infection. Early diagnostic investigations are critical to differentiating LAHPS caused by autoimmune or infectious disease, as the prognosis, treatment and outcome are distinct.
Asunto(s)
Síndrome Antifosfolípido , Enfermedades Autoinmunes , Hipoprotrombinemias , Lupus Eritematoso Sistémico , Femenino , Masculino , Humanos , Niño , Preescolar , Hipoprotrombinemias/diagnóstico , Inhibidor de Coagulación del Lupus , Protrombina , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/diagnóstico , Enfermedades Autoinmunes/diagnósticoRESUMEN
Lupus anti-coagulant hypoprothrombinemia syndrome (LAHPS) is a rare condition that can be difficult to treat. It increases the risk of thrombosis and bleeding due to the presence of lupus anti-coagulant and factor II deficiency, respectively. There are a limited number of cases described in the literature. Herein we describe a case of LAHPS with bleeding symptoms as a first clinical manifestation of systemic lupus erythematosus (SLE) in an 8-year-old female. She has had multiple recurrences of her bleeding symptoms, requiring treatment with steroids, cyclophosphamide, mycophenolate mofetil, and rituximab. Her course was later complicated by development of arthritis and lupus nephritis. Her complicated course provides a new perspective on the clinical course and treatment of LAHPS. We also present a comprehensive literature review which demonstrates the difficulty in treating patients with LAHPS with underlying SLE and the variability of the clinical course and management of LAHPS depending on the age at presentation.
Asunto(s)
Hipoprotrombinemias , Lupus Eritematoso Sistémico , Humanos , Femenino , Niño , Hipoprotrombinemias/complicaciones , Hipoprotrombinemias/diagnóstico , Hipoprotrombinemias/tratamiento farmacológico , Inhibidor de Coagulación del Lupus , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/diagnóstico , Lupus Eritematoso Sistémico/tratamiento farmacológico , Ciclofosfamida/uso terapéutico , Hemorragia/complicaciones , Progresión de la EnfermedadRESUMEN
Lupus anticoagulant-hypoprothrombinaemia syndrome (LAHPS) is a rare disorder caused by the presence of lupus anticoagulant (LA) and acquired prothrombin deficiency, which may present with severe haemorrhagic manifestations. LAHPS is usually associated with systemic lupus erythematosus (SLE), or infections and it is more frequent in the paediatric population and female gender. We describe a 42-year-old man with thrombotic antiphospholipid syndrome (APS) on chronic anticoagulation treatment with acenocoumarol who presented with spontaneous intracranial bleeding, prolongation of prothrombin time (PT), activated partial thromboplastin time (APTT) and low factor II levels (after optimal anticoagulation reversal) as a debut of SLE.
Asunto(s)
Síndrome Antifosfolípido , Hipoprotrombinemias , Lupus Eritematoso Sistémico , Masculino , Niño , Femenino , Humanos , Adulto , Síndrome Antifosfolípido/complicaciones , Síndrome Antifosfolípido/diagnóstico , Inhibidor de Coagulación del Lupus , Hipoprotrombinemias/complicaciones , Hipoprotrombinemias/diagnóstico , Lupus Eritematoso Sistémico/complicaciones , Protrombina , HemorragiaAsunto(s)
Síndrome Antifosfolípido , Hipoprotrombinemias , Lupus Eritematoso Sistémico , Humanos , Síndrome Antifosfolípido/complicaciones , Síndrome Antifosfolípido/diagnóstico , Inhibidor de Coagulación del Lupus , Hipoprotrombinemias/diagnóstico , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/diagnósticoRESUMEN
Hypoprothrombinemia-lupus anticoagulant syndrome (HLAS) is a rare disease in which patients present with varying degrees of bleeding and positive lupus anticoagulant with reduced prothrombin on laboratory tests. This article reports a case of HLAS in a middle-aged woman with recurrent gingival bleeding and epistaxis as the first presentation. After admission, tests revealed prolonged prothrombin time (PT), activated partial thromboplastin time (APTT), and reduced coagulation factor II activity, and positive lupus anticoagulant (LA). Meanwhile, the patient had symptoms of dry mouth and dry eyes for a long time, and the examination of autoantibodies, tear secretion test and salivary gland emission computed tomography (ECT) were consistent with the diagnosis of Sjogren's syndrome. The final diagnosis was HLAS secondary to Sjogren's syndrome. After treatment with methylprednisolone and cyclophosphamide, the coagulation disorder gradually improved, and no recurrent bleeding occurred. HLAS is a rare clinical case, which reminds medical staff to be alert to the possibility of HLAS when encountering patients with unexplained prolonged APTT and PT and positive lupus anticoagulant.
Asunto(s)
Trastornos de la Coagulación Sanguínea Heredados , Hipoprotrombinemias , Síndrome de Sjögren , Persona de Mediana Edad , Femenino , Humanos , Hipoprotrombinemias/complicaciones , Hipoprotrombinemias/diagnóstico , Síndrome de Sjögren/complicaciones , Síndrome de Sjögren/diagnóstico , Inhibidor de Coagulación del Lupus , AutoanticuerposRESUMEN
BACKGROUND Lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS) is an exceptionally rare disease caused by prothrombin antibodies, resulting in reduced factor II levels. This disease can present with significant bleeding and is usually associated with autoimmune disorders, particularly systemic lupus erythematosus (SLE). There are currently no guidelines for the treatment of LAHPS, and corticosteroids remain the criterion standard therapy. Pseudotumor cerebri is a disease that involves an idiopathic rise in intracranial pressure in association with papilledema. The coexistence of pseudotumor cerebri with SLE is rare, with an overall incidence of 0.7%. CASE REPORT A 16-year-old male initially presented to our hospital with nausea, headaches, and decreased visual acuity. He was diagnosed with pseudotumor cerebri based on the findings of papilledema and a raised opening pressure on lumbar puncture. Three months later, he presented with macroscopic hematuria and persistent epistaxis. Further investigation revealed a prolonged activated partial thromboplastin time and prothrombin time, along with positive LA and reduced Factor II levels, resulting in a diagnosis of LAHPS. The patient received a dose of 1 mg/kg/day of prednisolone along with hydroxychloroquine, and he had a complete recovery with cessation of bleeding and normalization of laboratory parameters. CONCLUSIONS We are reporting a case of pseudotumor cerebri with a further presentation of LAHPS in a patient found to have SLE. As both associations are rare in the presence of SLE, it is vital to recognize them early to initiate adequate management and intervention to avoid life-threatening complications.
Asunto(s)
Síndrome Antifosfolípido , Hipoprotrombinemias , Lupus Eritematoso Sistémico , Papiledema , Seudotumor Cerebral , Adolescente , Corticoesteroides/uso terapéutico , Síndrome Antifosfolípido/complicaciones , Hemorragia , Humanos , Hidroxicloroquina/uso terapéutico , Hipoprotrombinemias/diagnóstico , Hipoprotrombinemias/tratamiento farmacológico , Hipoprotrombinemias/etiología , Inhibidor de Coagulación del Lupus , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/diagnóstico , Lupus Eritematoso Sistémico/tratamiento farmacológico , Masculino , Papiledema/complicaciones , Prednisolona/uso terapéutico , Protrombina/uso terapéutico , Seudotumor Cerebral/complicaciones , Seudotumor Cerebral/etiologíaRESUMEN
A boy, aged 2 years and 5 months, had recurrent epistaxis, and the coagulation function examination showed that activated partial thromboplastin time (APTT) was significantly prolonged. Further laboratory examinations showed that the prolonged APTT was not immediately corrected in the APTT correction test, with positive lupus anticoagulant and low prothrombin activity. The boy was diagnosed with hypoprothrombinemia-lupus anticoagulant syndrome. The condition was improved after treatment with glucocorticoid, immunoglobulin, and vitamin K1. The boy has been followed up for 6 months, and no epistaxis was observed. Prothrombin activity returned to normal, and lupus anticoagulant remained positive. This is a relatively rare disease, and for patients with bleeding symptoms and coagulation disorders, it is recommended to perform the tests such as APTT correction test, lupus anticoagulant testing, and coagulation factor dilution test, which can improve the detection rate of this disease, so as to achieve early diagnosis, provide rational treatment in the early stage, and improve the prognosis.
Asunto(s)
Síndrome Antifosfolípido , Trastornos de la Coagulación Sanguínea , Hipoprotrombinemias , Síndrome Antifosfolípido/diagnóstico , Preescolar , Epistaxis/etiología , Humanos , Hipoprotrombinemias/diagnóstico , Inhibidor de Coagulación del Lupus , Masculino , Tiempo de Tromboplastina Parcial , ProtrombinaRESUMEN
We describe the case of a 50-year-old woman with a history of SLE and APS that presented with a spontaneous subdural haematoma, prolonged aPTT, PT and INR and positive LA. The activity of the coagulation factors II, VIII, IX and XI was extremely low, and anti-prothrombin antibody IgG was positive. LAHS was established, with inhibition of the intrinsic pathway, as an acquired haemophilia. The patient received corticosteroids and cyclophosphamide as treatment. To the best of our knowledge, this is one of the few reports of spontaneous intracranial bleeding, an unusual and initial manifestation of LAHS in an adult patient.HighlightsLAHS is characterised by the presence of LA and hypoprothrombinaemia caused by anti-prothrombin antibodies.Prolonged aPTT and INR, and positive LA are important laboratory findings that help the suspicion of LAHS.Intracranial bleeding is an unusual manifestation of LAHS associated with low factor II activity.Corticosteroids are the first-line treatment of LAHS.The prognosis of LAHS is good with adequate treatment, with a reported mortality of 5%.
Asunto(s)
Síndrome Antifosfolípido , Hipoprotrombinemias , Síndrome Antifosfolípido/diagnóstico , Femenino , Hematoma Subdural/etiología , Humanos , Hipoprotrombinemias/diagnóstico , Inhibidor de Coagulación del Lupus , Persona de Mediana EdadRESUMEN
INTRODUCTION: Lupus anticoagulant hypoprothrombinemia syndrome (LAHPS) is a rare phenomenon that leads to concomitant thrombosis and hemorrhage in children with SLE. LAHPS in pediatric SLE (pSLE) has a protracted course requiring long-term immunosuppressive therapy. Due to the rarity of this syndrome and paucity of reported cases, there is lack of standardized management. We herewith report 5 children with pSLE with LAHPS.Methodology: We retrospectively reviewed clinical features, laboratory features, treatment and outcome for 5 children with lupus anticoagulant hypoprothrombinemia syndrome with SLE and a review of literature of similar cases published. RESULTS: Mean age of presentation was 10.2 ± 2.38 years (mean ± SD) and female to male ratio was 4:1. All children presented with mild to severe bleeding manifestations like gum bleed, epistaxis, hematuria, menorrhagia and subarachnoid bleed. Coagulation profile revealed prolonged PT and aPTT, with low prothrombin levels and positive Lupus anticoagulant in all children. Mixing studies were characteristic in these children. On comparing laboratory parameters majority had low C3, C4 levels, ANA and anti-DsDNA antibody positivity and three children had anticardiolipin positivity. One child had lupus nephritis along with LAHPS at presentation. All responded well to steroids and supportive measures. CONCLUSION: High index of suspicion is needed when child with lupus presents with bleeding manifestations for early diagnosis and treatment.
Asunto(s)
Hemorragia/etiología , Hipoprotrombinemias/complicaciones , Inhibidor de Coagulación del Lupus/sangre , Lupus Eritematoso Sistémico/complicaciones , Trombosis/etiología , Adolescente , Pruebas de Coagulación Sanguínea/estadística & datos numéricos , Transfusión Sanguínea/métodos , Niño , Diagnóstico Precoz , Femenino , Hemorragia/diagnóstico , Humanos , Hipoprotrombinemias/diagnóstico , Hipoprotrombinemias/tratamiento farmacológico , Hipoprotrombinemias/terapia , Inmunosupresores/administración & dosificación , Inmunosupresores/uso terapéutico , Lupus Eritematoso Sistémico/diagnóstico , Masculino , Tiempo de Tromboplastina Parcial/estadística & datos numéricos , Protrombina/análisis , Estudios Retrospectivos , Esteroides/administración & dosificación , Esteroides/uso terapéutico , Trombosis/diagnóstico , Resultado del TratamientoRESUMEN
We herein report an unusual case of lupus with bleeding diathesis in a Chinese adolescent boy. In the presence of lupus anticoagulant and hypoprothrombinemia, the diagnosis of lupus anticoagulant-hypoprothrombinemia syndrome was made. He responded promptly to immunosuppressive agents and achieved disease remission.
Asunto(s)
Hipoprotrombinemias/sangre , Inhibidor de Coagulación del Lupus/sangre , Coagulación Sanguínea , Factores de Coagulación Sanguínea/análisis , Niño , Hemorragia/sangre , Humanos , Hipoprotrombinemias/diagnóstico , MasculinoAsunto(s)
Hipoprotrombinemias/sangre , Inhibidor de Coagulación del Lupus/sangre , Lupus Eritematoso Sistémico/sangre , Adolescente , Femenino , Humanos , Hipoprotrombinemias/complicaciones , Hipoprotrombinemias/diagnóstico , Hipoprotrombinemias/inmunología , Inhibidor de Coagulación del Lupus/inmunología , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/diagnóstico , Lupus Eritematoso Sistémico/inmunología , Menorragia/etiología , Menorragia/inmunología , SíndromeRESUMEN
Lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS) is a rare bleeding disorder caused by antiprothrombin antibodies. LAHPS is associated with systemic lupus erythematosus (SLE) or infections. We describe two Japanese brothers with immunoglobulin-A vasculitis (IgAV) who met the diagnostic criteria of LAHPS. They presented with palpable purpura and abdominal pain, and had a prolonged activated partial thromboplastin time (APTT) and prothrombin deficiency with the presence of lupus anticoagulant. Pediatric LAHPS was reviewed in abstracts from the Japan Medical Abstracts Society that were written in Japanese and PubMed or Web of Science-listed articles in English between 1996 and 2019. Including our cases, pediatric LAHPS has been reported in 40 Japanese and 46 non-Japanese patients. We summarized the clinical and laboratory characteristics of all 86 cases, and found only one Japanese LAHPS case with IgAV, except for our cases. Of the 86 cases, most were associated with infections followed by SLE. The presence of SLE, older age, lower prothrombin levels, severe bleeding symptoms, and positivity of immunoglobulin G anticardiolipin antibodies and anticardiolipin/ß2-glycoprotein I antibodies and/or ß2-glycoprotein I-dependent anticardiolipin antibodies had higher odds of requiring treatment. Measuring the APTT and prothrombin time (PT) might be required in patients with IgAV when they do not have a typical clinical course or distinctive symptoms. LAHPS should be considered with prolongation of the APTT and/or PT. Additionally, it is important to maintain a balance between the risk of thrombosis and hemorrhage when normalization of the PT and FII levels occurs in LAHPS cases under treatment.
Asunto(s)
Coagulación Sanguínea , Hipoprotrombinemias/diagnóstico , Inmunoglobulina A/sangre , Inhibidor de Coagulación del Lupus/sangre , Tiempo de Tromboplastina Parcial , Tiempo de Protrombina , Vasculitis/diagnóstico , Biomarcadores/sangre , Niño , Preescolar , Humanos , Hipoprotrombinemias/sangre , Hipoprotrombinemias/inmunología , Japón , Masculino , Valor Predictivo de las Pruebas , Protrombina/metabolismo , Hermanos , Vasculitis/sangreRESUMEN
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.
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Síndrome Antifosfolípido/diagnóstico , Hipoprotrombinemias/diagnóstico , Lupus Eritematoso Sistémico/diagnóstico , Síndrome Antifosfolípido/tratamiento farmacológico , Niño , Ciclofosfamida/uso terapéutico , Femenino , Humanos , Hipoprotrombinemias/tratamiento farmacológico , Inmunoglobulinas/uso terapéutico , Inhibidor de Coagulación del Lupus , Lupus Eritematoso Sistémico/tratamiento farmacológico , Masculino , Metilprednisolona/uso terapéutico , Protrombina , Resultado del TratamientoRESUMEN
Lupus anticoagulant, also known as lupus antibody, is generally associated with thrombosis rather than bleeding events. Lupus anticoagulant-hypoprothrombinemia syndrome in children is rather rare but can lead to mild to life-threatening bleeding. Here, we report 3 cases of lupus anticoagulant-hypoprothrombinemia syndrome associated with systemic lupus erythematosus. They initially presented with mucocutaneous bleedings, and subsequently developed other symptoms fulfilling the laboratory criteria for systemic lupus erythematosus. Case 2 and 3 had significant epistaxis and intracerebral hemorrhage responded to systemic corticosteroid along with fresh frozen plasma. Three cases demonstrated acquired hypoprothrombinemia with no correction of mixing studies. Case 1 had low factor X level, which has never been reported previously. In all 3 cases, their coagulogram returned to normal level after corticosteroid treatment.
Asunto(s)
Hipoprotrombinemias/diagnóstico , Hipoprotrombinemias/etiología , Inhibidor de Coagulación del Lupus/inmunología , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/inmunología , Adolescente , Recuento de Células Sanguíneas , Pruebas de Coagulación Sanguínea , Médula Ósea/patología , Niño , Femenino , Humanos , Hipoprotrombinemias/tratamiento farmacológico , Inmunosupresores/uso terapéutico , Inhibidor de Coagulación del Lupus/sangre , Lupus Eritematoso Sistémico/sangre , Intercambio Plasmático , Síndrome , Resultado del TratamientoRESUMEN
BACKGROUND: Fibrinogen and prothrombin have been suggested to become rate limiting in trauma associated coagulopathy. Administration of fibrinogen is now recommended, however, the importance of prothrombin to patient outcome is unknown. METHODS: We have utilized two trauma patient databases (database 1 n = 358 and database 2 n = 331) to investigate the relationship of plasma prothrombin concentration on clinical outcome and coagulation status. Database 1 has been used to assess the relationship of plasma prothrombin to administered packed red blood cells (PRBC), clinical outcome and coagulation biomarkers (Prothrombin Time (PT), ROTEM EXTEM Coagulation Time (CT) and Maximum Clot Firmness (MCF)). ROC analyses have been performed to investigate the ability of admission coagulation biomarkers to predict low prothrombin concentration (database 1), massive transfusion and 24 h mortality (database 1 and 2). The importance of prothrombin was further investigated in vitro by PT and ROTEM assays in the presence of a prothrombin neutralizing monoclonal antibody and following step-wise dilution. RESULTS: Patients who survived the first 24 h had higher admission prothrombin levels compared to those who died (94 vs.67 IU/dL). Patients with lower transfusion requirements within the first 24 h (≤10 units of PRBCs) also had higher admission prothrombin levels compared to patients with massive transfusion demands (>10 units of PRBCs) (95 vs.62 IU/dL). Admission PT, in comparison to admission ROTEM EXTEM CT and MCF, was found to be a better predictor of prothrombin concentration <60 IU/dL (AUC 0.94 in database 1), of massive transfusion (AUC 0.92 and 0.81 in database 1 and 2 respectively) and 24 h mortality (AUC 0.90 and 0.78 in database 1 and 2, respectively). In vitro experiments supported a critical role for prothrombin in coagulation and demonstrated that PT and ROTEM EXTEM CT are sensitive methods to measure low prothrombin concentration. DISCUSSION: Our analyses suggest that prothrombin concentration at admission is predictive of mortality and transfusion and indicates that prothrombin and fibrinogen are rate limiting in coagulopathy. CONCLUSIONS: Admission PT is predictive of low prothrombin concentration and clinical outcome. PT could therefore be used as a surrogate for prothrombin concentration and further evaluation of point-of-care devices for faster PT analysis is warranted.
Asunto(s)
Hemorragia/terapia , Hipoprotrombinemias/diagnóstico , Valor Predictivo de las Pruebas , Tiempo de Protrombina , Adulto , Femenino , Humanos , Masculino , Persona de Mediana Edad , Plasma , Estudios Prospectivos , Resultado del Tratamiento , Adulto JovenRESUMEN
The lupus anticoagulant-hypoprothrombinemia syndrome (LA-HPS) - the association of acquired factor II deficiency and lupus anticoagulant - is a rare disease that may cause a predisposition not only to thrombosis but also to severe bleeding. We are reporting on a 36-year-old female patient presenting with co-existing cerebral venous thrombosis and subdural hemorrhage. The coagulation screening showed a prolonged prothrombin time (PT), activated partial thromboplastin time (aPTT), and a normal fibrinogen level and platelet count. Evaluation of the clotting factors revealed decreased levels of factors II (37%). Factors V, VIII, IX and XI were normal. Lupus anticoagulant (LA) was demonstrated by the Dilute Russell's Viper Venom Test (DRVVT). Immunological work-up was positive for IgG type anticardiolipines antibodies (aCL). Successful management consisted first of oral prednisone (60mg/d). Thus, anticoagulation was introduced once factor II had stabilized.
Asunto(s)
Hematoma Subdural/diagnóstico , Hipoprotrombinemias/diagnóstico , Trombosis Intracraneal/diagnóstico , Inhibidor de Coagulación del Lupus/análisis , Adulto , Síndrome Antifosfolípido/complicaciones , Venas Cerebrales , Femenino , Hematoma Subdural/complicaciones , Humanos , Hipoprotrombinemias/sangre , Hipoprotrombinemias/etiología , Trombosis Intracraneal/complicaciones , Inhibidor de Coagulación del Lupus/efectos adversos , Tiempo de Tromboplastina Parcial , Prednisona/uso terapéutico , Protrombina/análisis , Tiempo de ProtrombinaRESUMEN
Lupus anticoagulant-hypoprothrombinemia syndrome is a rare condition characterized by the association of acquired factor II deficiency and lupus anticoagulant. Contrary to classical antiphospholipid syndrome, it may cause severe life-threatening bleeding (89% of published cases). We report a patient, positive for antidomain I antibodies, with initially primary lupus anticoagulant-hypoprothrombinemia syndrome without previous clinical manifestation or underlying systemic disease. Five years later, he experienced the first systemic lupus erythematous flare. Within a few days, catastrophic antiphospholipid syndrome was diagnosed with heart, liver and kidney involvement. The patient recovered under pulse steroids, intravenous heparin and intravenous immunoglobulins.