Subscribe to RSS
DOI: 10.1055/s-0044-1779739
Lupus Anticoagulant-Hypoprothrombinemia Syndrome: Literature Review and Description of Local Case in a 3-Year-Old Chinese Girl
![](https://www.thieme-connect.de/media/sth/202404/lookinside/thumbnails/10-1055-s-0044-1779739_03201-1.jpg)
Abstract
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.
Ethical Approval
The study protocol was approved by the Tongji Hospital Ethics Committee for Research in Health. Informed consent was obtained from individuals included in this study.
Publication History
Article published online:
23 February 2024
© 2024. Thieme. All rights reserved.
Thieme Medical Publishers, Inc.
333 Seventh Avenue, 18th Floor, New York, NY 10001, USA
-
References
- 1 Sakamoto A, Ogura M, Hattori A. et al. Lupus anticoagulant hypoprothrombinemia syndrome associated with bilateral adrenal haemorrhage in a child: early diagnosis and intervention. Thromb J 2021; 19 (01) 19
- 2 Lu Y, Huang XM. Autoimmune hemolytic anemia as an initial presentation in children with systemic lupus erythematosus: two case reports. J Int Med Res 2022; 50 (08) 3000605221115390
- 3 Rapaport SI, Ames SB, Duvall BJ. A plasma coagulation defect in systemic lupus erythematosus arising from hypoprothrombinemia combined with antiprothrombinase activity. Blood 1960; 15: 212-227
- 4 Fujiwara K, Shimizu J, Tsukahara H, Shimada A. Lupus anticoagulant-hypoprothrombinemia syndrome and immunoglobulin-A vasculitis: a report of Japanese sibling cases and review of the literature. Rheumatol Int 2019; 39 (10) 1811-1819
- 5 Ieko M, Yoshida M, Naito S, Ohmura K, Takahashi N. Lupus anticoagulant-hypoprothrombinemia syndrome and similar diseases: experiences at a single center in Japan. Int J Hematol 2019; 110 (02) 197-204
- 6 Fei Y, Tang N, Zhang H, Li G, Zhang H, Zhang C. Significantly prolonged prothrombin time and activated partial thromboplastin time with no bleeding tendency: a patient with lupus anticoagulant-hypoprothrombinemia syndrome positive for immunoglobulin M anti-phosphatidylserine/prothrombin complex antibodies. Semin Thromb Hemost 2020; 46 (04) 507-511
- 7 Ozdel S, Baglan E, Azapağası EÜ, Eroglu FK, Kargın Cakıcı E, Bulbul M. An extremely rare cause of prolonged menstruation: lupus anticoagulant-hypoprothrombinemia syndrome. J Obstet Gynaecol 2020; 40 (06) 887-888
- 8 Koyama D, Hanajiri R, Kanematsu T. et al. [Lupus anticoagulant-hypoprothrombinemia syndrome associated with follicular lymphoma]. Rinsho Ketsueki 2020; 61 (07) 745-749
- 9 Kocheril AP, Vettiyil GI, George AS. et al. Pediatric systemic lupus erythematosus with lupus anticoagulant hypoprothrombinemia syndrome—a case series with review of literature. Lupus 2021; 30 (04) 641-648
- 10 Chan EY, Leung KFS, Soo ETL, Ma ALT. A Chinese boy with lupus anticoagulant-hypoprothrombinemia syndrome: a case report and review of the literature. J Pediatr Hematol Oncol 2021; 43 (03) e445-e447
- 11 Li JZ, Tian X, Liao CS, He XL, Zhu CG. [Recurrent epistaxis with coagulation disorders in a boy aged 2 years]. Zhongguo Dang Dai Er Ke Za Zhi 2022; 24 (07) 817-820
- 12 Sun YH, Nie WJ, Tian DD, Ye Q. Lupus anticoagulant-hypoprothrombinemia syndrome in children: three case reports and systematic review of the literature. Lupus 2023; 32 (10) 1211-1221
- 13 Mabuchi H, Moriya K, Hoshino Y, Take J, Kawaguchi H, Imai K. A case of suspected lupus anticoagulant-hypoprothrombinemia syndrome in a boy due to mycoplasma infection. Pediatr Int 2023; 65 (01) e15592
- 14 Meireles E, Machado F, Teles L, Chumakova A, Sequeira J, Spínola A. A case report of severe bleeding due to lupus anticoagulant hypoprothrombinemia syndrome. J Thromb Thrombolysis 2020; 49 (02) 334-336
- 15 Pengo V, Zardo L, Cattini MG. et al. Prothrombin is responsible for the lupus cofactor phenomenon in a patient with lupus anticoagulant/hypoprothrombinemia syndrome. TH Open 2020; 4 (01) e40-e44
- 16 Vandamme S, Desclée E, Ver Elst K. et al. Lupus anticoagulant hypoprothrombinaemia syndrome: an instructive paediatric case. J Paediatr Child Health 2021; 57 (03) 443-444
- 17 Brblić T, Brčić K, Margetić S, Čaržavec D, Gaćina P, Bogdanić N. Lupus anticoagulant-hypoprothrombinemia syndrome with severe bleeding diathesis after coronavirus disease 2019: a case report. Croat Med J 2022; 63 (05) 490-494
- 18 Omer MH, Salama H, Alghaythi AM, Alharbi AA, AlJohani G. Lupus anticoagulant-hypoprothrombinemia syndrome and pseudotumor cerebri as an initial presentation of systemic lupus erythematosus in a 16-year-old male patient: a case report and literature review. Am J Case Rep 2022; 23: e938051
- 19 Acedo N, Alonso A, Feijoó ES, García C, Ortiz AM, Alegre A. Lupus anticoagulant hypoprothrombinemia syndrome: a case report. Clin Case Rep 2023; 11 (04) e7071
- 20 Matijasic Stjepovic N, Kranjcec I, Gagro A, Jakovljevic G. Lupus anticoagulant hypoprothrombinemia syndrome—could it be a sequelae of COVID-19?. Blood Res 2023; 58 (01) 77-79
- 21 Monti M, Martini T, Pengo V. et al. Association between severe acute respiratory syndrome coronavirus 2 infection (coronavirus disease 2019) and lupus anticoagulant-hypoprothrombinemia syndrome: a case report and literature assessment. Semin Thromb Hemost 2023; 49 (05) 558-560
- 22 Pérez ML, Laso RV, Velasco-Rodríguez D. et al. Lupus anticoagulant-hypoprothrombinemia syndrome: a cerebral bleeding case report as systemic lupus erythematosus debut. Reumatol Clín (Engl Ed) 2023; 19 (04) 223-227
- 23 Lipari A, Sorrentino S, Tamburini C, Castaman G, Prisco D, De Candia E. Bleeding and thrombotic events in a patient with lupus anticoagulant-associated hypoprothrombinemia and antiphospholipid antibody syndromes: managing hemostasis between Scylla and Charybdis. Intern Emerg Med 2023; 18 (04) 1233-1238
- 24 Llorente-Chávez A, Plascencia-Félix JF, Merayo-Chalico J. Lupus anticoagulant-hypoprothrombinaemia syndrome: subdural haematoma as an unusual and initial manifestation. Mod Rheumatol Case Rep 2021; 5 (02) 306-309
- 25 Peralta-Amaro AL, Triana-González S, Manzo-Carballo MF, Vera Lastra OL, García-Chávez J, Lucas-Hernández A. Antiphospholipid syndrome and lupus anticoagulant-hypoprothrombinemia. QJM 2023; 116 (04) 308-309
- 26 Nusrat S, Tewari S, Khan O. Successful treatment of lupus anticoagulant hypoprothrombinemia syndrome with rituximab. Thromb J 2023; 21 (01) 77
- 27 Chumsky J, Kahn PJ, Carroll WL, Pierce KA, Hillier K. Lupus anti-coagulant hypoprothrombinemia syndrome across different ages: a case report and review of the literature. Clin Rheumatol 2023; 42 (08) 2231-2236
- 28 Schulman S, Kearon C. Subcommittee on Control of Anticoagulation of the Scientific and Standardization Committee of the International Society on Thrombosis and Haemostasis. Definition of major bleeding in clinical investigations of antihemostatic medicinal products in non-surgical patients. J Thromb Haemost 2005; 3 (04) 692-694
- 29 Schulman S, Angerås U, Bergqvist D, Eriksson B, Lassen MR, Fisher W. Subcommittee on Control of Anticoagulation of the Scientific and Standardization Committee of the International Society on Thrombosis and Haemostasis. Definition of major bleeding in clinical investigations of antihemostatic medicinal products in surgical patients. J Thromb Haemost 2010; 8 (01) 202-204
- 30 Mazodier K, Arnaud L, Mathian A. et al. Lupus anticoagulant-hypoprothrombinemia syndrome: report of 8 cases and review of the literature. Medicine (Baltimore) 2012; 91 (05) 251-260
- 31 Cetin Gedik K, Siddique S, Aguiar CL. Rituximab use in pediatric lupus anticoagulant hypoprothrombinemia syndrome—report of three cases and review of the literature. Lupus 2018; 27 (07) 1190-1197
- 32 Clark CA, Davidovits J, Spitzer KA, Laskin CA. The lupus anticoagulant: results from 2257 patients attending a high-risk pregnancy clinic. Blood 2013; 122 (03) 341-347 , quiz 466
- 33 Forastiero R. Bleeding in the antiphospholipid syndrome. Hematology 2012; 17 (Suppl. 01) S153-S155
- 34 Simel DL, St Clair EW, Adams J, Greenberg CS. Correction of hypoprothrombinemia by immunosuppressive treatment of the lupus anticoagulant-hypoprothrombinemia syndrome. Am J Med 1987; 83 (03) 563-566
- 35 Bhattacharya M, Biswas A, Kannan M. et al. Clinicohematologic spectrum in patients with lupus anticoagulant. Clin Appl Thromb Hemost 2005; 11 (02) 191-195
- 36 Sarker T, Roy S, Hollon W, Rajpurkar M. Lupus anticoagulant acquired hypoprothrombinemia syndrome in childhood: two distinct patterns and review of the literature. Haemophilia 2015; 21 (06) 754-760
- 37 Komvilaisak P, Wisanuyotin S, Jettrisuparb A, Wiangnon S. Lupus anticoagulant-hypoprothrombinemia syndrome (LAC-HPS) in children with systemic lupus erythematosus: report of 3 cases. J Pediatr Hematol Oncol 2017; 39 (08) e521-e524
- 38 Mulliez SM, De Keyser F, Verbist C. et al. Lupus anticoagulant-hypoprothrombinemia syndrome: report of two cases and review of the literature. Lupus 2015; 24 (07) 736-745
- 39 Chen X, Nedved D, Plapp FV, Cunningham MT. Fatal pulmonary embolism and pulmonary hemorrhage in lupus anticoagulant hypoprothrombinemia syndrome: a case report and review of literature. Blood Coagul Fibrinolysis 2018; 29 (08) 708-713
- 40 Pilania RK, Suri D, Jindal AK. et al. Lupus anticoagulant hypoprothrombinemia syndrome associated with systemic lupus erythematosus in children: report of two cases and systematic review of the literature. Rheumatol Int 2018; 38 (10) 1933-1940
- 41 Bertin D, Beziane A, Resseguier N. et al. Interest of IgG and IgM antiprothrombin autoantibodies in the exploration of antiphospholipid syndrome: a 5-year retrospective study. Rheumatology (Oxford) 2020; 59 (07) 1539-1544
- 42 Moser KA, Puetz J. Challenging hemostasis scenarios in pediatric patients—two case studies. Int J Lab Hematol 2015; 37 (Suppl. 01) 72-77