21. Family History The Three-Generation Pedigree history Disease etiology Gene DVT DVT, pulmonary embolism Hereditary thrombophilia Most common activated protein c resistancefactor V Leiden http://tmsyn.wc.ask.com/r?t=an&s=hb&uid=24312681243126812&sid=343126

22. Factor V Leiden / Thrombophilia Support Page - Living With Thrombophilia activated protein c resistance Protein C S Deficiency Antithrombin Deficiency and other clotting disorders. If you have comments, questions, http://www.fvleiden.org/

Home Email Forum Programs Resources ... Search Factor V Leiden Prothrombin 20210 Mutation Activated Protein C Resistance Antithrombin Deficiency and other clotting disorders If you have comments, questions, or want to post something on this web site, please send email to Deborah Okner Smith Why am I doing this? page updated 8/20/2005 links updated 8/27/2005 Factor V Leiden is the most common hereditary blood coagualtion disorder in the United States. It is present 5% of the in the Caucasian population and 1.2% of the African American population. Factor V Leiden increases the risk of venous thrombosis 3-8 fold for heterozygous (one bad gene inherited) and substantially more, 30-140 fold, for homozygous (two bad gene inherited) individuals. The prothrombin 20210 mutation is the second most common inherited clotting abnormality. It is more common than protein S and C deficiency and Antithrombin deficiency combined; 2% of the general population is heterozygous. It is only a mild risk factor for clots, but together with other risk factors (such as oral contraceptives, surgery, trauma, high blood pressure, obesity, smoking, etc) or combined with other clotting disorders (like Factor V Leiden), the risk of clotting increases dramatically. Factor V Leiden can be associated with the following complications: Venous Thrombosis blood clots in veins, such as:

23. APCR And Factor V Leiden Mutation activated protein c resistance and the Factor V Leiden Mutation CO004550 Abstract Resistance to activated protein C (APC) is a condition which leads http://www.mgh.harvard.edu/labmed/lab/coag/handbook/CO004550.htm

Laboratory Medicine Laboratories Coagulation Handbook APCR and Factor V Leiden Mutation Coag Test Handbook Index From: Elizabeth M. Van Cott, M.D., and Michael Laposata, M.D., Ph.D., "Coagulation." In: Jacobs DS et al, ed. The Laboratory Test Handbook , 5th Edition. Lexi-Comp, Cleveland, 2001; 327-358. Activated Protein C Resistance and the Factor V Leiden Mutation [CO004550] Related Information Activated Partial Thromboplastin Time Antithrombin Hypercoagulation Panel Polymerase Chain Reaction Protein C Protein S Synonyms Activated Protein C Resistance; APC; Protein C Resistance, Activated Applies to Factor V Leiden Mutation Abstract Resistance to activated protein C (APC) is a condition which leads to a hypercoagulable state with an increased risk for venous thrombosis. The effect of exogenous APC on patient's clotting time [usually activated partial thromboplastin time (PTT)] is used to detect presence of resistance to APC (as occurs in individuals with the factor V Leiden mutation). A few laboratories might use clotting times other than the PTT. DNA-based assays can be used to directly detect the presence of the factor V Leiden mutation. Specimen Plasma (for clotting time-based screening assay) and whole blood (for DNA-based confirmatory assay)

24. Activated Protein C Resistance Information Diseases Database activated protein c resistance,APC resistance,Factor V Leiden disease, Disease Database Information. http://www.diseasesdatabase.com/ddb154.htm

Diseases Database Index Sponsors Contact ... Previous Page Activated protein C resistance information Search 3 synonyms or equivalents were found. Activated protein C resistance aka/or APC resistance aka/or Factor V Leiden disease may cause or feature (Follow link for list.) may be a risk factor for (Follow link for list.) may be allelic with (Follow link for list.) belong(s) to the category of (Follow link for list.) Activated protein C resistance: Definition(s) via UMLS Code translations and terms via UMLS Activated protein C resistance: specific web sites Send Activated protein C resistance to medical search engines (JavaScript enabled browsers only.) If your browser has no JavaScript you can still use these: Search using Internet medical databases Search using Internet search engines (non-specialist) We subscribe to the HONcode principles of the Health On the Net Foundation Valid XHTML 1.0 Served 2005-09-08 19:13:43 View metadata Last major update 2005-09-03. The medical information here is presented for education, background reading and general interest. The Diseases Database is not a diagnostic or clinical decision-making tool. Please consult your own licensed physician regarding diagnosis and treatment of any medical condition!

25. Entrez PubMed STUDY DESIGN activated protein c resistance was analyzed in women with previous Results of the activated protein c resistance test were expressed as http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=7

26. Entrez PubMed When factor Va is resistant to degradation by activated protein C, this anticoagulation pathway does not operate properly, and patients have an increased http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=1

27. Log In Problems Venous thromboembolism, a serious health problem and an important cause of morbidity, affects about 1 in 1000 persons annually. http://www.medscape.com/viewarticle/405768

Unable to Deliver Requested Page In order to use Medscape, your browser must be set to accept cookies delivered by the Medscape site. To find out how to adjust your browser settings to accept cookies, please click here Medscape uses cookies to customize the site based on the information we collect at registration. The cookies contain no personally identifiable information and have no effect once you leave the Medscape site. You can read more about our use of cookies in our About Medscape Help WebMD Health

28. Activated Protein C Resistance - New Treatments, January 22, 2005 New treatments for activated protein c resistance, January 22, 2005. http://www.medical-library.org/journals_6a/activated_protein_c.htm

Click here to view next page of this article New Treatments for Activated Protein C Resistance Resistance to activated protein C is a congenital inherited hypercoagulable disease. The problem here is that normally protein C with a co-factor of protein S controls the activity, if you will, down the coagulation pathway starting with number 11, then 12, 9, 8 and so on as the cascade moves down. This system here normally protein C co-factor S inactivates number 5 and number 8 coagulation factor proteins. They are kind of keeping a balance here to prevent ongoing conversion of soluble fibrinogen to insoluble fibrin. We see here that here is factor V and normally this undergoes degradation. But in resistance to activated protein C there is at position 506 in the factor V molecule, arginine moiety is replaced by glutamine, and this is what identifies this. The factor V gene also has an abnormality in it at position 1691. The factor V at 506, the factor V molecule, this arginine is replaced by a glutamine, it’s resistant now to the normal degradation of activated protein C, and the factor V gene here at 1691 a glutamine is replaced by an arginine. This problem is variously reported in different articles and publications to be at a frequency rate in some places of 30, 40, 50, 60% of the populations that are studied. However you and I all know that thrombosis is not in any way shape or form found in that frequency. So one must be somewhat concerned about this and the absolute direct connection that it may have. This may not always really be the answer for this situation, but of the things that you can look for today, it’s certainly going to be high on the list for an etiologic or diagnostic test that can be done. So don’t forget about this population of resistance to activated protein C. The presence of the factor V Leyden molecule, which does not undergo normal degradation as it should, by protein C with a co-factor of protein S.

29. RCPA - The Manual - Activated Protein C (APC) Resistance Test - Plasma activated protein c resistance is associated with an increased risk of venous thromboembolism. However, as the condition is detectable in ~5% of all http://www.rcpamanual.edu.au/sections/pathologytest.asp?s=33&i=226

30. Activated Protein C Resistance Assay Detects Thrombotic Risk Factors Other Than Key Words, activated protein c resistance; Casecontrol study; Factor V Leiden; Activated protein C (APC) resistance is a common risk factor for venous http://www.ajcp.com/previews/abstracts/202180.html

Coagulation and Transfusion Medicine / Original Article Activated Protein C Resistance Assay Detects Thrombotic Risk Factors Other Than Factor V Leiden Laurie L. Graf, CLSp(MB), Carolyn H. Welsh, MD, Zainab Qamar, and Richard A. Marlar, PhD Key Words: Activated protein C resistance; Case-control study; Factor V Leiden; Factor VIII; HR2 haplotype; Venous thromboembolism DOI: 10.1092/LX9E6Y043P58YVCL Abstract Activated protein C (APC) resistance is a common risk factor for venous thromboembolism (VTE) attributed to various mechanisms, including factor V Leiden (FVL) polymorphism. FVL is considered responsible for up to 95% of APC resistance; however other factor V polymorphisms and elevated factor VIII levels also have been implicated. We value your input... If you have something to say regarding this article or ajcp.com in general, share it with us. If you would like to give us feedback on the Web site in general or make corrections to this article, click on Feedback below. To write correspondence to the editor regarding this article, please click on Comment below. Thank you. January 2003 Articles TOC Feedback Comment American Society for Clinical Pathology 2100 West Harrison Street Chicago, Illinois 60612

31. The Effect Of Lupus Anticoagulant In The Second-Generation Assay For Activated P The activated protein c resistance (APCR) assay is the test of choice to screen for factor V Leiden. We evaluated the effect of lupus anticoagulant on the http://www.ajcp.com/previews/abstracts/202186.html

Coagulation and Transfusion Medicine / Original Article The Effect of Lupus Anticoagulant in the Second-Generation Assay for Activated Protein C Resistance Brian D. Ragland, MD, Cari E. Reed, MT(ASCP), Barbara M. Eiland, MT(ASCP), Patricia H. Tichenor, MT(ASCP), Christine L. Hudson, MT(ASCP), George A. Fritsma, MS, MT(ASCP), Brian K. Adler, MD, and Marisa B. Marques, MD Key Words: Lupus anticoagulant; Modified activated protein C resistance (APCR) assay; APCR ratio; Factor V Leiden DOI: 10.1092/909FL47FDQGLE2VD Abstract DOI: 10.1092/909FL47FDQGLE2VD We value your input... If you have something to say regarding this article or ajcp.com in general, share it with us. If you would like to give us feedback on the Web site in general or make corrections to this article, click on Feedback below. To write correspondence to the editor regarding this article, please click on Comment below. Thank you. January 2003 Articles TOC Feedback Comment American Society for Clinical Pathology 2100 West Harrison Street Chicago, Illinois 60612

32. Is Acquired Activated Protein C Resistance A Cardiovascular Risk? • Response -- Sampram ES, Lindblad B, Dahlback B. activated protein c resistance in Rodeghiero F, Tosetto A. activated protein c resistance and factor V Leiden http://circ.ahajournals.org/cgi/content/full/101/12/e120

This Article Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal ... Request Permissions PubMed PubMed Citation Articles by Kario, K. Articles by Willeit, J. Related Collections Coagulation and fibronolysis Pathophysiology Risk Factors Circulation. Circulation Electronic Pages Is Acquired Activated Protein C Resistance a Cardiovascular Risk? Kazuomi Kario, MD, PhD Hypertension Center Department of Internal Medicine, Cornell University Medical College/The New York Presbyterian Hospital, New York, NY Toshiyuki Sakata, PhD Laboratory of Clinical Chemistry National Cardiovascular Center, Osaka, Japan Toshiyuki Miyata, PhD Research Institute National Cardiovascular Center, Osaka, Japan Introduction Top Introduction References To the Editor: We have read the article by Kiechl et al demonstrating that acquired activated protein C resistance (APC) may be a risk factor for atherosclerosis and arterial thrombosis in whites.

33. Poor Response To Activated Protein C As A Prominent Risk Predictor Of Advanced A Background—The potential role of activated protein C (APC) resistance in arterial Is Acquired activated protein c resistance a Cardiovascular Risk? http://circ.ahajournals.org/cgi/content/abstract/99/5/614

This Article Full Text Full Text (PDF) Alert me when this article is cited ... Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal ... Request Permissions PubMed PubMed Citation Articles by Kiechl, S. Articles by Willeit, J. Related Collections Coagulation and fibronolysis Coronary imaging: angiography/ultrasound/Doppler/CC Pathophysiology Circulation. Clinical Investigation and Reports Poor Response to Activated Protein C as a Prominent Risk Predictor of Advanced Atherosclerosis and Arterial Disease Stefan Kiechl, MD Armin Muigg, MD Peter Santer, MD Manfred Mitterer, MD Georg Egger, MD Martin Oberhollenzer, MD Friedrich Oberhollenzer, MD Agnes Mayr, MD Arno Gasperi, MD Werner Poewe, MD Johann Willeit, MD From the Department of Neurology, Innsbruck University Clinic, Innsbruck, Austria (S.K., A. Muigg, W.P., J.W.); the Departments of Laboratory Medicine (P.S., A. Mair), Internal Medicine (G.E., M.O., F.O.), and Neurology (A.G.), Bruneck Hospital, Bruneck, Italy; and the Department of Hematology, Bozen Hospital, Bozen, Italy (M.M.). Background resistance in arterial thrombosis and disease is a matter of ongoing controversy.

34. Activated Protein C Resistance And Mutations In Factor V activated protein c resistance (APC RESISTANCE) AND MUTATIONS IN FACTOR V. MOLECULAR BASIS When thrombin (IIa) is generated, it has both procoagulant http://www.hemex.com/testmenus/apc_resistance.htm

Home About HEMEX Meet Us At Publications ... Contacting HEMEX var site="s17hemex" ACTIVATED PROTEIN C RESISTANCE (APC RESISTANCE) AND MUTATIONS IN FACTOR V MOLECULAR BASIS: When thrombin (IIa) is generated, it has both procoagulant activities and anticoagulant activities. Excess thrombin is washed downstream where it binds to thrombomodulin (TM) on endothelial cells of the vessel wall. Protein C from plasma binds to the IIa/TM complex and is cleaved to its active form, termed "activated Protein C" (APC). APC is one of the most physiologically important anticoagulants as it selectively degrades the coagulation cofactors, Va and VIIIa to limit thrombin generation, fibrin formation and blood clotting in vivo. It has been shown that APC resistance results from a mutant Factor Va molecule, termed Factor V Leiden. This molecule has a specific point mutation (Arg -> Gln ) which cannot be degraded by APC in more than 90% of all APC resistant patients2. The defective Va is able to clot as normal and clotting continues because of this resistance to inactivation by APC. Other APC cleavage sites in the Factor Va molecule are potential mutation sites. These sites include: Arg -> Gln and Arg -> Gln . The other 10% of APC resistance includes Acquired APC Resistance and these secondary sites. Recent studies have shown the occurrence of APC resistance to vary from 2 - 16% depending on the population studied. This data suggests that screening protocols for hereditary thrombotic disorders should include testing for APC resistance as an important genetic risk factor. Hypercoagulability has been explained by hereditary deficiencies of Protein C, Protein S and Antithrombin in only 9 to 21% of thrombotic cases in patients without the usual risk factors for thrombosis (i.e., cancer, recent surgery, lupus anticoagulant)

35. Thrombophilia Screen Testing includes antithrombin , protein C, protein S, activated protein c resistance, factor V leiden, factor II variant lupus type inhibitor. http://www.calgarylabservices.com/LabTests/AlphabeticalListing/T/Thrombophilia-S

Home Contact Us Useful Links Site Map ... Patients List All Test Names Beginning With: A B C D ... Cytopathology Thrombophilia Screen Source Blood Mnemonic THROMB and DNATR Specimen Requirements Adult : 4 x 5 mL blue top sodium citrate tubes (4.5 mL draw) AND 1 x 5 mL lavender top EDTA tube Pediatric : 3 x short-draw (2.7 mL draw) blue top sodium citrate tubes AND 1 x short-draw lavender top EDTA tube. Specimen Handling PSC and RRL : Send specimens UNSPUN at room temperature directly to DSC Special Coagulation. Must arrive within 2 hours of collection. Extra-regional : Lavender tube - ship unspun at room temperature. Blue top tubes – centrifuge at 3500 rpm for 20 minutes, aliquot plasma into 8-10 plastic tubes. Freeze and transport frozen to DSC Special Coagulation. Note: Weekend collections are not recommended. Additional Information Testing includes antithrombin , protein C, protein S, activated protein C resistance, factor V leiden, factor II variant lupus type inhibitor. Specimens MUST be accompanied with a copy of the original requistion and a Thrombosis history form. Phone Special Coagulation 770-3599/770-3598 with any questions. Testing Location Special Coagulation  Testing Frequency Alternate Name(s) Hypercoaguable screen/workup, Thrombosis study/workup

36. EMedicine - Protein C Deficiency Article By Eleanor S Pollak, MD activated protein c resistance Factor V Leiden Prothrombin 20210 Gto-A mutation Cancer-related thrombosis Homocystinuria http://www.emedicine.com/med/topic1923.htm

37. Factor V Leiden effects of activated protein C, thus the term activated protein c resistance. The result is that factor V Leiden is inactivated by activated protein C http://www-admin.med.uiuc.edu/hematology/PtFacV2.htm

University of Illinois - Urbana/Champaign Carle Cancer Center Hematology Resource Page Patient Resources Factor V Leiden Home Factor V Leiden Antiphospholipid Syndrome General Clotting Information ... Protein S deficiency Factor V Leiden is a genetically acquired trait that can result in a thrombophilic (hypercoaguable) state resulting in the phenomenon of activated protein C resistance (APCR) as described below. Associated with factor V Leiden, APCR was first described in 1993; factor V Leiden was subsequently discovered in 1994. Over 95% of patients with APCR have factor V Leiden. Factor V Leiden's overall impact on the coagulation cascade described below. Mechanism of Action of Factor V Leiden: Factor V Leiden is characterized by a phenomenon called APCR where a genetic mutation in the factor V gene causes a change in the factor V protein making it resistant to inactivation by protein C. Epidemiology of Factor V Leiden: Factor V Leiden is seen more commonly in the northern European populations. About 4-7% of the general population is heterozygous (see * below) for factor V Leiden. About 0.06 to 0.25% of the population is homozygous (see * below) for factor V Leiden. The factor V Leiden mutation is relatively uncommon in the native populations of Asia, Africa and North America. In contrast, in Greece and southern Sweden, rates above 10% have been reported.

38. Arch Dermatol -- Mali Acroangiodermatitis In Homozygous Activated Protein C Resi Mali Acroangiodermatitis in Homozygous activated protein c resistance led to the rare diagnosis of homozygous activated protein c resistance (APCR). http://archderm.ama-assn.org/cgi/content/extract/141/3/396

Select Journal or Resource JAMA Archives of Dermatology Facial Plastic Surgery Family Medicine (1992-2000) General Psychiatry Internal Medicine Neurology Ophthalmology Surgery Student JAMA (1998-2004) JAMA CareerNet For The Media Meetings Peer Review Congress Vol. 141 No. 3, March 2005 Featured Link E-mail Alerts Correspondence Article Options Full text PDF Send to a Friend Readers Reply Submit a reply Similar articles in this journal Literature Track Add to File Drawer Download to Citation Manager PubMed citation Articles in PubMed by Scholz S Kerl H Contact me when this article is cited Topic Collections Dermatologic Disorders, Other Topic Collection Alerts VIGNETTES Mali Acroangiodermatitis in Homozygous Activated Protein C Resistance Since this article does not have an abstract, we have provided the first 150 words of the full text and any section headings. We describe 2 patients whose dermatologic signs of Mali acroangiodermatitis led to the rare diagnosis of homozygous activated protein C resistance (APCR). Since its discovery in 1993, APCR has been found to be the most frequent hereditary coagulation defect.

39. ACTIVATED PROTEIN C RESISTANCE activated protein c resistance (0890240). Synonyms APCR Applies To A screening assay for the detection of activated protein c resistance (APCR) in http://www.utmb.edu/lsg/LabSurvivalGuide/hem/ACTIVATEDproteinC_Resistance.htm

LSG Home Page A lphabetical Index A B C D ... LSG PDF Version LINKS POCT Web Accreditation Certificates Pathology Clinical Services Department of Pathology ACTIVATED PROTEIN C RESISTANCE (089-0240) Synonyms: APCR CPT 4 Code Test Order Mnemonic: APCR Applies To: A screening assay for the detection of activated protein C resistance (APCR) in patients with a history of recurrent venous thrombosis. Most patients with a positive APCR screening assay have a specific mutation in the coagulant factor V gene (Factor V Leiden mutation). Test Includes: APCV Ratio; Interpretation of result. Lab: Hematopathology Request Form: Hematology A Collection: Patient should be at rest for 10-20 minutes prior to collection. Standard venipuncture collection for Coagulation specimens. Discard 1st ml of blood or collect other tubes (EDTA, Serum-separator, etc.) prior to collecting sample in 3.2% citrate (light blue-top) tube. Storage Instructions: Viable for 4 hours at room temperature. If time from draw to delivery is to be greater than 4 hours, centrifuge the sample, separate the plasma from cells, and snap freeze (-70°C) the plasma. Snap frozen plasma is viable for 6 months. Special Instructions: Prior to ordering this test, verify that the APTT is in the normal range

40. ACTIVATED PROTEIN C RESISTANCE activated protein c resistance. TEST CODEAPRTC CPT CODE85335. SYNONYMS TEST INCLUDES LABORATORY HematologyCoagulation http://www.healthsystem.virginia.edu/internet/labtests/clinical/a/aprtc.cfm

[ Skip Navigation ] Health System Home For Health Answers Healthcare Professionals ... Advanced Search Health System Search Search for Information People UVa Home Health System News Health System Events (all) Health System Publications ... Site Index Site Search Search this site: ACTIVATED PROTEIN C RESISTANCE TEST CODE: APRTC CPT CODE: SYNONYMS: TEST INCLUDES: LABORATORY: Hematology-Coagulation SPECIMEN: Blue Top, 4.5 mL citrated blood MINIMUM VOLUME: Small Blue Top, 2.7 mL citrated blood AVAILABILITY: By consultation only TURNAROUND TIME: Final report pending Pathologist review. Contact laboratory at 924-8007 for STAT testing and STAT results. SPECIAL INSTRUCTIONS: REFERENCE RANGE: Print this Page Email this Page UVa Medical Laboratories Handbook PO Box 800168 Charlottesville , VA 22908 fax: 434-982-1880 For comments and suggestions about this page: Webmaster Last Modified: April 23 2003 of the University of Virginia About This Site

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