rspНаучно-практическая ревматологияRheumatology Science and Practice1995-44841995-4492IMA-PRESS, LLC10.47360/1995-4484-2025-318-323rsp-3755Research ArticleОПИСАНИЕ СЛУЧАЯCASE DESCRIPTIONПриобретенная гемофилия у пациентки с системной красной волчанкой: описание клинического наблюденияAcquired hemophilia in patient with Systemic lupus erythematosus: Clinical case reporthttps://orcid.org/0000-0003-1923-415XАлексееваС. А.AlekseevaS. A.

115522, Москва, Каширское шоссе, 34а

Sofya A. Alekseeva.

115522, Moscow, Kashirskoye Highway, 34A

https://orcid.org/0000-0001-7074-0926ЗозуляН. И.ZozulyaN. I.

125167, Москва, Новый Зыковский пр-д, 4

Nadezhda I. Zozulya.

125167, Moscow, Novy Zykovsky road, 4

https://orcid.org/0000-0003-3552-2522РешетнякТ. М.ReshetnyakT. M.

Решетняк Татьяна Магомедалиевна.

115522, Москва, Каширское шоссе, 34а

Tatiana M. Reshetnyak.

115522, Moscow, Kashirskoye Highway, 34A

reshetnyak.tatjana@yandex.ruФГБНУ «Научно-исследовательский институт ревматологии им. В.А. Насоновой»РоссияV.A. Nasonova Research Institute of RheumatologyRussian FederationФГБУ «Национальный медицинский исследовательский центр гематологии» Минздрава РоссииРоссияNational Medical Research Center for HematologyRussian Federation202502072025633318323Copyright © Алексеева С.А., Зозуля Н.И., Решетняк Т.М., 20252025Алексеева С.А., Зозуля Н.И., Решетняк Т.М.Alekseeva S.A., Zozulya N.I., Reshetnyak T.M.This work is licensed under a Creative Commons Attribution 4.0 License.https://rsp.mediar-press.net/rsp/article/view/3755

Приобретенная гемофилия (ПГ) – редкое заболевание, развивающееся в результате синтеза антител к эндогенному VIII, редко – к IX фактору свертывания крови. Клиническим проявлением ПГ являются спонтанные кровотечения. Данное заболевание чаще развивается у пациентов старшего возраста и в 50% случаев отмечается при злокачественных новообразованиях и аутоиммунных ревматических заболеваниях. В данной статье описан клинический случай: ПГ, дебютировавшая незадолго до манифестации системной красной волчанки.

Acquired hemophilia is a rare disease that develops because of the synthesis of antibodies to endogenous VIII blood clotting factor. The most common symptom of acquired hemophilia is spontaneous bleeding. Acquired hemophilia is observed more frequently in older population and in 50% is secondary to malignant tumors and autoimmune rheumatic diseases. In this article, we present a clinical case of autoimmune hemophilia that developed shortly before the onset of systemic lupus erythematosus.

приобретенная гемофилиясистемная красная волчанкаспонтанные кровотеченияаутоантителаacquired hemophiliasystemic lupus erythematosusspontaneous bleedingautoantibodiesСтатья подготовлена в рамках темы ФГБНУ НИИР им. В.А. Насоновой РК 125020501434-1.ReferencesCollins P, Baudo F, Huth-Kühne A, Ingerslev J, Kessler CM, Castellano ME, et al. Consensus recommendations for the diagnosis and treatment of acquired hemophilia A. BMC Res Notes. 2010;3:161. doi: 10.1186/1756-0500-3-161Collins P, Baudo F, Huth-Kühne A, Ingerslev J, Kessler CM, Castellano ME, et al. Consensus recommendations for the diagnosis and treatment of acquired hemophilia A. BMC Res Notes. 2010;3:161. doi: 10.1186/1756-0500-3-161Shen CY, Fu LS, Lin HK, Han SM, Wang JD. Macrohematuria as initial presentation in a girl with factor VIII and factor IX inhibitors associated with systemic lupus erythematosus. J Pediatr Hematol Oncol. 2012;34(8):e344-e345. doi: 10.1097/MPH.0b013e3182678ec1Shen CY, Fu LS, Lin HK, Han SM, Wang JD. Macrohematuria as initial presentation in a girl with factor VIII and factor IX inhibitors associated with systemic lupus erythematosus. J Pediatr Hematol Oncol. 2012;34(8):e344-e345. doi: 10.1097/MPH.0b013e3182678ec1Akahoshi M, Aizawa K, Nagano S, Inoue H, Sadanaga A, Arinobu Y, et al. Acquired hemophilia in a patient with systemic lupus erythematosus: A case report and literature review. Mod Rheumatol. 2008;18(5):511-515. doi: 10.1007/s10165-008-0084-6Akahoshi M, Aizawa K, Nagano S, Inoue H, Sadanaga A, Arinobu Y, et al. Acquired hemophilia in a patient with systemic lupus erythematosus: A case report and literature review. Mod Rheumatol. 2008;18(5):511-515. doi: 10.1007/s10165-008-0084-6Zanon E. Acquired hemophilia A: An update on the etiopathogenesis, diagnosis, and treatment. Diagnostics (Basel). 2023;13(3):420. doi: 10.3390/diagnostics13030420Zanon E. Acquired hemophilia A: An update on the etiopathogenesis, diagnosis, and treatment. Diagnostics (Basel). 2023;13(3):420. doi: 10.3390/diagnostics13030420Суренков АА, Орел ЕБ, Зозуля НИ, Двирнык ВН. Особенности клинико-лабораторной диагностики редкой коагулопатии – приобретенной гемофилии. Гематология и трансфузиология. 2022;67(4):535-550. doi: 10.35754/0234-5730-2022-67-4-535-550Surenkov AA, Orel EB, Zozulya NI, Dvirnyk VN. Features of clinical and laboratory diagnosis of rare coagulopathy – acquired hemophilia. Russian Journal of Hematology and Transfusiology. 2022;67(4):535-550 (In Russ.). doi: 10.35754/0234-5730-2022-67-4-535-550Charlebois J, Rivard GÉ, St-Louis J. Management of acquired hemophilia A: Review of current evidence. Transfus Apheresis Sci. 2018;57(6):717-720. doi: 10.1016/j.transci.2018.10.011Charlebois J, Rivard GÉ, St-Louis J. Management of acquired hemophilia A: Review of current evidence. Transfus Apheresis Sci. 2018;57(6):717-720. doi: 10.1016/j.transci.2018.10.011Nishino Y, Ueki K, Suto M, Uchiumi H, Ota F, Tamura S, et al. Successful treatment of patients with rheumatic disorders and acquired factor VIII inhibitors with cyclophosphamide and prednisolone combination therapy: Two case reports. J Int Med Res. 2001;29(5):432-436. doi: 10.1177/147323000102900508Nishino Y, Ueki K, Suto M, Uchiumi H, Ota F, Tamura S, et al. Successful treatment of patients with rheumatic disorders and acquired factor VIII inhibitors with cyclophosphamide and prednisolone combination therapy: Two case reports. J Int Med Res. 2001;29(5):432-436. doi: 10.1177/147323000102900508Aringer M, Costenbader K, Daikh D, Brinks R, Mosca M, Ramsey-Goldman R, et al. 2019 European League Against Rheumatism/American College of Rheumatology classification criteria for systemic lupus erythematosus. Arthritis Rheumatol. 2019;71(9): 1400-1412. doi: 10.1002/art.40930Aringer M, Costenbader K, Daikh D, Brinks R, Mosca M, Ramsey-Goldman R, et al. 2019 European League Against Rheumatism/American College of Rheumatology classification criteria for systemic lupus erythematosus. Arthritis Rheumatol. 2019;71(9): 1400-1412. doi: 10.1002/art.40930Ассоциация ревматологов России. Федеральные клинические рекомендации по диагностике и лечению болезни Шегрена. М.;2013:7-8.Association of Rheumatologists of Russia. Federal clinical guidelines for the diagnosis and treatment of Sjogren’s disease. Moscow;2013:7-8 (In Russ.).Gladman DD, Ibañez D, Urowltz MB. Systemic lupus erythematosus disease activity index 2000. J Rheum. 2002;29(2):288-291. doi: 10.1016/b978-0-323-04434-9.50060-9Gladman DD, Ibañez D, Urowltz MB. Systemic lupus erythematosus disease activity index 2000. J Rheum. 2002;29(2):288-291. doi: 10.1016/b978-0-323-04434-9.50060-9Bombardier C, Gladman DD, Urowitz MB, Caron D, Chang CH. Derivation of the SLEDAI. A disease activity index for lupus patients. The Committee on Prognosis Studies in SLE. Arthritis Rheum. 1992;35(6):630-640. doi: 10.1002/art.1780350606Bombardier C, Gladman DD, Urowitz MB, Caron D, Chang CH. Derivation of the SLEDAI. A disease activity index for lupus patients. The Committee on Prognosis Studies in SLE. Arthritis Rheum. 1992;35(6):630-640. doi: 10.1002/art.1780350606Gladman D, Ginzler E, Goldsmith C, Fortin P, Liang M, Urowitz M, et al. The development and initial validation of the Systemic Lupus International Collaborating Clinics/American College of Rheumatology damage index for systemic lupus erythematosus. Arthritis Rheum. 1996;39(3):363-369. doi: 10.1002/art.1780390303Gladman D, Ginzler E, Goldsmith C, Fortin P, Liang M, Urowitz M, et al. The development and initial validation of the Systemic Lupus International Collaborating Clinics/American College of Rheumatology damage index for systemic lupus erythematosus. Arthritis Rheum. 1996;39(3):363-369. doi: 10.1002/art.1780390303Андрианова ИА, Иванова ММ. Ранний индекс повреждения у больных системной красной волчанкой. Научно-практическая ревматология. 2005;42(5):19-22.Andrianova IA, Ivanova MM. Early damage index in systemic lupus erythematosus. Nauchno-Prakticheskaya Revmatologia = Rheumatology Science and Practice. 2005;42(5):19-22 (In Russ.).Seror R, Bowman SJ, Brito-Zeron P, Theander E, Bootsma H, Tzioufas A, et al. EULAR Sjögren’s syndrome disease activity index (ESSDAI): A user guide. RMD Open. 2015;1(1):e000022. doi: 10.1136/rmdopen-2014-000022Seror R, Bowman SJ, Brito-Zeron P, Theander E, Bootsma H, Tzioufas A, et al. EULAR Sjögren’s syndrome disease activity index (ESSDAI): A user guide. RMD Open. 2015;1(1):e000022. doi: 10.1136/rmdopen-2014-000022Seethala S, Collins NP, Comerci G. An unusual etiology for elevation of activated partial thromboplastin time (aPTT) in SLE: Acquired hemophilia and lupus anticoagulant. Case Rep Hematol. 2013;2013:1-3. doi: 10.1155/2013/521785Seethala S, Collins NP, Comerci G. An unusual etiology for elevation of activated partial thromboplastin time (aPTT) in SLE: Acquired hemophilia and lupus anticoagulant. Case Rep Hematol. 2013;2013:1-3. doi: 10.1155/2013/521785Ye M, Deng R, Shen F, Hou Z, Lin L. Systemic lupus erythematosus with acquired hemophilia A: A case report and literature review. J Central South Univ (Med Sci). 2023;48(5):789-794. doi: 10.11817/j.issn.1672-7347.2023.220440Ye M, Deng R, Shen F, Hou Z, Lin L. Systemic lupus erythematosus with acquired hemophilia A: A case report and literature review. J Central South Univ (Med Sci). 2023;48(5):789-794. doi: 10.11817/j.issn.1672-7347.2023.220440Kruse-Jarres R, Kempton CL, Baudo F, Collins PW, Knoebl P, Leissinger CA, et al. Acquired hemophilia A: Updated review of evidence and treatment guidance. Am J Hematol. 2017;92(7):695-705. doi: 10.1002/ajh.24777Kruse-Jarres R, Kempton CL, Baudo F, Collins PW, Knoebl P, Leissinger CA, et al. Acquired hemophilia A: Updated review of evidence and treatment guidance. Am J Hematol. 2017;92(7):695-705. doi: 10.1002/ajh.24777Porru G, Mura V, Piga M, Ibba V, Vacca A, Cauli A, et al. Hemarthrosis as acute presentation of acquired hemophilia in a patient with systemic lupus erythematosus: Successful treatment and long-lasting remission. Clin Rheumatol. 2008;27(12):1581-1584. doi: 10.1007/s10067-008-0993-yPorru G, Mura V, Piga M, Ibba V, Vacca A, Cauli A, et al. Hemarthrosis as acute presentation of acquired hemophilia in a patient with systemic lupus erythematosus: Successful treatment and long-lasting remission. Clin Rheumatol. 2008;27(12):1581-1584. doi: 10.1007/s10067-008-0993-yCao XY, Li MT, Zhang X, Zhao Y, Zeng XF, Zhang FC, et al. Characteristics of acquired inhibitors to factor VIII and von Willebrand factor secondary to systemic lupus erythematosus: Experiences from a Chinese Tertiary Medical Center. J Clin Rheumatol. 2021;27(5):201-205. doi: 10.1097/RHU.0000000000001284Cao XY, Li MT, Zhang X, Zhao Y, Zeng XF, Zhang FC, et al. Characteristics of acquired inhibitors to factor VIII and von Willebrand factor secondary to systemic lupus erythematosus: Experiences from a Chinese Tertiary Medical Center. J Clin Rheumatol. 2021;27(5):201-205. doi: 10.1097/RHU.0000000000001284Brings HA, Waas JK, Mccrae KR, Baele HR, Goldstone J. Successful management of life-threatening hemorrhage in a patient with synchronous lupus anticoagulant and factor VIII inhibitor. J Vasc Surg. 2002;36(4):853-855. doi: 10.1067/mva.2002.127336Brings HA, Waas JK, Mccrae KR, Baele HR, Goldstone J. Successful management of life-threatening hemorrhage in a patient with synchronous lupus anticoagulant and factor VIII inhibitor. J Vasc Surg. 2002;36(4):853-855. doi: 10.1067/mva.2002.127336Aggarwal A, Grewal R, Green RJ, Boggio L, Green D, Weksler BB, et al. Rituximab for autoimmune haemophilia: A proposed treatment algorithm. Haemophilia. 2005;11(1):13-19. doi: 10.1111/j.1365-2516.2005.01060.xAggarwal A, Grewal R, Green RJ, Boggio L, Green D, Weksler BB, et al. Rituximab for autoimmune haemophilia: A proposed treatment algorithm. Haemophilia. 2005;11(1):13-19. doi: 10.1111/j.1365-2516.2005.01060.xStasi R, Brunetti M, Stipa E, Amadori S. Selective B-cell depletion with rituximab for the treatment of patients with acquired hemophilia. Blood. 2004;103(12):4424-4428. doi: 10.1182/blood-2003-11-4075Stasi R, Brunetti M, Stipa E, Amadori S. Selective B-cell depletion with rituximab for the treatment of patients with acquired hemophilia. Blood. 2004;103(12):4424-4428. doi: 10.1182/blood-2003-11-4075Collins P, Baudo F, Knoebl P, Lévesque H, Nemes L, Pellegrini F, et al.; EACH2 registry collaborators. Immunosuppression for acquired hemophilia A: Results from the European Acquired Haemophilia Registry (EACH2). Blood. 2012;120(1):47-55. doi: 10.1182/blood-2012-02-409185Collins P, Baudo F, Knoebl P, Lévesque H, Nemes L, Pellegrini F, et al.; EACH2 registry collaborators. Immunosuppression for acquired hemophilia A: Results from the European Acquired Haemophilia Registry (EACH2). Blood. 2012;120(1):47-55. doi: 10.1182/blood-2012-02-409185Delgado J, Jimenez-Yuste V, Hernandez-Navarro F, Villar A. Acquired haemophilia: Review and meta-analysis focused on therapy and prognostic factors. Br J Haematol. 2003;121(1):21-35. doi: 10.1046/j.1365-2141.2003.04162.xDelgado J, Jimenez-Yuste V, Hernandez-Navarro F, Villar A. Acquired haemophilia: Review and meta-analysis focused on therapy and prognostic factors. Br J Haematol. 2003;121(1):21-35. doi: 10.1046/j.1365-2141.2003.04162.xOnishi S, Hojo N, Sakai I, Yasukawa M, Hato T, Minamoto Y, et al. Rupture of the gallbladder in a patient with acquired factor VIII inhibitors and systemic lupus erythematosus. Intern Med. 2004;43(11):1073-1077. doi: 10.2169/internalmedicine.43.1073Onishi S, Hojo N, Sakai I, Yasukawa M, Hato T, Minamoto Y, et al. Rupture of the gallbladder in a patient with acquired factor VIII inhibitors and systemic lupus erythematosus. Intern Med. 2004;43(11):1073-1077. doi: 10.2169/internalmedicine.43.1073Rezaieyazdi Z, Sharifi-Doloui D, Hashemzadeh K, Shirdel A, Mansouritorghabeh H. Acquired haemophilia A in a woman with autoimmune hepatitis and systemic lupus erythematosus; Review of literature. Blood Coagulat Fibrinolys. 2012;23(1):71-74. doi: 10.1097/MBC.0b013e32834c6cceRezaieyazdi Z, Sharifi-Doloui D, Hashemzadeh K, Shirdel A, Mansouritorghabeh H. Acquired haemophilia A in a woman with autoimmune hepatitis and systemic lupus erythematosus; Review of literature. Blood Coagulat Fibrinolys. 2012;23(1):71-74. doi: 10.1097/MBC.0b013e32834c6cceKnoebl P, Marco P, Baudo F, Collins P, Huth-Kühne A, Nemes L, et al.; EACH2 Registry Contributors. Demographic and clinical data in acquired hemophilia A: Results from the European Acquired Haemophilia Registry (EACH2). J Thromb Haemost. 2012;10(4):622-631. doi: 10.1111/j.1538-7836.2012.04654.xKnoebl P, Marco P, Baudo F, Collins P, Huth-Kühne A, Nemes L, et al.; EACH2 Registry Contributors. Demographic and clinical data in acquired hemophilia A: Results from the European Acquired Haemophilia Registry (EACH2). J Thromb Haemost. 2012;10(4):622-631. doi: 10.1111/j.1538-7836.2012.04654.xShen P, Li J, Tu S, Chen G, Chen C. Acquired hemophilia A in a woman with systemic lupus erythematosus: A case report and review of literature. Medicine (United States). 2020;99(43):E22926. doi: 10.1097/MD.0000000000022926Shen P, Li J, Tu S, Chen G, Chen C. Acquired hemophilia A in a woman with systemic lupus erythematosus: A case report and review of literature. Medicine (United States). 2020;99(43):E22926. doi: 10.1097/MD.0000000000022926Beydon M, McCoy S, Nguyen Y, Sumida T, Mariette X, Seror R. Epidemiology of Sjögren syndrome. Nat Rev Rheumatol. 2024;20(3):158-169. doi: 10.1038/s41584-023-01057-6Beydon M, McCoy S, Nguyen Y, Sumida T, Mariette X, Seror R. Epidemiology of Sjögren syndrome. Nat Rev Rheumatol. 2024;20(3):158-169. doi: 10.1038/s41584-023-01057-6

The authors declare that there are no conflicts of interest present.