関: lupus anticoagulant

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limit, block, or decrease the action or function of; "inhibit the action of the enzyme"; "inhibit the rate of a chemical reaction"
control and refrain from showing; of emotions, desires, impulses, or behavior (同)bottle up, suppress
limit the range or extent of; "Contact between the young was inhibited by strict social customs"
a substance that retards or stops an activity
any of several forms of ulcerative skin disease
a constellation in the southern hemisphere near Centaurus

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〈感情・欲望・行動・作用など〉‘を'抑制する / (…しないように)〈人〉‘を'抑制する,妨げる《+『名』+『from』+『名』(do『ing』)》
抑制する人(物) / 化学反応抑制剤
狼瘡(ろうそう)(皮膚結核の一種)
凝固,凝結

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2016/06/09 19:54:00」(JST)

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Lupus anticoagulant (also known as lupus antibody, LA, LAC, or lupus inhibitors) is an immunoglobulin^[1] that binds to phospholipids and proteins associated with the cell membrane. Lupus anticoagulant is a misnomer, as it is actually a prothrombotic agent. Lupus anticoagulant antibodies in living systems cause an increase in inappropriate blood clotting. The name derives from their properties in vitro, since in laboratory tests, these antibodies increase aPTT. Investigators speculate that the antibodies interfere with phospholipids used to induce in vitro coagulation. In vivo, the antibodies are thought to interact with platelet membrane phospholipids, increasing adhesion and aggregation of platelets, which accounts for the in vivo prothrombotic characteristics.

The condition was first described by hematologist C. Lockard Conley.^[2]^[3]

Terminology

Both words in the term "lupus anticoagulant" can be misleading:

Most patients with a lupus anticoagulant do not actually have lupus erythematosus, and only a small proportion will proceed to develop this disease (which causes joint pains, skin problems and renal failure, amongst other complications). Patients with lupus erythematosus are more likely to develop a lupus anticoagulant than the general population.
The term "anticoagulant" accurately describes its function in vitro. However in vivo, it functions as a pro-coagulant.^[4]

Workup

The presence of prolonged clotting times on a routine plasma test often triggers functional testing of the blood clotting function, as well as serological testing to identify common autoantibodies such as antiphospholipid antibodies. These antibodies tend to delay in-vitro coagulation in phospholipid-dependent laboratory tests such as the partial thromboplastin time.

The initial workup of a prolonged PTT is a mixing test whereby the patient's plasma is mixed with normal pooled plasma and the clotting is re-assessed. If a clotting inhibitor such as a lupus anticoagulant is present, the inhibitor will interact with the normal pooled plasma and the clotting time will remain abnormal. However, if the clotting time of the mixed plasma corrects towards normal, the diagnosis of an inhibitor such as the lupus anticoagulant is excluded; the diagnosis is a deficient clotting factor that is replenished by the normal plasma.

If the mixing test indicates an inhibitor, diagnosis of a lupus anticoagulant is then confirmed with phospholipid-sensitive functional clotting testing, such as the dilute Russell's viper venom time, or the Kaolin clotting time. Excess phospholipid will eventually correct the prolongation of these prolonged clotting tests (conceptually known as "phospholipid neutralization" in the clinical coagulation laboratory), confirming the diagnosis of a lupus anticoagulant.

Treatment

Treatment for a lupus anticoagulant is usually undertaken in the context of documented thrombosis, such as extremity phlebitis or dural sinus vein thrombosis. Patients with a well-documented (i.e., present at least twice) lupus anticoagulant and a history of thrombosis should be considered candidates for indefinite treatment with anticoagulants. Patients with no history of thrombosis and a lupus anticoagulant should probably be observed. Current evidence suggests that the risk of recurrent thrombosis in patients with an antiphospholipid antibody is enhanced whether that antibody is measured on serological testing or functional testing. The Sapporo criteria specify that both serological and functional tests must be positive to diagnose the antiphospholipid antibody syndrome.^[5]

Miscarriages may be more prevalent in patients with a lupus anticoagulant. Some of these miscarriages may potentially be prevented with the administration of aspirin and unfractionated heparin. The Cochrane Database of Systematic Reviews provide a deeper understanding on the subject.^[6]

Thrombosis is treated with anticoagulants (LMWHs and warfarin).^[7]

References

^ Antonia Joussen; T.W. Gardner; B. Kirchhof (23 October 2007). Retinal Vascular Disease. Springer. pp. 430–. ISBN 978-3-540-29541-9. Retrieved 29 June 2010.
^ Conley, C. Lockard (1952). "A hemorrhagic disorder caused by circulating anticoagulant in patients with disseminated lupus erythematosus". Journal of Clinical Investigation 31: 621–622. doi:10.1172/JCI102648.
^ "Lock Conley looks back and blushes". Hopkins Medicine. Spring–Summer 2006. Retrieved 5 December 2013.
^ "wustl.edu". Retrieved 2009-02-17.
^ Viard JP, Amoura Z, Bach JF (1991). "[Anti-beta 2 glycoprotein I antibodies in systemic lupus erythematosus: a marker of thrombosis associated with a circulating anticoagulant]". C. R. Acad. Sci. III, Sci. Vie (in French) 313 (13): 607–12. PMID 1782567.
^ Empson, M; Lassere, M; Craig, J; Scott, J (Apr 18, 2005). "Prevention of recurrent miscarriage for women with antiphospholipid antibody or lupus anticoagulant." (PDF). The Cochrane database of systematic reviews (2): CD002859. doi:10.1002/14651858.CD002859.pub2. PMID 15846641.
^ Dolitzky M, Inbal A, Segal Y, Weiss A, Brenner B, Carp H (2006). "A randomized study of thromboprophylaxis in women with unexplained consecutive recurrent miscarriages". Fertil Steril 86 (2): 362–6. doi:10.1016/j.fertnstert.2005.12.068. PMID 16769056.

UpToDate Contents

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1. 凝固検査の臨床使用 clinical use of coagulation tests
2. 後天性の凝固因子阻害物質 acquired inhibitors of coagulation
3. 術前の止血状態評価 preoperative assessment of hemostasis
4. Hematologic manifestations of systemic lupus erythematosus
5. 出血症状を有する小児に対するアプローチ approach to the child with bleeding symptoms

English Journal

Transient presence of lupus anticoagulant associated with mycoplasma pneumonia.

Wang Chun K1, See Wan Y2, Poon Chuen W2.
Asian cardiovascular & thoracic annals.Asian Cardiovasc Thorac Ann.2016 Mar;24(3):286-7. doi: 10.1177/0218492315625158. Epub 2016 Jan 7.
We report a case of asymptomatic transient presence of lupus anticoagulant with isolated prolonged activated partial thromboplastin time in a 30-year-old lady with pneumonia caused by Mycoplasma pneumoniae. After treatment of pneumonia, lupus anticoagulant was not detected and the activated partial
PMID 26744491

Dabigatran deliberate overdose: two cases and suggestions for laboratory monitoring.

Vlad I1, Armstrong J1, Ridgley J2, Pascu O1.
Clinical toxicology (Philadelphia, Pa.).Clin Toxicol (Phila).2016 Mar;54(3):286-9. doi: 10.3109/15563650.2015.1126287. Epub 2016 Jan 6.
CONTEXT: Dabigatran etexilate (dabigatran) is a direct thrombin inhibitor anticoagulant agent. There is limited information about the changes in coagulation profile and outcomes in overdose. A monoclonal antibody has been developed to neutralize the anticoagulant effect of dabigatran. Case reports d
PMID 26735702

The lectin complement pathway serine proteases (MASPs) represent a possible crossroad between the coagulation and complement systems in thromboinflammation.

Kozarcanin H1, Lood C2, Munthe-Fog L3, Sandholm K4, Hamad OA1, Bengtsson AA2, Skjoedt MO3, Huber-Lang M5, Garred P3, Ekdahl KN1,4, Nilsson B1.
Journal of thrombosis and haemostasis : JTH.J Thromb Haemost.2016 Mar;14(3):531-45. doi: 10.1111/jth.13208. Epub 2016 Feb 15.
Essentials The lectin pathway's MASP-1/2 activates coagulation factors but the trigger of the activation is unknown. MASP-1/2 activation was assessed by quantifying complexes between MASPs and antithrombin/C1-inhibitor. Activated platelets and fibrin were demonstrated to activate MASP-1 and MASP-2 b
PMID 26614707