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Acquired factor X deficiency

MedGen UID:
543977
•Concept ID:
C0272328
•
Disease or Syndrome
Synonyms: Acquired factor 10 deficiency; Acquired Factor X Deficiency; acquired factor X deficiency; Acquired factor X deficiency disease; Acquired Stuart factor deficiency disease; aFX
SNOMED CT: Acquired factor X deficiency (33820001); Acquired factor 10 deficiency (33820001); Acquired factor X deficiency disease (33820001); Acquired Stuart factor deficiency disease (33820001)
 
Monarch Initiative: MONDO:0021134
Orphanet: ORPHA599501

Definition

An acquired coagulation disorder characterized by the partial or complete absence of factor X activity in the blood. [from NCI]

Professional guidelines

PubMed

Utility of factor X concentrate for the treatment of acquired factor X deficiency in systemic light-chain amyloidosis.
Mahmood S, Blundell J, Drebes A, Hawkins PN, Wechalekar AD
Blood 2014 May 1;123(18):2899-900. doi: 10.1182/blood-2014-02-556514. PMID: 24786460

Recent clinical studies

Etiology

Management of Acquired Factor X Deficiency Caused by In Vitro Non-neutralizing Anti-factor X Autoantibodies with Pre-emptive Corticosteroid Therapy.
Onishi A, Shimura Y, Nakamura T, Souri M, Osaki T, Mizutani S, Tsukamoto T, Kobayashi T, Ichinose A, Kuroda J
Intern Med 2023;62(16):2401-2406. Epub 2023 Aug 15 doi: 10.2169/internalmedicine.0849-22. PMID: 37587057Free PMC Article
Acquired Factor X Deficiency in Patients With Primary Light Chain Amyloidosis.
Dejhansathit S, Suvannasankha A
J Investig Med High Impact Case Rep 2019 Jan-Dec;7:2324709619832332. doi: 10.1177/2324709619832332. PMID: 30947547Free PMC Article
Autologous stem cell transplantation in immunoglobulin light chain amyloidosis with factor X deficiency.
Cordes S, Gertz MA, Buadi FK, Lin Y, Lacy MQ, Kapoor P, Kumar SK, McCurdy A, Dispenzieri A, Dingli D, Hayman SR, Hogan WJ, Pruthi RK
Blood Coagul Fibrinolysis 2016 Jan;27(1):101-8. doi: 10.1097/MBC.0000000000000367. PMID: 26218969
Splenectomy in plasma cell dyscrasias: a review of the clinical practice.
Kapoor P, Singh E, Radhakrishnan P, Mehta P
Am J Hematol 2006 Dec;81(12):946-54. doi: 10.1002/ajh.20736. PMID: 16937390
Factor X deficiency.
Uprichard J, Perry DJ
Blood Rev 2002 Jun;16(2):97-110. doi: 10.1054/blre.2002.0191. PMID: 12127953

Diagnosis

Acquired factor X deficiency in a multiple myeloma without amyloidosis: a case report.
Coussee A, Alliet G, Hervent AS, Vynckier L, Emmerechts J, Van Hecke S, Persijn L
Acta Clin Belg 2023 Dec;78(6):524-528. Epub 2023 Oct 16 doi: 10.1080/17843286.2023.2265650. PMID: 37800976
Identification of non-neutralizing anti-factor X autoantibodies in three Japanese cases of autoimmune acquired factor X deficiency.
Souri M, Osaki T, Shimura Y, Ichikawa S, Mori M, Ogawa Y, Ichinose A
Haemophilia 2023 Mar;29(2):555-563. Epub 2022 Dec 8 doi: 10.1111/hae.14711. PMID: 36478471
Acquired factor X deficiency associated with atypical AL-amyloidosis.
Furuhata M, Doki N, Hishima T, Okamoto T, Koyama T, Kaito S, Oshikawa G, Kobayashi T, Kakihana K, Sakamaki H, Ohashi K
Intern Med 2014;53(16):1841-5. Epub 2014 Aug 15 doi: 10.2169/internalmedicine.53.2191. PMID: 25130122
Acquired-transient factor X deficiency in a teenager with extensive burns.
Mascarenhas A, Eusébio M, Freitas O, Almeida T
BMJ Case Rep 2011 Feb 17;2011 doi: 10.1136/bcr.12.2010.3618. PMID: 22707501Free PMC Article
Factor X deficiency.
Uprichard J, Perry DJ
Blood Rev 2002 Jun;16(2):97-110. doi: 10.1054/blre.2002.0191. PMID: 12127953

Therapy

Role of individual factor X concentrate pharmacokinetic studies in perioperative management of AL amyloidosis-associated acquired factor X deficiency.
Lewis AR, Peffley N, Klompas AM, Ashrani AA
Transfusion 2023 Sep;63(9):1773-1777. Epub 2023 Jul 10 doi: 10.1111/trf.17477. PMID: 37427705
Acquired Factor X Deficiency in Patients With Primary Light Chain Amyloidosis.
Dejhansathit S, Suvannasankha A
J Investig Med High Impact Case Rep 2019 Jan-Dec;7:2324709619832332. doi: 10.1177/2324709619832332. PMID: 30947547Free PMC Article
Acquired factor X deficiency in light-chain (AL) amyloidosis is rare and associated with advanced disease.
Patel G, Hari P, Szabo A, Rein L, Kreuziger LB, Chhabra S, Dhakal B, D'Souza A
Hematol Oncol Stem Cell Ther 2019 Mar;12(1):10-14. Epub 2018 Jun 28 doi: 10.1016/j.hemonc.2018.05.002. PMID: 30879471
Systemic AL amyloidosis with acquired factor X deficiency: A study of perioperative bleeding risk and treatment outcomes in 60 patients.
Thompson CA, Kyle R, Gertz M, Heit J, Pruthi R, Pardanani A
Am J Hematol 2010 Mar;85(3):171-3. doi: 10.1002/ajh.21603. PMID: 20052750Free PMC Article
Splenectomy in plasma cell dyscrasias: a review of the clinical practice.
Kapoor P, Singh E, Radhakrishnan P, Mehta P
Am J Hematol 2006 Dec;81(12):946-54. doi: 10.1002/ajh.20736. PMID: 16937390

Prognosis

Acquired factor X deficiency in light-chain (AL) amyloidosis is rare and associated with advanced disease.
Patel G, Hari P, Szabo A, Rein L, Kreuziger LB, Chhabra S, Dhakal B, D'Souza A
Hematol Oncol Stem Cell Ther 2019 Mar;12(1):10-14. Epub 2018 Jun 28 doi: 10.1016/j.hemonc.2018.05.002. PMID: 30879471
The Long-term Outcomes after VAD plus SCT Therapy in a Patient with AL Amyloidosis and Severe Factor X Deficiency.
Iwadate D, Hasegawa E, Hoshino J, Hayami N, Sumida K, Yamanouchi M, Sekine A, Kawada M, Hiramatsu R, Suwabe T, Sawa N, Yuasa M, Wake A, Fujii T, Ohashi K, Takaichi K, Ubara Y
Intern Med 2018 Mar 1;57(5):701-706. Epub 2017 Nov 1 doi: 10.2169/internalmedicine.9263-17. PMID: 29093408Free PMC Article
Importance of pharmacokinetic studies in the management of acquired factor X deficiency.
Lim MY, McCarthy T, Chen SL, Rollins-Raval MA, Ma AD
Eur J Haematol 2016 Jan;96(1):60-4. Epub 2015 May 18 doi: 10.1111/ejh.12548. PMID: 25782416
Systemic AL amyloidosis with acquired factor X deficiency: A study of perioperative bleeding risk and treatment outcomes in 60 patients.
Thompson CA, Kyle R, Gertz M, Heit J, Pruthi R, Pardanani A
Am J Hematol 2010 Mar;85(3):171-3. doi: 10.1002/ajh.21603. PMID: 20052750Free PMC Article
Life-threatening bleeding and acquired factor V deficiency associated with primary systemic amyloidosis.
Emori Y, Sakugawa M, Niiya K, Kiguchi T, Kojima K, Takenaka K, Shinagawa K, Ishimaru F, Ikeda K, Tanimoto M, Yamasaki R, Ohara N, Harada M
Blood Coagul Fibrinolysis 2002 Sep;13(6):555-9. doi: 10.1097/00001721-200209000-00011. PMID: 12192308

Clinical prediction guides

Acquired factor X deficiency in a multiple myeloma without amyloidosis: a case report.
Coussee A, Alliet G, Hervent AS, Vynckier L, Emmerechts J, Van Hecke S, Persijn L
Acta Clin Belg 2023 Dec;78(6):524-528. Epub 2023 Oct 16 doi: 10.1080/17843286.2023.2265650. PMID: 37800976
Acquired factor X deficiency in light-chain (AL) amyloidosis is rare and associated with advanced disease.
Patel G, Hari P, Szabo A, Rein L, Kreuziger LB, Chhabra S, Dhakal B, D'Souza A
Hematol Oncol Stem Cell Ther 2019 Mar;12(1):10-14. Epub 2018 Jun 28 doi: 10.1016/j.hemonc.2018.05.002. PMID: 30879471
Importance of pharmacokinetic studies in the management of acquired factor X deficiency.
Lim MY, McCarthy T, Chen SL, Rollins-Raval MA, Ma AD
Eur J Haematol 2016 Jan;96(1):60-4. Epub 2015 May 18 doi: 10.1111/ejh.12548. PMID: 25782416
Acquired factor X deficiency associated with atypical AL-amyloidosis.
Furuhata M, Doki N, Hishima T, Okamoto T, Koyama T, Kaito S, Oshikawa G, Kobayashi T, Kakihana K, Sakamaki H, Ohashi K
Intern Med 2014;53(16):1841-5. Epub 2014 Aug 15 doi: 10.2169/internalmedicine.53.2191. PMID: 25130122
Systemic AL amyloidosis with acquired factor X deficiency: A study of perioperative bleeding risk and treatment outcomes in 60 patients.
Thompson CA, Kyle R, Gertz M, Heit J, Pruthi R, Pardanani A
Am J Hematol 2010 Mar;85(3):171-3. doi: 10.1002/ajh.21603. PMID: 20052750Free PMC Article

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