Database : MEDLINE
Search on : Hypoprothrombinemias [Words]
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[PMID]: 28787393
[Au] Autor:Komvilaisak P; Wisanuyotin S; Jettrisuparb A; Wiangnon S
[Ad] Address:Department of Pediatrics, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand.
[Ti] Title:Lupus Anticoagulant-hypoprothrombinemia Syndrome (LAC-HPS) in Children With Systemic Lupus Erythematosus: Report of 3 Cases.
[So] Source:J Pediatr Hematol Oncol;39(8):e521-e524, 2017 Nov.
[Is] ISSN:1536-3678
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract: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.
[Mh] MeSH terms primary: Hypoprothrombinemias/diagnosis
Hypoprothrombinemias/etiology
Lupus Coagulation Inhibitor/immunology
Lupus Erythematosus, Systemic/complications
Lupus Erythematosus, Systemic/immunology
[Mh] MeSH terms secundary: Adolescent
Blood Cell Count
Blood Coagulation Tests
Bone Marrow/pathology
Child
Female
Humans
Hypoprothrombinemias/drug therapy
Immunosuppressive Agents/therapeutic use
Lupus Coagulation Inhibitor/blood
Lupus Erythematosus, Systemic/blood
Plasma Exchange
Syndrome
Treatment Outcome
[Pt] Publication type:CASE REPORTS; JOURNAL ARTICLE
[Nm] Name of substance:0 (Immunosuppressive Agents); 0 (Lupus Coagulation Inhibitor)
[Em] Entry month:1711
[Cu] Class update date: 171103
[Lr] Last revision date:171103
[Js] Journal subset:IM
[Da] Date of entry for processing:170809
[St] Status:MEDLINE
[do] DOI:10.1097/MPH.0000000000000891

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[PMID]: 28292321
[Au] Autor:Balendran CA; Lövgren A; Hansson KM; Nelander K; Olsson M; Johansson KJ; Brohi K; Fries D; Berggren A
[Ad] Address:Personalised HealthCare and Biomarkers, Innovative Medicines and Early Development Biotech Unit, AstraZeneca, Pepparedsleden 1, Mölndal, 431 83, Sweden. Clare.a.balendran@astrazeneca.com.
[Ti] Title:Prothrombin time is predictive of low plasma prothrombin concentration and clinical outcome in patients with trauma hemorrhage: analyses of prospective observational cohort studies.
[So] Source:Scand J Trauma Resusc Emerg Med;25(1):30, 2017 Mar 14.
[Is] ISSN:1757-7241
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract: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.
[Mh] MeSH terms primary: Hemorrhage/therapy
Hypoprothrombinemias/diagnosis
Predictive Value of Tests
Prothrombin Time
[Mh] MeSH terms secundary: Adult
Female
Humans
Male
Middle Aged
Plasma
Prospective Studies
Treatment Outcome
Young Adult
[Pt] Publication type:JOURNAL ARTICLE; OBSERVATIONAL STUDY
[Em] Entry month:1710
[Cu] Class update date: 171011
[Lr] Last revision date:171011
[Js] Journal subset:IM
[Da] Date of entry for processing:170316
[St] Status:MEDLINE
[do] DOI:10.1186/s13049-016-0332-2

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[PMID]: 27862892
[Au] Autor:Foord A; Baca N; Buchbinder D; Mahajerin A
[Ad] Address:Hematology-Oncology Hospitalist Service, Division of Pediatrics, Seattle Children's Hospital, Seattle, Washington.
[Ti] Title:Lupus anticoagulant hypoprothrombinemia syndrome associated with severe thrombocytopenia in a child.
[So] Source:Pediatr Blood Cancer;64(6), 2017 Jun.
[Is] ISSN:1545-5017
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract: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.
[Mh] MeSH terms primary: Autoimmune Diseases
Hypoprothrombinemias
Lupus Coagulation Inhibitor/blood
Oral Hemorrhage
Thrombocytopenia
[Mh] MeSH terms secundary: Autoimmune Diseases/blood
Autoimmune Diseases/complications
Autoimmune Diseases/therapy
Child
Humans
Hypoprothrombinemias/blood
Hypoprothrombinemias/complications
Hypoprothrombinemias/therapy
Male
Oral Hemorrhage/blood
Oral Hemorrhage/etiology
Oral Hemorrhage/therapy
Severity of Illness Index
Syndrome
Thrombocytopenia/blood
Thrombocytopenia/etiology
Thrombocytopenia/therapy
[Pt] Publication type:CASE REPORTS; JOURNAL ARTICLE
[Nm] Name of substance:0 (Lupus Coagulation Inhibitor)
[Em] Entry month:1708
[Cu] Class update date: 170831
[Lr] Last revision date:170831
[Js] Journal subset:IM
[Da] Date of entry for processing:161119
[St] Status:MEDLINE
[do] DOI:10.1002/pbc.26357

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[PMID]: 26886363
[Au] Autor:Harel R; Shani D; Donohoe K
[Ad] Address:Hematology/Oncology Faculty, NSLIJ/Lenox Hill Hospital, New York, USA.
[Ti] Title:A case of congenital prothrombin deficiency and idiopathic thrombocytopenic purpura in a pregnant female.
[So] Source:Blood Coagul Fibrinolysis;28(1):100-101, 2017 Jan.
[Is] ISSN:1473-5733
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract: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.
[Mh] MeSH terms primary: Hemorrhagic Disorders/complications
Hypoprothrombinemias/complications
Purpura, Thrombocytopenic, Idiopathic/complications
[Mh] MeSH terms secundary: Adult
Female
Humans
Pregnancy
[Pt] Publication type:CASE REPORTS; JOURNAL ARTICLE
[Em] Entry month:1703
[Cu] Class update date: 170817
[Lr] Last revision date:170817
[Js] Journal subset:IM
[Da] Date of entry for processing:160218
[St] Status:MEDLINE
[do] DOI:10.1097/MBC.0000000000000534

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[PMID]: 27756962
[Au] Autor:Choudhary B; Kumari S; Dhingra B; Jhaj R
[Ad] Address:Department of Pediatrics, All India Institute of Medical Sciences, Bhopal, Madhya Pradesh, India.
[Ti] Title:A clinically suspected case of Anaphylactoid reaction to vitamin K injection in a child - a case report and review of literature.
[So] Source:Indian J Pharmacol;48(4):455-457, 2016 Jul-Aug.
[Is] ISSN:1998-3751
[Cp] Country of publication:India
[La] Language:eng
[Ab] Abstract:Vitamin K is commonly indicated in pediatric patients with coagulopathies. Its commercial preparations are available in the market since long. Minor complications are very well-known, but life-threatening events are rarely reported in children. We present a case of 8-year-old child who developed life-threatening event following intravenous Vitamin K injection. She was survived after cardiopulmonary resuscitation. The reaction was most probably due to anaphylactoid reaction to Vitamin K.
[Mh] MeSH terms primary: Anaphylaxis/chemically induced
Antifibrinolytic Agents/adverse effects
Vitamin K/adverse effects
[Mh] MeSH terms secundary: Anaphylaxis/diagnosis
Anaphylaxis/therapy
Antifibrinolytic Agents/administration & dosage
Antifibrinolytic Agents/therapeutic use
Child
Female
Humans
Hypoprothrombinemias/prevention & control
Injections, Intravenous
Treatment Outcome
Vitamin K/administration & dosage
Vitamin K/therapeutic use
[Pt] Publication type:CASE REPORTS; JOURNAL ARTICLE
[Nm] Name of substance:0 (Antifibrinolytic Agents); 12001-79-5 (Vitamin K)
[Em] Entry month:1706
[Cu] Class update date: 170817
[Lr] Last revision date:170817
[Js] Journal subset:IM
[Da] Date of entry for processing:161021
[St] Status:MEDLINE

  6 / 843 MEDLINE  
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[PMID]: 27743753
[Au] Autor:Bel Feki N; Zayet S; Ben Ghorbel I; Houman MH
[Ad] Address:Service de médecine interne, CHU La Rabta, Tunis, Tunisia. Electronic address: belfeki.nabil@gmail.com.
[Ti] Title:Lupus anticoagulant-hypoprothrombinemia syndrome presenting with co-existing cerebral venous thrombosis and subdural hemorrhage.
[So] Source:J Mal Vasc;41(6):403-406, 2016 Dec.
[Is] ISSN:2214-8116
[Cp] Country of publication:France
[La] Language:eng
[Ab] Abstract: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.
[Mh] MeSH terms primary: Hematoma, Subdural/diagnosis
Hypoprothrombinemias/diagnosis
Intracranial Thrombosis/diagnosis
Lupus Coagulation Inhibitor/analysis
[Mh] MeSH terms secundary: Adult
Antiphospholipid Syndrome/complications
Cerebral Veins
Female
Hematoma, Subdural/complications
Humans
Hypoprothrombinemias/blood
Hypoprothrombinemias/etiology
Intracranial Thrombosis/complications
Lupus Coagulation Inhibitor/adverse effects
Partial Thromboplastin Time
Prednisone/therapeutic use
Prothrombin/analysis
Prothrombin Time
[Pt] Publication type:CASE REPORTS; JOURNAL ARTICLE
[Nm] Name of substance:0 (Lupus Coagulation Inhibitor); 9001-26-7 (Prothrombin); VB0R961HZT (Prednisone)
[Em] Entry month:1706
[Cu] Class update date: 170614
[Lr] Last revision date:170614
[Js] Journal subset:IM
[Da] Date of entry for processing:161017
[St] Status:MEDLINE

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[PMID]: 27473743
[Au] Autor:Jin J; Zehnder JL
[Ad] Address:From the Department of Special Coagulation, Clinical Laboratory, Stanford Medical Center, Stanford, CA.
[Ti] Title:Prozone Effect in the Diagnosis of Lupus Anticoagulant for the Lupus Anticoagulant-Hypoprothrombinemia Syndrome.
[So] Source:Am J Clin Pathol;146(2):262-7, 2016 Aug.
[Is] ISSN:1943-7722
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:OBJECTIVES: The main clinical sequela of a lupus anticoagulant is increased thrombosis risk. However, bleeding due to lupus anticoagulant-hypoprothrombinemia syndrome is a rare but well-described manifestation of antiphospholipid syndrome. The association of acute acquired hypoprothrombinemia is caused by a lupus anticoagulant's specificity to prothrombin, which results in clearance of prothrombin and bleeding due to hypoprothrombinemia (usually <10% of normal). Severe life-threatening bleeding is most frequently reported in children with systemic lupus erythematosus or in healthy children after viral infection. In such cases, steroid therapy is usually effective in controlling the bleeding problems and improving prothrombin levels. METHODS: We report one pediatric patient with a lupus anticoagulant who had acute hemorrhagic diathesis. RESULTS: The diagnosis in this case was complicated by the presence of a prozone effect in lupus anticoagulant testing. The prozone effect (also known as hook effect) refers to situations where very high concentrations of antibody mask detection, typically in antigen-antibody reactions, which depend on visualization of agglutination. Decreasing the antibody/antigen ratio results in detectable antigen-antibody complexes. CONCLUSIONS: We report for the first time a variation on this theme in a patient with a lupus anticoagulant-type antiphospholipid antibody and hypoprothrombinemia, which corrected with immunosuppression and restoration of normal prothrombin levels.
[Mh] MeSH terms primary: Antiphospholipid Syndrome/diagnosis
Hypoprothrombinemias/diagnosis
Lupus Coagulation Inhibitor
[Mh] MeSH terms secundary: Child, Preschool
Humans
Male
[Pt] Publication type:CASE REPORTS; JOURNAL ARTICLE
[Nm] Name of substance:0 (Lupus Coagulation Inhibitor)
[Em] Entry month:1702
[Cu] Class update date: 170817
[Lr] Last revision date:170817
[Js] Journal subset:AIM; IM
[Da] Date of entry for processing:160731
[St] Status:MEDLINE
[do] DOI:10.1093/ajcp/aqw106

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[PMID]: 27463687
[Au] Autor:Chen LJ; Hsiao FY; Shen LJ; Wu FL; Tsay W; Hung CC; Lin SW
[Ad] Address:Graduate Institute of Clinical Pharmacy, College of Medicine, National Taiwan University, Taipei, Taiwan.
[Ti] Title:Use of Hypoprothrombinemia-Inducing Cephalosporins and the Risk of Hemorrhagic Events: A Nationwide Nested Case-Control Study.
[So] Source:PLoS One;11(7):e0158407, 2016.
[Is] ISSN:1932-6203
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:OBJECTIVE: Existing data regarding the risk of hemorrhagic events associated with exposure to hypoprothrombinemia-inducing cephalosporins are limited by the small sample size. This population-based study aimed to examine the association between exposure to hypoprothrombinemia-inducing cephalosporins and hemorrhagic events using National Health Insurance Research Database in Taiwan. DESIGN: A nationwide nested case-control study. SETTING: National Health Insurance Research database. PARTICIPANTS: We conducted a nested case-control study within a cohort of 6191 patients who received hypoprothrombinemia-inducing cephalosporins and other antibiotics for more than 48 hours. Multivariable conditional logistic regressions were used to calculate the adjusted odds ratio (aOR) and 95% confidence interval (CI) for hemorrhagic events associated with exposure to hypoprothrombinemia-inducing cephalosporins (overall, cumulative dose measured as defined daily dose (DDD), and individual cephalosporins). RESULTS: Within the cohort, we identified 704 patients with hemorrhagic events and 2816 matched controls. Use of hypoprothrombinemia-inducing cephalosporins was associated with increased risk of hemorrhagic events (aOR, 1.71; 95% CI, 1.42-2.06), which increased with higher cumulative doses (<3 DDDs, aOR 1.62; 3-5 DDDs, aOR 1.78; and >5 DDDs, aOR 1.89). The aOR for individual cephalosporin was 2.88 (95% CI, 2.08-4.00), 1.35 (1.09-1.67) and 4.57 (2.63-7.95) for cefmetazole, flomoxef, and cefoperazone, respectively. Other risk factors included use of anticoagulants (aOR 2.08 [95% CI, 1.64-2.63]), liver failure (aOR 1.69 [1.30-2.18]), poor nutritional status (aOR 1.41 [1.15-1.73]), and history of hemorrhagic events (aOR 2.57 [1.94-3.41]) 6 months prior to the index date. CONCLUSIONS: Use of hypoprothrombinemia-inducing cephalosporins increases risk of hemorrhagic events. Close watch for hemorrhagic events is recommended when prescribing these cephalosporins, especially in patients who are at higher risk.
[Mh] MeSH terms primary: Cephalosporins/administration & dosage
Hemorrhage/chemically induced
Hypoprothrombinemias/chemically induced
[Mh] MeSH terms secundary: Adult
Aged
Case-Control Studies
Cohort Studies
Female
Hemorrhage/epidemiology
Humans
Male
Middle Aged
Taiwan/epidemiology
Young Adult
[Pt] Publication type:JOURNAL ARTICLE
[Nm] Name of substance:0 (Cephalosporins)
[Em] Entry month:1707
[Cu] Class update date: 170717
[Lr] Last revision date:170717
[Js] Journal subset:IM
[Da] Date of entry for processing:160728
[St] Status:MEDLINE
[do] DOI:10.1371/journal.pone.0158407

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[PMID]: 27380476
[Au] Autor:Galland J; Mohamed S; Revuz S; de Maistre E; de Laat B; Marie PY; Zuily S; Lévy B; Regnault V; Wahl D
[Ad] Address:aNancy University Hospital, Vascular Medicine Division and Regional Competence, Center for Rare Vascular And Systemic Autoimmune DiseasesbDijon University Hospital, Haematology laboratory, Nancy, FrancecBiochemistry, Cardiovascular Research Institute Maastricht (CARIM), Maastricht, University, Maastricht, the NetherlandsdNancy University Hospital, Nuclear Medecine & Nancyclotep Experimental Imaging, PlatformeUniversité de LorrainefInserm, UMR_S 1116gNancy University Hospital, Department of Critical Care Medicine, Nancy, France.
[Ti] Title:Lupus anticoagulant-hypoprothrombinemia syndrome and catastrophic antiphospholipid syndrome in a patient with antidomain I antibodies.
[So] Source:Blood Coagul Fibrinolysis;27(5):580-2, 2016 Jul.
[Is] ISSN:1473-5733
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract: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.
[Mh] MeSH terms primary: Antiphospholipid Syndrome/diagnosis
Hypoprothrombinemias/diagnosis
Lupus Coagulation Inhibitor/blood
Lupus Erythematosus, Systemic/diagnosis
[Mh] MeSH terms secundary: Adult
Antibodies, Antiphospholipid/blood
Antibodies, Antiphospholipid/immunology
Antiphospholipid Syndrome/complications
Antiphospholipid Syndrome/drug therapy
Antiphospholipid Syndrome/immunology
Heparin/therapeutic use
Humans
Hypoprothrombinemias/complications
Hypoprothrombinemias/drug therapy
Hypoprothrombinemias/immunology
Immunoglobulins, Intravenous/therapeutic use
Kidney/immunology
Kidney/metabolism
Kidney/pathology
Liver/immunology
Liver/metabolism
Liver/pathology
Lupus Coagulation Inhibitor/immunology
Lupus Erythematosus, Systemic/drug therapy
Lupus Erythematosus, Systemic/etiology
Lupus Erythematosus, Systemic/immunology
Male
Myocardium/immunology
Myocardium/metabolism
Myocardium/pathology
Steroids/therapeutic use
[Pt] Publication type:CASE REPORTS; JOURNAL ARTICLE
[Nm] Name of substance:0 (Antibodies, Antiphospholipid); 0 (Immunoglobulins, Intravenous); 0 (Lupus Coagulation Inhibitor); 0 (Steroids); 9005-49-6 (Heparin)
[Em] Entry month:1702
[Cu] Class update date: 170817
[Lr] Last revision date:170817
[Js] Journal subset:IM
[Da] Date of entry for processing:160706
[St] Status:MEDLINE
[do] DOI:10.1097/MBC.0000000000000545

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[PMID]: 27014983
[Au] Autor:Li Y; Lyu Me; Xue F; Liu W; Hao Y; Guan Y; Sun B; Lyu C; Gu X; Fu R; Huang Y; Liu W; Chen Y; Liu X; Zhang L; Yang R
[Ad] Address:Institute of Hematology & Blood Diseases Hospital, CAMS & PUMC, Tianjin 300020, China.
[Ti] Title:[Lupus anticoagulant: two cases report and literature review].
[So] Source:Zhonghua Xue Ye Xue Za Zhi;37(2):130-3, 2016 Feb.
[Is] ISSN:0253-2727
[Cp] Country of publication:China
[La] Language:chi
[Ab] Abstract:OBJECTIVE: To deepen the understanding of clinical manifestations and treatment of patients with positive lupus anticoagulant (LAC). METHODS: The clinical data of 2 patients were analyzed and related literature were reviewed. RESULTS: Case 1, a 31-year-old female, diagnosed as lupus anticoagulant positive, secondary to undifferentiated connective tissue disease, was presented with menorrhagia and thrombocytopenia. Anti-nuclear antibody (ANA) was positive 1:1000 (homogeneous type) with anti-double stranded DNA positive, and dRVVT LA1/LA2 was 3.4. Coagulation function was alleviated after treatment with glucocorticoid and total glucosides of paeony. Case 2, a 59-year-old female was presented with gingival bleeding, hematuria with the level of F II:C 13%. dRVVT LA1/LA2 was 2.0. Anti-nuclear antibody (ANA) was positive 1:1000 (type of cytoplasmic granule), anti-double stranded DNA was positive. The patient was diagnosed as hypoprothrombinemia-lupus anticoagulant syndrome (LAHS) and acquired coagulation factor deficiency. The signs of hemorrhage were alleviated after treatment with methylprednisolone 40 mg/day and cyclophosphamide, while the level of F II:C was below normal. CONCLUSION: Symptoms of patients with positive LAC are variable. The diagnosis relies on history of disease and laboratory test. Currently, there is no standardized treatment. Cases of LAHS should be thoroughly investigated for any known causes and related disorder.
[Mh] MeSH terms primary: Hypoprothrombinemias/diagnosis
Lupus Coagulation Inhibitor/blood
[Mh] MeSH terms secundary: Adult
Blood Coagulation
Cyclophosphamide/therapeutic use
Female
Glucocorticoids/therapeutic use
Hematologic Tests
Hemorrhage
Humans
Methylprednisolone/therapeutic use
Middle Aged
[Pt] Publication type:CASE REPORTS; ENGLISH ABSTRACT; JOURNAL ARTICLE; RESEARCH SUPPORT, NON-U.S. GOV'T; REVIEW
[Nm] Name of substance:0 (Glucocorticoids); 0 (Lupus Coagulation Inhibitor); 8N3DW7272P (Cyclophosphamide); X4W7ZR7023 (Methylprednisolone)
[Em] Entry month:1607
[Cu] Class update date: 160326
[Lr] Last revision date:160326
[Js] Journal subset:IM
[Da] Date of entry for processing:160326
[St] Status:MEDLINE
[do] DOI:10.3760/cma.j.issn.0253-2727.2016.02.009


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