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Prothrombin deficiency

MedGen UID:
1651913
•Concept ID:
C4722227
•
Disease or Syndrome
Synonym: Hypoprothrombinemias
 
Monarch Initiative: MONDO:0024307

Definition

A coagulation disorder characterized by the partial or complete absence of prothrombin (factor II) activity in the blood. [from NCI]

Professional guidelines

PubMed

Coagulation management and transfusion in massive postpartum hemorrhage.
Massoth C, Wenk M, Meybohm P, Kranke P
Curr Opin Anaesthesiol 2023 Jun 1;36(3):281-287. Epub 2023 Feb 22 doi: 10.1097/ACO.0000000000001258. PMID: 36815533
Livedoid vasculopathy: A review of pathogenesis and principles of management.
Vasudevan B, Neema S, Verma R
Indian J Dermatol Venereol Leprol 2016 Sep-Oct;82(5):478-88. doi: 10.4103/0378-6323.183635. PMID: 27297279
Prenatal diagnosis of hemoglobinopathies by DNA analysis.
Boehm C, Kazazian HH Jr
Crit Rev Oncol Hematol 1985;4(2):155-67. doi: 10.1016/s1040-8428(85)80014-7. PMID: 2998637

Recent clinical studies

Etiology

Lupus anticoagulant-hypoprothrombinemia syndrome and immunoglobulin-A vasculitis: a report of Japanese sibling cases and review of the literature.
Fujiwara K, Shimizu J, Tsukahara H, Shimada A
Rheumatol Int 2019 Oct;39(10):1811-1819. Epub 2019 Aug 7 doi: 10.1007/s00296-019-04404-7. PMID: 31392497
Bleeding manifestations in heterozygotes with prothrombin deficiency or abnormalities vs. unaffected family members as observed during a long follow-up study.
Girolami A, Santarossa C, Cosi E, Ferrari S, Lombardi AM, Girolami B
Blood Coagul Fibrinolysis 2017 Dec;28(8):623-626. doi: 10.1097/MBC.0000000000000648. PMID: 28665815
Pregnancy and oral contraceptives in congenital bleeding disorders of the vitamin K-dependent coagulation factors.
Girolami A, Randi ML, Ruzzon E, Lombardi AM, Girolami B, Fabris F
Acta Haematol 2006;115(1-2):58-63. doi: 10.1159/000089467. PMID: 16424651
Prenatal diagnosis of hemoglobinopathies by DNA analysis.
Boehm C, Kazazian HH Jr
Crit Rev Oncol Hematol 1985;4(2):155-67. doi: 10.1016/s1040-8428(85)80014-7. PMID: 2998637
Inherited bleeding syndromes in Iraq.
Al-Mondhiry HA
Thromb Haemost 1977 Jun 30;37(3):549-55. PMID: 302041

Diagnosis

Lupus anticoagulant-hypoprothrombinemia syndrome and immunoglobulin-A vasculitis: a report of Japanese sibling cases and review of the literature.
Fujiwara K, Shimizu J, Tsukahara H, Shimada A
Rheumatol Int 2019 Oct;39(10):1811-1819. Epub 2019 Aug 7 doi: 10.1007/s00296-019-04404-7. PMID: 31392497
Bleeding in the antiphospholipid syndrome.
Forastiero R
Hematology 2012 Apr;17 Suppl 1:S153-5. doi: 10.1179/102453312X13336169156654. PMID: 22507807
Hereditary prothrombin deficiency.
Ejaz MS, Latif N, Memon A
J Pak Med Assoc 2009 Sep;59(9):637-9. PMID: 19750864
Congenital deficiencies and abnormalities of prothrombin.
Girolami A, Scarano L, Saggiorato G, Girolami B, Bertomoro A, Marchiori A
Blood Coagul Fibrinolysis 1998 Oct;9(7):557-69. doi: 10.1097/00001721-199810000-00001. PMID: 9863703
Prenatal diagnosis of hemoglobinopathies by DNA analysis.
Boehm C, Kazazian HH Jr
Crit Rev Oncol Hematol 1985;4(2):155-67. doi: 10.1016/s1040-8428(85)80014-7. PMID: 2998637

Therapy

Congenital prothrombin deficiency.
Lancellotti S, De Cristofaro R
Semin Thromb Hemost 2009 Jun;35(4):367-81. Epub 2009 Jul 13 doi: 10.1055/s-0029-1225759. PMID: 19598065
Life-threatening bleeding, pregnancy and lupus anticoagulant: success after steroid and anticoagulant therapy.
Alijotas-Reig J, Ferrer-Raventos JC
Am J Reprod Immunol 2004 Aug;52(2):129-32. doi: 10.1111/j.1600-0897.2004.00197.x. PMID: 15274653
Chromogenic substrate (S-2238) prothrombin assay in prothrombin deficiencies and abnormalities. Lack of identity with clotting assays in congenital dysprothrombinemias.
Girolami A, Patrassi G, Toffanin F, Saggin L
Am J Clin Pathol 1980 Jul;74(1):83-7. doi: 10.1093/ajcp/74.1.83. PMID: 7395819
Hereditary prothrombin deficiency.
Kattlove HE, Shapiro SS, Spivack M
N Engl J Med 1970 Jan 8;282(2):57-61. doi: 10.1056/NEJM197001082820202. PMID: 4982446
Hypoprothrombinaemia in the newborn.
DOUGLAS AS, DAVIES P
Arch Dis Child 1955 Dec;30(154):509-12. doi: 10.1136/adc.30.154.509. PMID: 13275981Free PMC Article

Prognosis

Lupus anticoagulant-hypoprothrombinemia syndrome and immunoglobulin-A vasculitis: a report of Japanese sibling cases and review of the literature.
Fujiwara K, Shimizu J, Tsukahara H, Shimada A
Rheumatol Int 2019 Oct;39(10):1811-1819. Epub 2019 Aug 7 doi: 10.1007/s00296-019-04404-7. PMID: 31392497
A novel congenital dysprothrombinemia leading to defective prothrombin maturation.
Bafunno V, Bury L, Tiscia GL, Fierro T, Favuzzi G, Caliandro R, Sessa F, Grandone E, Margaglione M, Gresele P
Thromb Res 2014 Nov;134(5):1135-41. Epub 2014 Sep 8 doi: 10.1016/j.thromres.2014.08.028. PMID: 25242243
Congenital deficiencies and abnormalities of prothrombin.
Girolami A, Scarano L, Saggiorato G, Girolami B, Bertomoro A, Marchiori A
Blood Coagul Fibrinolysis 1998 Oct;9(7):557-69. doi: 10.1097/00001721-199810000-00001. PMID: 9863703
Transient lupus anticoagulant associated with prothrombin deficiency: unusual cause of bleeding in a 5-year-old girl.
Humphries JE, Acker MN, Pinkston JE, Ruddy S
Am J Pediatr Hematol Oncol 1994 Nov;16(4):372-6. PMID: 7978060
Prenatal diagnosis of hemoglobinopathies by DNA analysis.
Boehm C, Kazazian HH Jr
Crit Rev Oncol Hematol 1985;4(2):155-67. doi: 10.1016/s1040-8428(85)80014-7. PMID: 2998637

Clinical prediction guides

Lupus anticoagulant-hypoprothrombinemia syndrome and immunoglobulin-A vasculitis: a report of Japanese sibling cases and review of the literature.
Fujiwara K, Shimizu J, Tsukahara H, Shimada A
Rheumatol Int 2019 Oct;39(10):1811-1819. Epub 2019 Aug 7 doi: 10.1007/s00296-019-04404-7. PMID: 31392497
Congenital prothrombin defects: they are not only associated with bleeding but also with thrombosis: a new classification is needed.
Girolami A, Ferrari S, Cosi E, Girolami B, Lombardi AM
Hematology 2018 Mar;23(2):105-110. Epub 2017 Aug 1 doi: 10.1080/10245332.2017.1359900. PMID: 28762299
Bleeding in the antiphospholipid syndrome.
Forastiero R
Hematology 2012 Apr;17 Suppl 1:S153-5. doi: 10.1179/102453312X13336169156654. PMID: 22507807
Congenital deficiencies and abnormalities of prothrombin.
Girolami A, Scarano L, Saggiorato G, Girolami B, Bertomoro A, Marchiori A
Blood Coagul Fibrinolysis 1998 Oct;9(7):557-69. doi: 10.1097/00001721-199810000-00001. PMID: 9863703
Prenatal diagnosis of hemoglobinopathies by DNA analysis.
Boehm C, Kazazian HH Jr
Crit Rev Oncol Hematol 1985;4(2):155-67. doi: 10.1016/s1040-8428(85)80014-7. PMID: 2998637

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