RESUMEN
Lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS) is a rare, acquired coagulopathy syndrome. Here, we aim to summarize the clinical features of LAHPS to improve the understanding of the disease. The clinical data of 52 patients with LAHPS retrieved through PubMed from 2019 to 2023, supplemented with a local case of a child with LAHPS, were retrospectively analyzed, and the clinical characteristics were summarized. 56.6% of LAHPS patients were female, the median age at onset was 13.0 years (range, 1.2-85 years), and the median activity of factor II was 18.0% (range, 0.1-69%). 64.2% of LAHPS patients experienced hemorrhage, with 29.4% having multisite hemorrhage and 20.6% experiencing both nonsevere and severe hemorrhage. Most of the reported cases were secondary to autoimmune diseases (60.6%), followed by infections (33.3%). Corticosteroids were administered to 79.3% of patients with hemorrhage, and 90.6% of patients with LAHPS showed improvement. In conclusion, LAHPS is most commonly observed in female patients, particularly those under 18 years of age. LAHPS is characterized by hemorrhage, occurring at various sites and with varying degrees of severity, but the majority of patients improve with appropriate treatment and management.
Asunto(s)
Hipoprotrombinemias , Inhibidor de Coagulación del Lupus , Humanos , Hipoprotrombinemias/sangre , Hipoprotrombinemias/complicaciones , Femenino , Inhibidor de Coagulación del Lupus/sangre , Preescolar , Niño , Adolescente , Pueblos del Este de AsiaAsunto(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
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 , HemorragiaRESUMEN
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
Acquired antibodies against factor II (prothrombin) are rare and most commonly associated with severe liver disease or vitamin K antagonist treatment. In very rare cases, these antibodies and associated hypoprothrombinemia are found in patients with lupus anticoagulant (LAC), an antiphospholipid antibody that inhibits phospholipid-dependent coagulation tests. This uncommon entity, called lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS), may cause both severe, life-threatening bleeding and a predisposition to thrombosis. Coronavirus disease 2019 (COVID-19) is associated with a variety of coagulation abnormalities and an increased risk of thrombosis. Bleeding may occur, but it is less common than thromboembolism and has mostly been described in association with the severity of the disease and anticoagulation treatment in hospitalized patients, rarely in the post-acute phase of the disease. We report on a case of an 80-year-old man who developed LAHPS with prothrombin antibodies and severe bleeding after COVID-19.
Asunto(s)
Síndrome Antifosfolípido , COVID-19 , Hipoprotrombinemias , Masculino , Humanos , Anciano de 80 o más Años , Hipoprotrombinemias/complicaciones , Inhibidor de Coagulación del Lupus , COVID-19/complicaciones , Protrombina , Susceptibilidad a Enfermedades/complicaciones , Síndrome Antifosfolípido/complicaciones , Hemorragia/etiologíaAsunto(s)
COVID-19/sangre , Trombofilia/sangre , Trombosis/sangre , Adulto , Anciano , Antitrombina III/genética , Deficiencia de Antitrombina III/sangre , Deficiencia de Antitrombina III/complicaciones , Deficiencia de Antitrombina III/genética , COVID-19/complicaciones , COVID-19/fisiopatología , Estudios de Cohortes , Deficiencia del Factor V/sangre , Deficiencia del Factor V/complicaciones , Deficiencia del Factor V/genética , Femenino , Productos de Degradación de Fibrina-Fibrinógeno , Humanos , Hipoprotrombinemias/sangre , Hipoprotrombinemias/complicaciones , Hipoprotrombinemias/genética , Unidades de Cuidados Intensivos/estadística & datos numéricos , Modelos Logísticos , Masculino , Persona de Mediana Edad , Mortalidad , Proyectos Piloto , Proteína C/genética , Deficiencia de Proteína C/sangre , Deficiencia de Proteína C/complicaciones , Deficiencia de Proteína C/genética , Proteína S/genética , Deficiencia de Proteína S/sangre , Deficiencia de Proteína S/complicaciones , Deficiencia de Proteína S/genética , Síndrome de Dificultad Respiratoria/sangre , Síndrome de Dificultad Respiratoria/fisiopatología , SARS-CoV-2 , Índice de Severidad de la Enfermedad , Trombofilia/complicaciones , Trombofilia/genética , Trombofilia/fisiopatología , Trombosis/fisiopatologíaRESUMEN
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
Association of acquired factor II deficiency and lupus anticoagulant is a rare disease that can be related to sudden, severe or fatal haemorrhage. We present a 74-years-old woman with history of myelodysplastic syndrome, admitted to the Emergency Department due to spontaneous mucocutaneous bleeding. Coagulation assays revealed prolonged prothrombin time and activated partial thromboplastin time with evidence of an immediate acting inhibitor. Antithrombotic therapy usage, drug ingestion, disseminated intravascular coagulation, liver dysfunction and sepsis were excluded. Patient was admitted for close monitoring and etiological evaluation. A comprehensive bleeding diathesis workup was performed showing factor II levels severely decreased and transient positive lupus anticoagulant. Immunosuppression with methylprednisolone lasted for 3 days, followed by prednisolone. After 20 days she was discharged and follow-up was scheduled. Early diagnosis of lupus anticoagulant hypoprothrombinemia syndrome is critical, as it may result in fatal complications if not treated appropriately. There is no consensus regarding the best treatment, most being based on immunosuppression.
Asunto(s)
Hemorragia/sangre , Hemorragia/diagnóstico por imagen , Hipoprotrombinemias/sangre , Hipoprotrombinemias/diagnóstico por imagen , Inhibidor de Coagulación del Lupus/sangre , Anciano , Femenino , Hemorragia/etiología , Humanos , Hipoprotrombinemias/complicacionesAsunto(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 (LAHS) is a rare disorder characterized by development of lupus anticoagulant and antiprothrombin antibodies. The most common clinical manifestation is bleeding. Clinical management can be challenging due to the subtle balance between the bleeding and thrombotic tendencies. We report a novel case of LAHS in which the patient experienced the sequence of hemorrhage-thrombosis-hemorrhage before eventually dying of fatal pulmonary embolism and pulmonary hemorrhage. Specifically, she presented with multiple gastrointestinal bleeding episodes, followed by multifocal subdural hematomas, pulmonary embolism after normalization of prothrombin activity levels with immunosuppression, and finally with fatal pulmonary hemorrhage after enoxaparin treatment for pulmonary embolism. This case illustrates the importance of recognizing early minor bleeding episodes, and detecting specific antiprothrombin antibodies, in the diagnosis of LAHS. Furthermore, it highlights the complex challenge of normalizing prothrombin activity levels while at the same time preventing medical complications.
Asunto(s)
Hemorragia/etiología , Hipoprotrombinemias/complicaciones , Inhibidor de Coagulación del Lupus/sangre , Embolia Pulmonar/etiología , Anciano , Anticoagulantes/uso terapéutico , Síndrome Antifosfolípido/complicaciones , Enoxaparina/uso terapéutico , Resultado Fatal , Femenino , Hemorragia Gastrointestinal , Humanos , Protrombina/análisisRESUMEN
Hereditary prothrombin deficiency is an autosomal recessive disorder with an estimated incidence of 1 in 2 million . Presentation of the disease is variable; however, it is usually associated with moderate to severe bleeding tendencies including muscle hematomas, hemarthrosis, intracranial, mucosal, and postoperative bleeding. Here we report a case of a 35-year-old pregnant woman with congenital hypoprothrombinemia and idiopathic thrombocytopenic purpura, review the literature, and discuss its epidemiology, presentation, diagnosis, and treatment.
Asunto(s)
Trastornos Hemorrágicos/complicaciones , Hipoprotrombinemias/complicaciones , Púrpura Trombocitopénica Idiopática/complicaciones , Adulto , Femenino , Humanos , EmbarazoRESUMEN
Lupus anticoagulant hypoprothrombinemia syndrome (LAHPS) comprises lupus anticoagulant, acquired hypoprothrombinemia, and often mild thrombocytopenia or normal platelets. It is usually associated with autoimmunity or postviral illness. We describe a case of a 10-year-old boy with oral bleeding and severe thrombocytopenia initially suggestive of immune thrombocytopenia. Secondary to bleeding, evaluation demonstrated prolonged coagulation tests and subsequently revealed the presence of lupus anticoagulant and hypoprothrombinemia, along with marked autoimmunity, suggestive of LAHPS. He was treated with intravenous immunoglobulin and hydroxychloroquine. This case report and discussion highlight the diagnostic and therapeutic challenges associated with LAHPS and coincident severe thrombocytopenia.
Asunto(s)
Enfermedades Autoinmunes , Hipoprotrombinemias , Inhibidor de Coagulación del Lupus/sangre , Hemorragia Bucal , Trombocitopenia , Enfermedades Autoinmunes/sangre , Enfermedades Autoinmunes/complicaciones , Enfermedades Autoinmunes/terapia , Niño , Humanos , Hipoprotrombinemias/sangre , Hipoprotrombinemias/complicaciones , Hipoprotrombinemias/terapia , Masculino , Hemorragia Bucal/sangre , Hemorragia Bucal/etiología , Hemorragia Bucal/terapia , Índice de Severidad de la Enfermedad , Síndrome , Trombocitopenia/sangre , Trombocitopenia/etiología , Trombocitopenia/terapiaRESUMEN
: We report a 14-year-old girl who presented with subdural hematoma and a deranged coagulation profile suggestive of an inhibitor. Investigations revealed prothrombin deficiency along with positivity for antiphospholipid antibodies, which improved with steroid therapy. Bleeding diathesis in children and adolescents commonly results from thrombocytopenia, platelet function disorders, or coagulation factor deficiency; whereas bleeding because of coagulation factor inhibitors are extremely rare in this age group. This case also highlights the uncommon presentation of antiphospholipid antibody syndrome, as they often present with thrombosis or pregnancy complications rather than bleeding.
Asunto(s)
Síndrome Antifosfolípido/complicaciones , Hematoma Subdural/complicaciones , Hipoprotrombinemias/complicaciones , Inhibidor de Coagulación del Lupus/sangre , Adolescente , Síndrome Antifosfolípido/sangre , Síndrome Antifosfolípido/tratamiento farmacológico , Femenino , Hematoma Subdural/sangre , Hematoma Subdural/tratamiento farmacológico , Humanos , Hipoprotrombinemias/sangre , Hipoprotrombinemias/tratamiento farmacológico , Esteroides/uso terapéuticoRESUMEN
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.