関: factor II deficiency、prothrombin deficiency

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2012/04/15 04:04:05」(JST)

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Hypoprothrombinemia is a blood disorder in which a deficiency of prothrombin (Factor II) results in impaired blood clotting, leading to an increased physiological risk for bleeding, especially in the gastrointestinal system, cranial vault, and superficial integumentary system.

Causes

Hypoprothrombinemia can be the result of a genetic defect or it may be acquired as the result of another disease process, or an adverse effect of medication. For example, 5-10% of patients with systemic lupus erythematosus exhibit acquired hypoprothrombinemia due to the presence of autoantibodies which bind to prothrombin and remove it from the bloodstream (lupus anticoagulant-hypoprothrombinaemia syndrome).^[1]

Treatment

Hypoprothrombinemia can be treated with periodic infusions of purified prothrombin complexes.^[2]

References

^ Bajaj S, Rapaport S, Fierer D, Herbst K, Schwartz D (1983). "A mechanism for the hypoprothrombinemia of the acquired hypoprothrombinemia-lupus anticoagulant syndrome.". Blood 61 (4): 684–92. PMID 6403077.
^ Lechler E (1999). "Use of prothrombin complex concentrates for prophylaxis and treatment of bleeding episodes in patients with hereditary deficiency of prothrombin, factor VII, factor X, protein C protein S, or protein Z.". Thromb Res 95 (4 Suppl 1): S39–50. doi:10.1016/S0049-3848(99)00083-3. PMID 10499908.

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English Journal

A case of congenital prothrombin deficiency and idiopathic thrombocytopenic purpura in a pregnant female.

Harel R1, Shani D, Donohoe K.
Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis.Blood Coagul Fibrinolysis.2016 Feb 15. [Epub ahead of print]
Hereditary prothrombin deficiency is an autosomal recessive disorder with an estimated incidence of 1 in 2 million [1]. Presentation of the disease is variable; however, it is usually associated with moderate to severe bleeding tendencies including muscle hematomas, hemarthrosis, intracranial, mucos
PMID 26886363

A case of lupus anticoagulant-hypoprothrombinemia syndrome associated with systemic lupus erythematosus.

Abdo L1, Mekinian A2, Riviere S2, Fain O2.
Joint, bone, spine : revue du rhumatisme.Joint Bone Spine.2015 Dec 2. pii: S1297-319X(15)00267-5. doi: 10.1016/j.jbspin.2015.06.016. [Epub ahead of print]
PMID 26654379

Bruised Black and Blue: Acquired Hypoprothrombinemia.

Huang LW1, Anand S2, Hassan S2, Onwuemene O2.
The American journal of medicine.Am J Med.2015 Nov;128(11):e7-e11. doi: 10.1016/j.amjmed.2015.06.011. Epub 2015 Jul 6.
PMID 26159638

Japanese Journal

リウマチ性疾患の難治性病態の治療(第2回)抗リン脂質抗体症候群,劇症型APS,lupus anticoagulant-hypoprothrombinemia syndrome

大村一将,奥健志,渥美達也
分子リウマチ治療 8(3), 163-167, 2015-08
NAID 40020545242

橋本病と成人T細胞白血病リンパ腫（くすぶり型）を合併したループスアンチコアグラント低プロトロンビン血症症候群の1例

島袋奈津紀,西由希子,仲地佐和子 [他],玉城啓太,手登根伊織,森近一穂,友寄毅昭,福島卓也,益崎裕章
日本内科学会雑誌 103(8), 1935-1938, 2014
… ループスアンチコアグラント低プロトロンビン血症症候群（lupus anticoagulant-hypoprothrombinemia syndrome：LA-HPS）はLAにプロトロンビン欠乏を合併した疾患であり，血栓症だけでなく，重篤な出血を来たすことがある．症例は74歳女性．大球性貧血の精査目的に入院し，凝固検査にてAPTT延長を認めた．複数の凝固因子活性の低下とLA陽性からLA-HPSと診断した．同時に，橋本病，成人T細胞白血病リンパ腫（adult T-cell leukemia/lymphoma：A …
NAID 130005091968

アデノウイルス胃腸炎とマイコプラズマ肺炎に続発したループスアンチコアグラント陽性・低プロトロンビン血症

清水武,石黒精,高柳隆章,松井猛彦,利根川尚也,前川貴伸,板橋家頭夫
日本臨床免疫学会会誌 37(1), 55-60, 2014
… ト（LA）は小児において感染に伴い一過性に産生され，凝固異常をおこすことがある．特にLA陽性で低プロトロンビン血症を伴うループスアンチコアグラント陽性・低プロトロンビン血症（lupus anticoagulant-hypoprothrombinemia syndrome: LAHPS）では出血症状を示す．マイコプラズマ感染症の肺外病変として抗リン脂質抗体症候群による血栓症や一過性のLA産生を認めることがあるがLAHPSの報告はない．症例は9歳，女児．発熱，咳嗽 …
NAID 130003391984