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Нарушения гемостаза у пациентов с системным AL-амилоидозом
Нарушения гемостаза у пациентов с системным AL-амилоидозом
Рехтина И.Г., Хышова В.А., Зозуля Н.И., Двирнык В.Н., Менделеева Л.П. Нарушения гемостаза у пациентов с системным AL-амилоидозом. Терапевтический архив. 2023;95(9):746–750.
DOI: 10.26442/00403660.2023.09.202372
© ООО «КОНСИЛИУМ МЕДИКУМ», 2023 г.
DOI: 10.26442/00403660.2023.09.202372
DOI: 10.26442/00403660.2023.09.202372
© ООО «КОНСИЛИУМ МЕДИКУМ», 2023 г.
________________________________________________
DOI: 10.26442/00403660.2023.09.202372
Материалы доступны только для специалистов сферы здравоохранения. Авторизуйтесь или зарегистрируйтесь.
Аннотация
Цель. Проанализировать частоту и характер геморрагических и тромботических осложнений у больных системным AL-амилоидозом и сопоставить с лабораторными изменениями системы гемостаза.
Материалы и методы. В проспективное исследование включены 40 пациентов с впервые диагностированным AL-амилоидозом. Для выявления амилоида всем пациентам выполняли трепанобиопсию костного мозга и биопсию двенадцатиперстной кишки, а 28 (70%) больным – биопсию пораженного органа. До начала терапии всем пациентам определяли количество тромбоцитов, активированное частичное тромбопластиновое время, тромбиновое время, концентрацию фибриногена, время ХIIа-зависимого фибринолиза, содержание антитромбина III, D-димера, активность факторов свертывания крови VIII, X и фактора фон Виллебранда. Статистическая часть исследования проводилась с использованием системного обеспечения IBM SPSS Statistics 2017 г. (SPSS, Chicago, IL, США).
Результаты. У 20 (50%) больных диагностировали геморрагии на коже и слизистых по типу сосудистой пурпуры. Тромбозы до начала терапии перенесли 7 (17,5%), в том числе тромбозы вен ног (5 пациентов), ишемический инсульт (2 пациента). Выявлена отчетливая прямая корреляционная связь тромботических осложнений с кожным геморрагическим синдромом (p=0,007). В 15 (75%) случаях кожный геморрагический синдром сопровождался гиперкоагуляционными сдвигами системы гемостаза. Из 20 пациентов с кожным геморрагическим синдромом у 19 (95%) пациентов отмечалось поражение почек, в том числе у 15 больных – нефротический синдром. Гематомного типа кровоточивости, а также тяжелых кровотечений не наблюдалось, в том числе после биопсии внутренних органов. По совокупности показателей гемостаза чаще наблюдался гиперкоагуляционный синдром (у 23; 56% больных). Гипокоагуляция диагностирована лишь у 2 (5%) пациентов с поражением печени, у 16 (39%) пациентов отмечалась нормокоагуляция.
Заключение. Кожный геморрагический синдром – наиболее частое клиническое проявление нарушений в системе гемостаза у больных AL-амилоидозом. Установлена взаимосвязь геморрагий на коже с нефротическим синдромом, что может свидетельствовать о едином патогенетическом механизме. Кожный геморрагический синдром ассоциирован с гиперкоагуляционными сдвигами показателей гемостаза и высоким риском развития тромботических осложнений.
Ключевые слова: AL-амилоидоз, первичный амилоидоз, гемостаз
Materials and methods. The prospective study included 40 patients with newly diagnosed AL-amyloidosis. To detect amyloid, all patients underwent bone marrow trephine biopsy and duodenal biopsy, and 28 (70%) patients underwent biopsy of the affected organ. Before the start of therapy, all patients were determined the platelet count, activated partial thromboplastin time, thrombin time, fibrinogen concentration, time of XIIa-dependent fibrinolysis, antithrombin III, D-dimer, activity of blood coagulation factors VIII, X and vWF. The statistical part of the study was carried out using the IBM SPSS Statistics 2017 system software (SPSS, Chicago, IL, USA).
Results. In 20 (50%) patients, hemorrhages on the skin and mucous membranes were diagnosed as vascular purpura. Before the start of therapy, 7 (17.5%) patients had thrombosis, including leg vein thrombosis (5 patients), ischemic stroke (2 patients). There was a direct correlation between thrombotic complications and cutaneous hemorrhagic syndrome (p=0.007). In 15 (75%) cases, cutaneous hemorrhagic syndrome was accompanied by hypercoagulable shifts in the hemostasis system. Of the 20 patients with cutaneous hemorrhagic syndrome, 19 (95%) patients had kidney damage, including 15 patients with nephrotic syndrome. Hematoma type of bleeding, as well as heavy bleeding was not observed, including after a biopsy of the internal organs. According to the totality of hemostasis indicators, hypercoagulation syndrome was more often observed (in 23; 56% of patients). Hypocoagulation was diagnosed only in 2 (5%) patients with liver damage, 16 (39%) patients had normocoagulation.
Conclusion. Cutaneous hemorrhagic syndrome is the most common clinical manifestation of disorders in the hemostasis system in patients with AL-amyloidosis. The relationship of hemorrhages on the skin with nephrotic syndrome has been established, which may indicate a single pathogenetic mechanism. Cutaneous hemorrhagic syndrome is associated with hypercoagulable shifts in hemostasis and a high risk of thrombotic complications.
Keywords: AL-amyloidosis, primary amyloidosis, hemostasis
Материалы и методы. В проспективное исследование включены 40 пациентов с впервые диагностированным AL-амилоидозом. Для выявления амилоида всем пациентам выполняли трепанобиопсию костного мозга и биопсию двенадцатиперстной кишки, а 28 (70%) больным – биопсию пораженного органа. До начала терапии всем пациентам определяли количество тромбоцитов, активированное частичное тромбопластиновое время, тромбиновое время, концентрацию фибриногена, время ХIIа-зависимого фибринолиза, содержание антитромбина III, D-димера, активность факторов свертывания крови VIII, X и фактора фон Виллебранда. Статистическая часть исследования проводилась с использованием системного обеспечения IBM SPSS Statistics 2017 г. (SPSS, Chicago, IL, США).
Результаты. У 20 (50%) больных диагностировали геморрагии на коже и слизистых по типу сосудистой пурпуры. Тромбозы до начала терапии перенесли 7 (17,5%), в том числе тромбозы вен ног (5 пациентов), ишемический инсульт (2 пациента). Выявлена отчетливая прямая корреляционная связь тромботических осложнений с кожным геморрагическим синдромом (p=0,007). В 15 (75%) случаях кожный геморрагический синдром сопровождался гиперкоагуляционными сдвигами системы гемостаза. Из 20 пациентов с кожным геморрагическим синдромом у 19 (95%) пациентов отмечалось поражение почек, в том числе у 15 больных – нефротический синдром. Гематомного типа кровоточивости, а также тяжелых кровотечений не наблюдалось, в том числе после биопсии внутренних органов. По совокупности показателей гемостаза чаще наблюдался гиперкоагуляционный синдром (у 23; 56% больных). Гипокоагуляция диагностирована лишь у 2 (5%) пациентов с поражением печени, у 16 (39%) пациентов отмечалась нормокоагуляция.
Заключение. Кожный геморрагический синдром – наиболее частое клиническое проявление нарушений в системе гемостаза у больных AL-амилоидозом. Установлена взаимосвязь геморрагий на коже с нефротическим синдромом, что может свидетельствовать о едином патогенетическом механизме. Кожный геморрагический синдром ассоциирован с гиперкоагуляционными сдвигами показателей гемостаза и высоким риском развития тромботических осложнений.
Ключевые слова: AL-амилоидоз, первичный амилоидоз, гемостаз
________________________________________________
Materials and methods. The prospective study included 40 patients with newly diagnosed AL-amyloidosis. To detect amyloid, all patients underwent bone marrow trephine biopsy and duodenal biopsy, and 28 (70%) patients underwent biopsy of the affected organ. Before the start of therapy, all patients were determined the platelet count, activated partial thromboplastin time, thrombin time, fibrinogen concentration, time of XIIa-dependent fibrinolysis, antithrombin III, D-dimer, activity of blood coagulation factors VIII, X and vWF. The statistical part of the study was carried out using the IBM SPSS Statistics 2017 system software (SPSS, Chicago, IL, USA).
Results. In 20 (50%) patients, hemorrhages on the skin and mucous membranes were diagnosed as vascular purpura. Before the start of therapy, 7 (17.5%) patients had thrombosis, including leg vein thrombosis (5 patients), ischemic stroke (2 patients). There was a direct correlation between thrombotic complications and cutaneous hemorrhagic syndrome (p=0.007). In 15 (75%) cases, cutaneous hemorrhagic syndrome was accompanied by hypercoagulable shifts in the hemostasis system. Of the 20 patients with cutaneous hemorrhagic syndrome, 19 (95%) patients had kidney damage, including 15 patients with nephrotic syndrome. Hematoma type of bleeding, as well as heavy bleeding was not observed, including after a biopsy of the internal organs. According to the totality of hemostasis indicators, hypercoagulation syndrome was more often observed (in 23; 56% of patients). Hypocoagulation was diagnosed only in 2 (5%) patients with liver damage, 16 (39%) patients had normocoagulation.
Conclusion. Cutaneous hemorrhagic syndrome is the most common clinical manifestation of disorders in the hemostasis system in patients with AL-amyloidosis. The relationship of hemorrhages on the skin with nephrotic syndrome has been established, which may indicate a single pathogenetic mechanism. Cutaneous hemorrhagic syndrome is associated with hypercoagulable shifts in hemostasis and a high risk of thrombotic complications.
Keywords: AL-amyloidosis, primary amyloidosis, hemostasis
Полный текст
Список литературы
1. Muchtar E, Gertz MA, Kyle RA, et al. A Modern Primer on Light Chain Amyloidosis in 592 Patients With Mass Spectrometry – Verified Typing. Mayo Clin Proc. 2019;94(3):472-83. DOI:10.1016/j.mayocp.2018.08.006
2. Yood RA, Skinner M, Rubinow A, Talarico L. CAS. Bleeding manifestations in 100 patients with amyloidosis. J Am Med Assoc. 1983;10(249):1922-324.
3. Eder L, Bitterman H. Amyloid Purpura. N Engl J Med. 2007;356(23):2406. DOI:10.1056/nejmicm061510
4. Wechalekar AD, Gillmore JD, Bird J, et al. Guidelines on the management of AL amyloidosis. Br J Haematol. 2015;168(2):186-206. DOI:10.1111/bjh.13155
5. Stoopler ET, Alawi F, Laudenbach JM, Sollecito TP. Bullous amyloidosis of the oral cavity: A rare clinical presentation and review. Oral Surgery, Oral Med Oral Pathol Oral Radiol Endodontology. 2006;101(6):734-40. DOI:10.1016/j.tripleo.2006.01.003
6. Abdallah N, Muchtar E, Dispenzieri A, et al. Coagulation Abnormalities in Light Chain Amyloidosis. Mayo Clin Proc. 2021;96(2):377-87. DOI:10.1016/j.mayocp.2020.06.061
7. Patel G, Hari P, Szabo A, et al. Acquired factor X deficiency in light-chain (AL) amyloidosis is rare and associated with advanced disease. Hematol Oncol Stem Cell Ther.
2019;12(1):10-4. DOI:10.1016/j.hemonc.2018.05.002
8. Nguyen AL, Kamal M, Raghavan R, Nagaraj G. Acquired factor VII deficiency causing severe bleeding disorder secondary to AL amyloidosis of the liver. Hematol Rep. 2018;10(3). DOI:10.4081/hr.2018.7235
9. Emori Y, Sakugawa M, Niiya K, et al. Life-threatening bleeding and acquired factor V deficiency associated with primary systemic amyloidosis. Blood Coagul Fibrinolysis. 2002;13(6):555-9. DOI:10.1097/00001721-200209000-00011
10. Wiest R, Klouche M, Härle P, et al. Acquired combined factor X and XII deficiency with isolated bleeding complications in a patient with AL amyloidosis. Ann Hematol.
2005;84(3):196-9. DOI:10.1007/s00277-004-0970-8
11. Gamba G, Montani N, Anesi E, et al. Clotting alterations in primary systemic amyloidosis. Haematologica. 2000;85(3):289-92.
12. Uchiba M, Imamura T, Hata H, et al. Excessive fibrinolysis in AL-amyloidosis is induced by urokinae-type plasminogen activator from bone marrow plasma cells. Amyloid. 2009;16(2):89-93. DOI:10.1080/13506120902879269
13. Pudusseri A, Sanchorawala V, Sloan JM, et al. Prevalence and prognostic value of D-dimer elevation in patients with AL amyloidosis. Am J Hematol. 2019;94(10):1098-103. DOI:10.1002/ajh.25576
14. Migrino RQ, Hari P, Gutterman DD, et al. Systemic and microvascular oxidative stress induced by light chain amyloidosis. Int J Cardiol. 2010;145(1):67-8. DOI:10.1016/j.ijcard.2009.04.044
15. Gertz MA, Kyle RA. Hepatic amyloidosis (Primary [AL], immunoglobulin light chain): The natural history in 80 patients. Am J Med. 1988;85(1):73‑80.
DOI:10.1016/0002-9343(88)90505-0
16. Park MA, Mueller PS, Kyle RA, et al. Primary (AL) hepatic amyloidosis: Clinical features and natural history in 98 patients. Medicine (Baltimore).
2003;82(5):291-8. DOI:10.1097/01.md.0000091183.93122.c7
17. Kujawski B, Johnson D, Radadia K, et al. An Uncommon Presentation of Amyloidosis. Med Forum. 2015;15(1):8-11. DOI:10.29046/tmf.015.1.004
18. Kerlin BA, Ayoob R, Smoyer WE. Epidemiology and pathophysiology of nephrotic syndrome-associated thromboembolic disease. Clin J Am Soc Nephrol. 2012;7(3):513-20. DOI:10.2215/CJN.10131011
19. Berghoff M, Kathpal M, Khan F, et al. Endothelial dysfunction precedes C-fiber abnormalities in primary (AL) amyloidosis. Ann Neurol. 2003;53(6):725-30. DOI:10.1002/ana.10552
20. Bright M, Truran S, Schlundt B, Gutterman DD. Endothelium-Independent Microvascular Dysfunction Induced by AL Amyloidosis Light Chains in Human Adipose Arterioles: Novel Mechanism of Amyloid Injury. Annu Sci Meet. 2009;13:178.
21. Kastritis E, Papassotiriou I, Terpos E, et al. Clinical and prognostic significance of serum levels of von Willebrand factor and ADAMTS-13 antigens in AL amyloidosis. Blood. 2016;128(3):405-9. DOI:10.1182/blood-2016-02-702696
22. Palladini G, Hegenbart U, Milani P, et al. A staging system for renal outcome and early markers of renal response to chemotherapy in AL amyloidosis. Blood. 2014;124(15):2325-32. DOI:10.1182/blood-2014-04-570010
23. Palladini G, Merlini G. What is new in diagnosis and management of light chain amyloidosis? Blood. 2016;128(2):159-68. DOI:10.1182/blood-2016-01-629790
24. Perfetti V, Palladini G, Casarini S, et al. The repertoire of λ light chains causing predominant amyloid heart involvement and identification of a preferentially involved germline gene, IGLV1-44. Blood. 2012;119(1):144-50. DOI:10.1182/blood-2011-05-355784
25. Abraham RS, Geyer SM, Price-Troska TL, et al. Immunoglobulin light chain variable (V) region genes influence clinical presentation and outcome in light chain-associated amyloidosis (AL). Blood. 2003;101(10):3801-8. DOI:10.1182/blood-2002-09-2707
26. Choufani EB, Sanchorawala V, Ernst T, et al. Acquired factor X deficiency in patients with amyloid light-chain amyloidosis: Incidence, bleeding manifestations, and response to high-dose chemotherapy. Blood. 2001;97(6):1885-7. DOI:10.1182/blood.V97.6.1885
27. Cordes S, Gertz MA, Buadi FK, et al. Autologous stem cell transplantation in immunoglobulin light chain amyloidosis with factor X deficiency. Blood Coagul Fibrinolysis. 2016;27(1):101-8. DOI:10.1097/MBC.0000000000000367
28. Bever KM, Masha LI, Sun F, et al. Risk factors for venous thromboembolism in immunoglobulin light chain amyloidosis. Haematologica. 2016;101(1):86-90. DOI:10.3324/haematol.2015.133900
29. Freeman B, Sloan JM, Seldin DC, et al. Multiple arterial and venous thromboembolic complications in AL amyloidosis and cardiac involvement: A case report and literature review. Amyloid. 2012;19(3):156-60. DOI:10.3109/13506129.2012.694825
30. Glassock RJ. Prophylactic anticoagulation in nephrotic syndrome: A clinical conundrum. J Am Soc Nephrol. 2007;18(8):2221‑5. DOI:10.1681/ASN.2006111300
31. January CT, Wann LS, Calkins H, et al. Guideline for the Management of Patients With Atrial Fibrillation. Circulation. 2019;140:e125‑51. DOI:10.1161/CIR.0000000000000665
2. Yood RA, Skinner M, Rubinow A, Talarico L. CAS. Bleeding manifestations in 100 patients with amyloidosis. J Am Med Assoc. 1983;10(249):1922-324.
3. Eder L, Bitterman H. Amyloid Purpura. N Engl J Med. 2007;356(23):2406. DOI:10.1056/nejmicm061510
4. Wechalekar AD, Gillmore JD, Bird J, et al. Guidelines on the management of AL amyloidosis. Br J Haematol. 2015;168(2):186-206. DOI:10.1111/bjh.13155
5. Stoopler ET, Alawi F, Laudenbach JM, Sollecito TP. Bullous amyloidosis of the oral cavity: A rare clinical presentation and review. Oral Surgery, Oral Med Oral Pathol Oral Radiol Endodontology. 2006;101(6):734-40. DOI:10.1016/j.tripleo.2006.01.003
6. Abdallah N, Muchtar E, Dispenzieri A, et al. Coagulation Abnormalities in Light Chain Amyloidosis. Mayo Clin Proc. 2021;96(2):377-87. DOI:10.1016/j.mayocp.2020.06.061
7. Patel G, Hari P, Szabo A, et al. Acquired factor X deficiency in light-chain (AL) amyloidosis is rare and associated with advanced disease. Hematol Oncol Stem Cell Ther.
2019;12(1):10-4. DOI:10.1016/j.hemonc.2018.05.002
8. Nguyen AL, Kamal M, Raghavan R, Nagaraj G. Acquired factor VII deficiency causing severe bleeding disorder secondary to AL amyloidosis of the liver. Hematol Rep. 2018;10(3). DOI:10.4081/hr.2018.7235
9. Emori Y, Sakugawa M, Niiya K, et al. Life-threatening bleeding and acquired factor V deficiency associated with primary systemic amyloidosis. Blood Coagul Fibrinolysis. 2002;13(6):555-9. DOI:10.1097/00001721-200209000-00011
10. Wiest R, Klouche M, Härle P, et al. Acquired combined factor X and XII deficiency with isolated bleeding complications in a patient with AL amyloidosis. Ann Hematol.
2005;84(3):196-9. DOI:10.1007/s00277-004-0970-8
11. Gamba G, Montani N, Anesi E, et al. Clotting alterations in primary systemic amyloidosis. Haematologica. 2000;85(3):289-92.
12. Uchiba M, Imamura T, Hata H, et al. Excessive fibrinolysis in AL-amyloidosis is induced by urokinae-type plasminogen activator from bone marrow plasma cells. Amyloid. 2009;16(2):89-93. DOI:10.1080/13506120902879269
13. Pudusseri A, Sanchorawala V, Sloan JM, et al. Prevalence and prognostic value of D-dimer elevation in patients with AL amyloidosis. Am J Hematol. 2019;94(10):1098-103. DOI:10.1002/ajh.25576
14. Migrino RQ, Hari P, Gutterman DD, et al. Systemic and microvascular oxidative stress induced by light chain amyloidosis. Int J Cardiol. 2010;145(1):67-8. DOI:10.1016/j.ijcard.2009.04.044
15. Gertz MA, Kyle RA. Hepatic amyloidosis (Primary [AL], immunoglobulin light chain): The natural history in 80 patients. Am J Med. 1988;85(1):73‑80.
DOI:10.1016/0002-9343(88)90505-0
16. Park MA, Mueller PS, Kyle RA, et al. Primary (AL) hepatic amyloidosis: Clinical features and natural history in 98 patients. Medicine (Baltimore).
2003;82(5):291-8. DOI:10.1097/01.md.0000091183.93122.c7
17. Kujawski B, Johnson D, Radadia K, et al. An Uncommon Presentation of Amyloidosis. Med Forum. 2015;15(1):8-11. DOI:10.29046/tmf.015.1.004
18. Kerlin BA, Ayoob R, Smoyer WE. Epidemiology and pathophysiology of nephrotic syndrome-associated thromboembolic disease. Clin J Am Soc Nephrol. 2012;7(3):513-20. DOI:10.2215/CJN.10131011
19. Berghoff M, Kathpal M, Khan F, et al. Endothelial dysfunction precedes C-fiber abnormalities in primary (AL) amyloidosis. Ann Neurol. 2003;53(6):725-30. DOI:10.1002/ana.10552
20. Bright M, Truran S, Schlundt B, Gutterman DD. Endothelium-Independent Microvascular Dysfunction Induced by AL Amyloidosis Light Chains in Human Adipose Arterioles: Novel Mechanism of Amyloid Injury. Annu Sci Meet. 2009;13:178.
21. Kastritis E, Papassotiriou I, Terpos E, et al. Clinical and prognostic significance of serum levels of von Willebrand factor and ADAMTS-13 antigens in AL amyloidosis. Blood. 2016;128(3):405-9. DOI:10.1182/blood-2016-02-702696
22. Palladini G, Hegenbart U, Milani P, et al. A staging system for renal outcome and early markers of renal response to chemotherapy in AL amyloidosis. Blood. 2014;124(15):2325-32. DOI:10.1182/blood-2014-04-570010
23. Palladini G, Merlini G. What is new in diagnosis and management of light chain amyloidosis? Blood. 2016;128(2):159-68. DOI:10.1182/blood-2016-01-629790
24. Perfetti V, Palladini G, Casarini S, et al. The repertoire of λ light chains causing predominant amyloid heart involvement and identification of a preferentially involved germline gene, IGLV1-44. Blood. 2012;119(1):144-50. DOI:10.1182/blood-2011-05-355784
25. Abraham RS, Geyer SM, Price-Troska TL, et al. Immunoglobulin light chain variable (V) region genes influence clinical presentation and outcome in light chain-associated amyloidosis (AL). Blood. 2003;101(10):3801-8. DOI:10.1182/blood-2002-09-2707
26. Choufani EB, Sanchorawala V, Ernst T, et al. Acquired factor X deficiency in patients with amyloid light-chain amyloidosis: Incidence, bleeding manifestations, and response to high-dose chemotherapy. Blood. 2001;97(6):1885-7. DOI:10.1182/blood.V97.6.1885
27. Cordes S, Gertz MA, Buadi FK, et al. Autologous stem cell transplantation in immunoglobulin light chain amyloidosis with factor X deficiency. Blood Coagul Fibrinolysis. 2016;27(1):101-8. DOI:10.1097/MBC.0000000000000367
28. Bever KM, Masha LI, Sun F, et al. Risk factors for venous thromboembolism in immunoglobulin light chain amyloidosis. Haematologica. 2016;101(1):86-90. DOI:10.3324/haematol.2015.133900
29. Freeman B, Sloan JM, Seldin DC, et al. Multiple arterial and venous thromboembolic complications in AL amyloidosis and cardiac involvement: A case report and literature review. Amyloid. 2012;19(3):156-60. DOI:10.3109/13506129.2012.694825
30. Glassock RJ. Prophylactic anticoagulation in nephrotic syndrome: A clinical conundrum. J Am Soc Nephrol. 2007;18(8):2221‑5. DOI:10.1681/ASN.2006111300
31. January CT, Wann LS, Calkins H, et al. Guideline for the Management of Patients With Atrial Fibrillation. Circulation. 2019;140:e125‑51. DOI:10.1161/CIR.0000000000000665
2. Yood RA, Skinner M, Rubinow A, Talarico L. CAS. Bleeding manifestations in 100 patients with amyloidosis. J Am Med Assoc. 1983;10(249):1922-324.
3. Eder L, Bitterman H. Amyloid Purpura. N Engl J Med. 2007;356(23):2406. DOI:10.1056/nejmicm061510
4. Wechalekar AD, Gillmore JD, Bird J, et al. Guidelines on the management of AL amyloidosis. Br J Haematol. 2015;168(2):186-206. DOI:10.1111/bjh.13155
5. Stoopler ET, Alawi F, Laudenbach JM, Sollecito TP. Bullous amyloidosis of the oral cavity: A rare clinical presentation and review. Oral Surgery, Oral Med Oral Pathol Oral Radiol Endodontology. 2006;101(6):734-40. DOI:10.1016/j.tripleo.2006.01.003
6. Abdallah N, Muchtar E, Dispenzieri A, et al. Coagulation Abnormalities in Light Chain Amyloidosis. Mayo Clin Proc. 2021;96(2):377-87. DOI:10.1016/j.mayocp.2020.06.061
7. Patel G, Hari P, Szabo A, et al. Acquired factor X deficiency in light-chain (AL) amyloidosis is rare and associated with advanced disease. Hematol Oncol Stem Cell Ther.
2019;12(1):10-4. DOI:10.1016/j.hemonc.2018.05.002
8. Nguyen AL, Kamal M, Raghavan R, Nagaraj G. Acquired factor VII deficiency causing severe bleeding disorder secondary to AL amyloidosis of the liver. Hematol Rep. 2018;10(3). DOI:10.4081/hr.2018.7235
9. Emori Y, Sakugawa M, Niiya K, et al. Life-threatening bleeding and acquired factor V deficiency associated with primary systemic amyloidosis. Blood Coagul Fibrinolysis. 2002;13(6):555-9. DOI:10.1097/00001721-200209000-00011
10. Wiest R, Klouche M, Härle P, et al. Acquired combined factor X and XII deficiency with isolated bleeding complications in a patient with AL amyloidosis. Ann Hematol.
2005;84(3):196-9. DOI:10.1007/s00277-004-0970-8
11. Gamba G, Montani N, Anesi E, et al. Clotting alterations in primary systemic amyloidosis. Haematologica. 2000;85(3):289-92.
12. Uchiba M, Imamura T, Hata H, et al. Excessive fibrinolysis in AL-amyloidosis is induced by urokinae-type plasminogen activator from bone marrow plasma cells. Amyloid. 2009;16(2):89-93. DOI:10.1080/13506120902879269
13. Pudusseri A, Sanchorawala V, Sloan JM, et al. Prevalence and prognostic value of D-dimer elevation in patients with AL amyloidosis. Am J Hematol. 2019;94(10):1098-103. DOI:10.1002/ajh.25576
14. Migrino RQ, Hari P, Gutterman DD, et al. Systemic and microvascular oxidative stress induced by light chain amyloidosis. Int J Cardiol. 2010;145(1):67-8. DOI:10.1016/j.ijcard.2009.04.044
15. Gertz MA, Kyle RA. Hepatic amyloidosis (Primary [AL], immunoglobulin light chain): The natural history in 80 patients. Am J Med. 1988;85(1):73‑80.
DOI:10.1016/0002-9343(88)90505-0
16. Park MA, Mueller PS, Kyle RA, et al. Primary (AL) hepatic amyloidosis: Clinical features and natural history in 98 patients. Medicine (Baltimore).
2003;82(5):291-8. DOI:10.1097/01.md.0000091183.93122.c7
17. Kujawski B, Johnson D, Radadia K, et al. An Uncommon Presentation of Amyloidosis. Med Forum. 2015;15(1):8-11. DOI:10.29046/tmf.015.1.004
18. Kerlin BA, Ayoob R, Smoyer WE. Epidemiology and pathophysiology of nephrotic syndrome-associated thromboembolic disease. Clin J Am Soc Nephrol. 2012;7(3):513-20. DOI:10.2215/CJN.10131011
19. Berghoff M, Kathpal M, Khan F, et al. Endothelial dysfunction precedes C-fiber abnormalities in primary (AL) amyloidosis. Ann Neurol. 2003;53(6):725-30. DOI:10.1002/ana.10552
20. Bright M, Truran S, Schlundt B, Gutterman DD. Endothelium-Independent Microvascular Dysfunction Induced by AL Amyloidosis Light Chains in Human Adipose Arterioles: Novel Mechanism of Amyloid Injury. Annu Sci Meet. 2009;13:178.
21. Kastritis E, Papassotiriou I, Terpos E, et al. Clinical and prognostic significance of serum levels of von Willebrand factor and ADAMTS-13 antigens in AL amyloidosis. Blood. 2016;128(3):405-9. DOI:10.1182/blood-2016-02-702696
22. Palladini G, Hegenbart U, Milani P, et al. A staging system for renal outcome and early markers of renal response to chemotherapy in AL amyloidosis. Blood. 2014;124(15):2325-32. DOI:10.1182/blood-2014-04-570010
23. Palladini G, Merlini G. What is new in diagnosis and management of light chain amyloidosis? Blood. 2016;128(2):159-68. DOI:10.1182/blood-2016-01-629790
24. Perfetti V, Palladini G, Casarini S, et al. The repertoire of λ light chains causing predominant amyloid heart involvement and identification of a preferentially involved germline gene, IGLV1-44. Blood. 2012;119(1):144-50. DOI:10.1182/blood-2011-05-355784
25. Abraham RS, Geyer SM, Price-Troska TL, et al. Immunoglobulin light chain variable (V) region genes influence clinical presentation and outcome in light chain-associated amyloidosis (AL). Blood. 2003;101(10):3801-8. DOI:10.1182/blood-2002-09-2707
26. Choufani EB, Sanchorawala V, Ernst T, et al. Acquired factor X deficiency in patients with amyloid light-chain amyloidosis: Incidence, bleeding manifestations, and response to high-dose chemotherapy. Blood. 2001;97(6):1885-7. DOI:10.1182/blood.V97.6.1885
27. Cordes S, Gertz MA, Buadi FK, et al. Autologous stem cell transplantation in immunoglobulin light chain amyloidosis with factor X deficiency. Blood Coagul Fibrinolysis. 2016;27(1):101-8. DOI:10.1097/MBC.0000000000000367
28. Bever KM, Masha LI, Sun F, et al. Risk factors for venous thromboembolism in immunoglobulin light chain amyloidosis. Haematologica. 2016;101(1):86-90. DOI:10.3324/haematol.2015.133900
29. Freeman B, Sloan JM, Seldin DC, et al. Multiple arterial and venous thromboembolic complications in AL amyloidosis and cardiac involvement: A case report and literature review. Amyloid. 2012;19(3):156-60. DOI:10.3109/13506129.2012.694825
30. Glassock RJ. Prophylactic anticoagulation in nephrotic syndrome: A clinical conundrum. J Am Soc Nephrol. 2007;18(8):2221‑5. DOI:10.1681/ASN.2006111300
31. January CT, Wann LS, Calkins H, et al. Guideline for the Management of Patients With Atrial Fibrillation. Circulation. 2019;140:e125‑51. DOI:10.1161/CIR.0000000000000665
________________________________________________
2. Yood RA, Skinner M, Rubinow A, Talarico L. CAS. Bleeding manifestations in 100 patients with amyloidosis. J Am Med Assoc. 1983;10(249):1922-324.
3. Eder L, Bitterman H. Amyloid Purpura. N Engl J Med. 2007;356(23):2406. DOI:10.1056/nejmicm061510
4. Wechalekar AD, Gillmore JD, Bird J, et al. Guidelines on the management of AL amyloidosis. Br J Haematol. 2015;168(2):186-206. DOI:10.1111/bjh.13155
5. Stoopler ET, Alawi F, Laudenbach JM, Sollecito TP. Bullous amyloidosis of the oral cavity: A rare clinical presentation and review. Oral Surgery, Oral Med Oral Pathol Oral Radiol Endodontology. 2006;101(6):734-40. DOI:10.1016/j.tripleo.2006.01.003
6. Abdallah N, Muchtar E, Dispenzieri A, et al. Coagulation Abnormalities in Light Chain Amyloidosis. Mayo Clin Proc. 2021;96(2):377-87. DOI:10.1016/j.mayocp.2020.06.061
7. Patel G, Hari P, Szabo A, et al. Acquired factor X deficiency in light-chain (AL) amyloidosis is rare and associated with advanced disease. Hematol Oncol Stem Cell Ther.
2019;12(1):10-4. DOI:10.1016/j.hemonc.2018.05.002
8. Nguyen AL, Kamal M, Raghavan R, Nagaraj G. Acquired factor VII deficiency causing severe bleeding disorder secondary to AL amyloidosis of the liver. Hematol Rep. 2018;10(3). DOI:10.4081/hr.2018.7235
9. Emori Y, Sakugawa M, Niiya K, et al. Life-threatening bleeding and acquired factor V deficiency associated with primary systemic amyloidosis. Blood Coagul Fibrinolysis. 2002;13(6):555-9. DOI:10.1097/00001721-200209000-00011
10. Wiest R, Klouche M, Härle P, et al. Acquired combined factor X and XII deficiency with isolated bleeding complications in a patient with AL amyloidosis. Ann Hematol.
2005;84(3):196-9. DOI:10.1007/s00277-004-0970-8
11. Gamba G, Montani N, Anesi E, et al. Clotting alterations in primary systemic amyloidosis. Haematologica. 2000;85(3):289-92.
12. Uchiba M, Imamura T, Hata H, et al. Excessive fibrinolysis in AL-amyloidosis is induced by urokinae-type plasminogen activator from bone marrow plasma cells. Amyloid. 2009;16(2):89-93. DOI:10.1080/13506120902879269
13. Pudusseri A, Sanchorawala V, Sloan JM, et al. Prevalence and prognostic value of D-dimer elevation in patients with AL amyloidosis. Am J Hematol. 2019;94(10):1098-103. DOI:10.1002/ajh.25576
14. Migrino RQ, Hari P, Gutterman DD, et al. Systemic and microvascular oxidative stress induced by light chain amyloidosis. Int J Cardiol. 2010;145(1):67-8. DOI:10.1016/j.ijcard.2009.04.044
15. Gertz MA, Kyle RA. Hepatic amyloidosis (Primary [AL], immunoglobulin light chain): The natural history in 80 patients. Am J Med. 1988;85(1):73‑80.
DOI:10.1016/0002-9343(88)90505-0
16. Park MA, Mueller PS, Kyle RA, et al. Primary (AL) hepatic amyloidosis: Clinical features and natural history in 98 patients. Medicine (Baltimore).
2003;82(5):291-8. DOI:10.1097/01.md.0000091183.93122.c7
17. Kujawski B, Johnson D, Radadia K, et al. An Uncommon Presentation of Amyloidosis. Med Forum. 2015;15(1):8-11. DOI:10.29046/tmf.015.1.004
18. Kerlin BA, Ayoob R, Smoyer WE. Epidemiology and pathophysiology of nephrotic syndrome-associated thromboembolic disease. Clin J Am Soc Nephrol. 2012;7(3):513-20. DOI:10.2215/CJN.10131011
19. Berghoff M, Kathpal M, Khan F, et al. Endothelial dysfunction precedes C-fiber abnormalities in primary (AL) amyloidosis. Ann Neurol. 2003;53(6):725-30. DOI:10.1002/ana.10552
20. Bright M, Truran S, Schlundt B, Gutterman DD. Endothelium-Independent Microvascular Dysfunction Induced by AL Amyloidosis Light Chains in Human Adipose Arterioles: Novel Mechanism of Amyloid Injury. Annu Sci Meet. 2009;13:178.
21. Kastritis E, Papassotiriou I, Terpos E, et al. Clinical and prognostic significance of serum levels of von Willebrand factor and ADAMTS-13 antigens in AL amyloidosis. Blood. 2016;128(3):405-9. DOI:10.1182/blood-2016-02-702696
22. Palladini G, Hegenbart U, Milani P, et al. A staging system for renal outcome and early markers of renal response to chemotherapy in AL amyloidosis. Blood. 2014;124(15):2325-32. DOI:10.1182/blood-2014-04-570010
23. Palladini G, Merlini G. What is new in diagnosis and management of light chain amyloidosis? Blood. 2016;128(2):159-68. DOI:10.1182/blood-2016-01-629790
24. Perfetti V, Palladini G, Casarini S, et al. The repertoire of λ light chains causing predominant amyloid heart involvement and identification of a preferentially involved germline gene, IGLV1-44. Blood. 2012;119(1):144-50. DOI:10.1182/blood-2011-05-355784
25. Abraham RS, Geyer SM, Price-Troska TL, et al. Immunoglobulin light chain variable (V) region genes influence clinical presentation and outcome in light chain-associated amyloidosis (AL). Blood. 2003;101(10):3801-8. DOI:10.1182/blood-2002-09-2707
26. Choufani EB, Sanchorawala V, Ernst T, et al. Acquired factor X deficiency in patients with amyloid light-chain amyloidosis: Incidence, bleeding manifestations, and response to high-dose chemotherapy. Blood. 2001;97(6):1885-7. DOI:10.1182/blood.V97.6.1885
27. Cordes S, Gertz MA, Buadi FK, et al. Autologous stem cell transplantation in immunoglobulin light chain amyloidosis with factor X deficiency. Blood Coagul Fibrinolysis. 2016;27(1):101-8. DOI:10.1097/MBC.0000000000000367
28. Bever KM, Masha LI, Sun F, et al. Risk factors for venous thromboembolism in immunoglobulin light chain amyloidosis. Haematologica. 2016;101(1):86-90. DOI:10.3324/haematol.2015.133900
29. Freeman B, Sloan JM, Seldin DC, et al. Multiple arterial and venous thromboembolic complications in AL amyloidosis and cardiac involvement: A case report and literature review. Amyloid. 2012;19(3):156-60. DOI:10.3109/13506129.2012.694825
30. Glassock RJ. Prophylactic anticoagulation in nephrotic syndrome: A clinical conundrum. J Am Soc Nephrol. 2007;18(8):2221‑5. DOI:10.1681/ASN.2006111300
31. January CT, Wann LS, Calkins H, et al. Guideline for the Management of Patients With Atrial Fibrillation. Circulation. 2019;140:e125‑51. DOI:10.1161/CIR.0000000000000665
Авторы
И.Г. Рехтина, В.А. Хышова*, Н.И. Зозуля, В.Н. Двирнык, Л.П. Менделеева
ФГБУ «Национальный медицинский исследовательский центр гематологии» Минздрава России, Москва, Россия
*viktoria2102@icloud.com
National Medical Research Center for Hematology, Moscow, Russia
*viktoria2102@icloud.com
ФГБУ «Национальный медицинский исследовательский центр гематологии» Минздрава России, Москва, Россия
*viktoria2102@icloud.com
________________________________________________
National Medical Research Center for Hematology, Moscow, Russia
*viktoria2102@icloud.com
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