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hypoprothrombinemia lupus anticoagulant

K Cetin Gedik, S Siddique, C L Aguiar
Lupus anticoagulant hypoprothrombinemia syndrome (LA-HPS) is a rare condition that may predispose both to thrombosis and bleeding due to positive lupus anticoagulant (LA) and factor II (FII) deficiency. It can be seen in association with infections or systemic lupus erythematosus (SLE) and may require glucocorticoids (GCs) and/or immunosuppressive medications. Pediatric LA-HPS cases in the literature and three cases that received only rituximab (RTX) for LA-HPS (in addition to GCs) at two institutions between January 2010 and June 2017 were analyzed descriptively...
January 1, 2018: Lupus
Sreejesh Sreedharanunni, Jasmina Ahluwalia, Narender Kumar, Sunil K Bose, Rishi Dhawan, Pankaj Malhotra
: 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...
July 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
Aimee Foord, Nicole Baca, David Buchbinder, Arash Mahajerin
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...
June 2017: Pediatric Blood & Cancer
N Bel Feki, S Zayet, I Ben Ghorbel, M-H Houman
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...
December 2016: Journal des Maladies Vasculaires
Jing Jin, James L Zehnder
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...
August 2016: American Journal of Clinical Pathology
Joris Galland, Shirine Mohamed, Sabine Revuz, Emmanuel de Maistre, Bas de Laat, Pierre-Yves Marie, Stéphane Zuily, Bruno Lévy, Véronique Regnault, Denis Wahl
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...
July 2016: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
Yang Li, Ming' en Lyu, Feng Xue, Wenjie Liu, Yating Hao, Yue Guan, Boyang Sun, Cuicui Lyu, Xueping Gu, Rongfeng Fu, Yueting Huang, Wei Liu, Yunfei Chen, Xiaofan Liu, Lei Zhang, Renchi Yang
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...
February 2016: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
Masahiro Ieko, Sumiyoshi Naito, Mika Yoshida, Nobuhiko Takahashi
Antiphospholipid syndrome (APS), an acquired thrombotic condition, is a complex clinical state characterized by the presence of circulating antiphospholipid antibodies in patients with thrombosis or pregnancy morbidity. Revised APS classification criteria are used for diagnosis, which include at least one clinical criterion (thrombosis or pregnancy loss) and at least one of the laboratory criteria [anticardiolipin antibodies, anti-β2GPI antibodies, lupus anticoagulant (LA)]. LA is also an independent risk factor for developing thrombosis, though some LA-positive cases have been reported to have a bleeding symptom...
October 2015: Rinsho Byori. the Japanese Journal of Clinical Pathology
T Sarker, S Roy, W Hollon, M Rajpurkar
INTRODUCTION: Lupus anticoagulant associated with acquired prothrombin deficiency also known as 'lupus anticoagulant hypoprothrombinemia syndrome' (LAHS) is an entity that is well described in adults and is usually associated with autoimmune conditions (LAHS-AI). However, in children, LAHS has unique features that are distinct from the adult type. AIMS: We report two paediatric cases of LAHS, describe their distinct patterns and review the paediatric literature on LAHS...
November 2015: Haemophilia: the Official Journal of the World Federation of Hemophilia
Natsuki Shimabukuro, Yukiko Nishi, Sawako Nakachi, Keita Tamaki, Iori Tedokon, Kazuho Morichika, Takeaki Tomoyose, Takuya Fukushima, Hiroaki Masuzaki
No abstract text is available yet for this article.
August 10, 2014: Nihon Naika Gakkai Zasshi. the Journal of the Japanese Society of Internal Medicine
Giulia Pazzola, Stéphane Zuily, Doruk Erkan
Antiphospholipid antibody-positive patients can develop bleeding due to capillaritis, microthrombosis, antiprothrombin antibodies, thrombocytopenia, and/or excessive antithrombotic therapy. Clinical characteristics of patients, e.g., renal impairment, elderly, or concomitant medications, are closely related to the risk of bleeding. The management of bleeding in antiphospholipid antibody (aPL)-positive patients is challenging due to the baseline increased risk of thrombosis. If anticoagulation is stopped, it should be restarted as soon as possible once the acute bleeding is controlled; the continuation of anticoagulation despite active bleeding may be required in selected cases...
February 2015: Current Rheumatology Reports
S M N Mulliez, F De Keyser, C Verbist, A Vantilborgh, W Wijns, I Beukinga, K M J Devreese
Lupus anticoagulant-hypoprothrombinemia syndrome (LA-HPS) is a rare acquired disorder caused by prothrombin antibodies. The disease is most common in the pediatric age group (<16 years), and more prevalent in women. There are well-established clinical diseases associated with LA-HPS, most notably systemic lupus erythematosus (SLE) and viral infections. The clinical manifestation of LA-HPS varies greatly in severity and it may cause severe life-threatening bleeding diathesis. LA-HPS is to be suspected when a patient presents with bleeding and a prolonged activated partial thromboplastin and prothrombin time, in combination with a lupus anticoagulant...
June 2015: Lupus
Laetitia Mauge, Amélie Passeron, Martine Alhenc-Gelas, Jacques Pouchot, Luc Darnige
No abstract text is available yet for this article.
April 2015: Annals of Hematology
Ji Soo Kim, Min Jae Kim, E Young Bae, Dae Chul Jeong
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...
April 2014: Korean Journal of Pediatrics
Takeshi Shimizu, Akira Ishiguro, Takaaki Takayanagi, Takehiko Matsui, Naoya Tonegawa, Takanobu Maekawa, Kazuo Itabashi
We describe a previously healthy 9-year-old girl who had multiple purpura several days after acute adenovirus gastroenteritis and mycoplasma pneumonia. Initial laboratory evaluation revealed a prolonged prothrombin time (PT) and APTT, low complement levels (C4, CH50), and positive immune complex (C1q) in her serum. Platelet count, fibrinogen, and other routine blood chemistry tests were normal. The prolonged APTT was not corrected by mixture of the patient's plus normal plasma. Clotting activities of factors II, V, VIII, IX, X, XI, and XII reduced...
2014: Nihon Rinshō Men'eki Gakkai Kaishi, Japanese Journal of Clinical Immunology
Ashutosh P Jadhav, Nima Aghaebrahim, Brian T Jankowitz, Tudor G Jovin
No abstract text is available yet for this article.
November 2013: JAMA Neurology
Cristiana Carvalho, Carolina Viveiro, Paulo Maia, Teresa Rezende
Acquired inhibitors of coagulation causing bleeding manifestations are rare in children. They emerge, normally in the context of autoimmune diseases or drug ingestion, but transient and self-limiting cases can occur after viral infection. We describe, an otherwise healthy, 7-year-old girl who had gingival bleeding after a tooth extraction. The prothrombin time (PT) and the activated partial thromboplastin time (APTT) were both prolonged with evidence of an immediate acting inhibitor (lupic anticoagulant). Further coagulation studies demonstrated prothrombin (FII) deficiency and prothrombin directed (FII) antibodies...
2013: BMJ Case Reports
R D Paschal, A T Neff
Hypoprothrombinemia associated with a lupus anticoagulant (LA) was first reported in the literature over 50 years ago. The hypoprothrombinemia-lupus anticoagulant syndrome (HLAS) is a rare bleeding diathesis that has been associated with LAs in adult and paediatric patients with systemic lupus erythematosus (SLE) and with transient LAs due to other causes. There are no standard recommendations for treating haemorrhage associated with this syndrome. Herein, we report a patient with SLE and HLAS who achieved a durable remission following treatment with intravenous immune globulin (IVIG), prednisone and rituximab...
March 2013: Haemophilia: the Official Journal of the World Federation of Hemophilia
Shingo Morinaga, Yoko Ide, Masaru Kusumoto, Mika Ogata, Kazutaka Takaki, Emiko Matsumoto
We report a case of a 1-year-old boy diagnosed with lupus anticoagulant-hypoprothrombinemia syndrome (LA-HPS), which is a rare disorder. His initial presentation of sinusitis was accompanied by hemorrhagic episodes including ecchymoses and epistaxis 6 months after antibiotic therapy. Laboratory results revealed prolonged prothrombin time (PT) and activated partial thromboplastin time (APTT) that did not correct with mixing studies. Factors II, VIII, IX, X, XI, and XII activities were 20%, 44%, 42.5%, 59%, 4%, and 10%, respectively...
July 2012: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
Karin Mazodier, Laurent Arnaud, Alexis Mathian, Nathalie Costedoat-Chalumeau, Julien Haroche, Camille Frances, Jean-Robert Harlé, Gilles Pernod, Eric Lespessailles, Philippe Gaudin, Hilaire Charlanne, Eric Hachulla, Patrick Niaudet, Jean-Charles Piette, Zahir Amoura
The lupus anticoagulant-hypoprothrombinemia syndrome (LAHS)--the association of acquired factor II deficiency and lupus anticoagulant--is a rare disease drastically different from antiphospholipid syndrome in that it may cause predisposition not only to thrombosis but also to severe bleeding. We performed a retrospective study of 8 patients with LAHS referred to 6 French tertiary care centers between January 2003 and February 2011, and a literature review retrieving all related articles published between 1960 and April 2011...
September 2012: Medicine (Baltimore)
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