Von Willebrand Disease: Diagnosis, Types, and Treatment | Clinique Omicron<\/title>\n<meta name=\"description\" content=\"La maladie de von Willebrand est la coagulopathie h\u00e9r\u00e9ditaire la plus fr\u00e9quente. 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Medicine<\/span>\n <h1>Von Willebrand disease<\/h1>\n<style>.oc-fiche-cta{margin:8px 0 34px;padding:22px 24px;border:1.5px solid rgba(50,60,82,.15);border-left:4px solid #FF611C;border-radius:12px;background:#F4F6F8;font-family:Poppins,sans-serif}.oc-fiche-cta-t{font-size:18px;font-weight:600;color:#323C52;margin:0 0 4px;line-height:1.35}.oc-fiche-cta-x{font-size:15px;color:#4D6577;margin:0 0 14px;line-height:1.55}.oc-fiche-cta-b{display:flex;flex-wrap:wrap;gap:10px}.oc-fiche-cta-b a{display:inline-flex;align-items:center;justify-content:center;min-height:44px;padding:10px 18px;border-radius:8px;font-size:14px;font-weight:600;text-decoration:none;box-sizing:border-box}.oc-fiche-cta-b .oc-p{background:#FF611C;color:#fff}.oc-fiche-cta-b .oc-p:hover{background:#E04E0C}.oc-fiche-cta-b .oc-s{background:#fff;color:#323C52;border:1.5px solid rgba(50,60,82,.25)}@media (max-width:600px){.co-wrap h1{font-size:24px;line-height:1.25;text-transform:none}.oc-fiche-cta{padding:18px 16px}.oc-fiche-cta-b a{flex:1 1 100%}body .co-wrap > .co-table,body .co-wrap > .co-table tbody,body .co-wrap > .co-table tr,body .co-wrap > .co-table td{display:block;width:100%;box-sizing:border-box}body .co-wrap > .co-table{min-width:0;table-layout:auto}body .co-wrap > .co-table thead{display:none}body .co-wrap > .co-table tr{margin:0 0 10px;border:1px solid rgba(77,101,119,.18);border-radius:8px;overflow:hidden}body .co-wrap > .co-table td{padding:9px 12px;border:0}body .co-wrap > .co-table td:first-child{font-weight:600;color:#323C52;background:rgba(77,101,119,.07)}#ocw{transform:scale(.8);transform-origin:bottom right}}<\/style><div class=\"oc-fiche-cta\" data-oc-cta=\"fiche-haut-consult\"><p class=\"oc-fiche-cta-t\">Obtenir un avis adapt\u00e9 \u00e0 votre situation<\/p><p class=\"oc-fiche-cta-x\">Consultation en clinique ou en t\u00e9l\u00e9consultation, partout au Qu\u00e9bec.<\/p><div class=\"oc-fiche-cta-b\"><a class=\"oc-p\" href=\"https:\/\/cliniqueomicron.ca\/rendez-vous\/\">Make an appointment<\/a><a class=\"oc-s\" href=\"https:\/\/cliniqueomicron.ca\/consultation-en-ligne\/\">Consult online<\/a><a class=\"oc-s\" href=\"tel:+15146063350\">514 606-3350<\/a><\/div><\/div>\n\n\n <div class=\"co-intro\">\n La maladie de von Willebrand (MvW) est la coagulopathie h\u00e9r\u00e9ditaire la plus fr\u00e9quente \u2014 touchant environ 1 % de la population mondiale sous sa forme l\u00e9g\u00e8re + bien que la pr\u00e9valence des formes cliniquement significatives n\u00e9cessitant une prise en charge soit estim\u00e9e \u00e0 1 pour 1 000 \u00e0 1 pour 10 000 selon les crit\u00e8res diagnostiques utilis\u00e9s. Elle est caus\u00e9e par un d\u00e9ficit quantitatif ou qualitatif du facteur von Willebrand (VWF) \u2014 une grande glycoprot\u00e9ine multim\u00e9rique synth\u00e9tis\u00e9e par les cellules endoth\u00e9liales et les m\u00e9gacaryocytes + qui joue un double r\u00f4le essentiel dans l'h\u00e9mostase primaire : d'une part + comme prot\u00e9ine d'adh\u00e9sion plaquettaire au sous-endoth\u00e9lium vasculaire l\u00e9s\u00e9 (via la liaison au collag\u00e8ne et au r\u00e9cepteur plaquettaire GPIb) + permettant la formation du clou plaquettaire \u00e0 haute contrainte de cisaillement (art\u00e8res + art\u00e9rioles) + et d'autre part + comme prot\u00e9ine chaperon du facteur VIII de la coagulation (FVIII) + dont il prot\u00e8ge la d\u00e9gradation pr\u00e9matur\u00e9e dans le plasma + expliquant pourquoi un d\u00e9ficit en VWF entra\u00eene secondairement une r\u00e9duction du FVIII (d'o\u00f9 l'allongement du TCA dans certains types de MvW). La MvW a \u00e9t\u00e9 d\u00e9crite pour la premi\u00e8re fois en 1926 par le m\u00e9decin finlandais Erik Adolf von Willebrand + qui d\u00e9crivit une diath\u00e8se h\u00e9morragique familiale touchant une famille d'\u00eeles \u00c5land + avec un tableau de saignements muqueux r\u00e9cidivants (\u00e9pistaxis + m\u00e9norragies + saignements gingivaux + ecchymoses faciles) \u2014 distinct de l'h\u00e9mophilie classique (saignements musculaires et articulaires) par son si\u00e8ge muqueux et sa transmission autosomique (dominant pour les types 1 et 2 + r\u00e9cessif pour le type 3 s\u00e9v\u00e8re). La grande variabilit\u00e9 ph\u00e9notypique + le chevauchement avec les valeurs normales basses dans le type 1 + et l'influence des groupes sanguins ABO sur les taux de VWF (groupe O \u2192 VWF naturellement 20\u201325 % plus bas que les autres groupes) rendent le diagnostic parfois difficile et n\u00e9cessitent souvent plusieurs dosages r\u00e9p\u00e9t\u00e9s et des tests fonctionnels sp\u00e9cialis\u00e9s.\n <\/div>\n\n <h2>Classification of von Willebrand disease types<\/h2>\n\n <table class=\"co-table\">\n <thead>\n <tr>\n <th>Type<\/th>\n <th>D\u00e9ficit<\/th>\n <th>Frequency<\/th>\n <th>VWF:Ag<\/th>\n <th>VWF:RCo<\/th>\n <th>FVIII<\/th>\n <th>Transmission<\/th>\n <\/tr>\n <\/thead>\n <tbody>\n <tr>\n <td>Type 1 (le plus fr\u00e9quent)<\/td>\n <td>Quantitatif partiel \u2014 r\u00e9duction proportionnelle de toutes les formes multim\u00e9rique du VWF<\/td>\n <td>70\u201380 % des MvW<\/td>\n <td>Bas (20\u201350 UI\/dL)<\/td>\n <td>Proportionately<\/td>\n <td>L\u00e9g\u00e8rement bas ou normal<\/td>\n <td>Autosomique dominant \u00e0 p\u00e9n\u00e9trance variable<\/td>\n <\/tr>\n <tr>\n <td>Type 2A<\/td>\n <td>Qualitatif \u2014 perte des grands multim\u00e8res (les plus actifs) \u2192 d\u00e9faut d'adh\u00e9sion plaquettaire<\/td>\n <td>10\u201315 % des MvW<\/td>\n <td>Normal ou l\u00e9g\u00e8rement bas<\/td>\n <td>Tr\u00e8s bas (ratio RCo\/Ag <0,6)<\/td>\n <td>Normal<\/td>\n <td>Autosomal dominant<\/td>\n <\/tr>\n <tr>\n <td>Type 2B<\/td>\n <td>Qualitatif \u2014 gain de fonction \u2192 VWF anormal \u00e0 affinit\u00e9 augment\u00e9e pour GPIb \u2192 agr\u00e9gation spontan\u00e9e des plaquettes \u2192 consommation des grands multim\u00e8res + thrombop\u00e9nie<\/td>\n <td>5 % of the MvW<\/td>\n <td>Normal or low<\/td>\n <td>Tr\u00e8s bas<\/td>\n <td>Normal<\/td>\n <td>Autosomique dominant + DDAVP contre-indiqu\u00e9 (aggrave la thrombop\u00e9nie)<\/td>\n <\/tr>\n <tr>\n <td>Type 2 meters<\/td>\n <td>Qualitatif \u2014 d\u00e9faut de liaison au collag\u00e8ne ou \u00e0 GPIb sans perte des grands multim\u00e8res<\/td>\n <td>Rare<\/td>\n <td>Normal<\/td>\n <td>Tr\u00e8s bas<\/td>\n <td>Normal<\/td>\n <td>Autosomal dominant<\/td>\n <\/tr>\n <tr>\n <td>Type 2N (Normandy)<\/td>\n <td>Qualitatif \u2014 d\u00e9faut de liaison au FVIII \u2192 d\u00e9gradation rapide du FVIII \u2192 tableau ressemblant \u00e0 l'h\u00e9mophilie A l\u00e9g\u00e8re<\/td>\n <td>Rare<\/td>\n <td>Normal<\/td>\n <td>Normal<\/td>\n <td>Tr\u00e8s bas (5\u201340 %)<\/td>\n <td>Autosomique r\u00e9cessif \u2014 peut mimer une h\u00e9mophilie A \u2192 test de liaison VWF-FVIII<\/td>\n <\/tr>\n <tr>\n <td>Type 3 (le plus s\u00e9v\u00e8re)<\/td>\n <td>Quantitatif complet \u2014 absence quasi-totale de VWF<\/td>\n <td>1\u20135 % des MvW<\/td>\n <td>Ind\u00e9tectable ou <3 UI\/dL<\/td>\n <td>Ind\u00e9tectable<\/td>\n <td>Tr\u00e8s bas (1\u201310 %)<\/td>\n <td>Autosomique r\u00e9cessif \u2014 saignements s\u00e9v\u00e8res muqueux + articulaires + musculaires<\/td>\n <\/tr>\n <\/tbody>\n <\/table>\n\n <h2>Clinical presentation<\/h2>\n <ul class=\"co-list\">\n <li><strong>Saignements muqueux (caract\u00e9ristiques de la MvW) :<\/strong> recurrent epistaxis + menorrhagia (often inaugural in women - very heavy first menstruation) + gingival bleeding + easy bruising + prolonged post-dental extraction bleeding + digestive bleeding (telangiectasias + intestinal angiodysplasias in severe forms)<\/li>\n <li><strong>M\u00e9norragies :<\/strong> touchent 32\u2013100 % des femmes avec MvW + souvent m\u00e9connu et attribu\u00e9 \u00e0 tort \u00e0 un cycle irr\u00e9gulier + score PBAC (Pictorial Blood Assessment Chart) >100 = m\u00e9norragies significatives<\/li>\n <li><strong>Saignements post-op\u00e9ratoires :<\/strong> After surgery + childbirth + dental extractions \u2192 mandatory hematologic planning before any invasive procedure<\/li>\n <li><strong>Type 3 s\u00e9v\u00e8re :<\/strong> can resemble hemophilia with muscle and joint bleeding (hemarthrosis) plus mucosal bleeding<\/li>\n <li><strong>Score h\u00e9morragique (BAT \u2014 Bleeding Assessment Tool ISTH) :<\/strong> standardized tool to quantify bleeding severity + score \u2265 3 (male) or \u2265 5 (female) = significant hemorrhagic diathesis \u2192 mandatory investigation<\/li>\n <\/ul>\n\n <h2>Diagnostic biological assessment<\/h2>\n <ul class=\"co-list\">\n <li><strong>Bilan de premi\u00e8re intention :<\/strong> NFS + platelets (thrombocytopenia in type 2B) + PT + aPTT (prolonged if FVIII is low - type 2N + type 3) + bleeding time or PFA-100 (prolonged occlusion time)<\/li>\n <li><strong>Bilan sp\u00e9cialis\u00e9 (h\u00e9matologie) :<\/strong> VWF:Ag (VWF antigen \u2014 quantitative) + VWF:RCo (ristocetin cofactor activity \u2014 functional) + FVIII:C (coagulant) + multimeric analysis by electrophoresis + VWF-FVIII binding test (type 2N) + RIPA (ristocetin-induced platelet aggregation \u2014 type 2B)<\/li>\n <li><strong>VWF:RCo \/ VWF:Ag ratio<\/strong> ratio <0,6 = d\u00e9faut qualitatif (type 2) \u2192 oriente vers les types 2A + 2B + 2M<\/li>\n <li><strong>R\u00e9p\u00e9ter les dosages :<\/strong> VWF is an acute phase protein that increases during inflammation, stress, pregnancy, exercise, and estrogen therapy. Doses can be falsely normal in these contexts. Repeat at a distance from confounding factors.<\/li>\n <li><strong>ABO Blood Group:<\/strong> Group O is associated with lower VWF 20\u201325 % levels than the other groups \u2192 take this into account when interpreting the reference ranges<\/li>\n <\/ul>\n\n <h2>Treatment<\/h2>\n <ul class=\"co-list\">\n <li><strong>Desmopressine (DDAVP \u2014 Minirin\u00ae ou Octostim\u00ae) \u2014 traitement de choix du type 1 :<\/strong> Synthetic vasopressin analogue \u2192 release of endothelial von Willebrand factor (vWF) stores (Weibel-Palade bodies) \u2192 transient increase in vWF and Factor VIII (FVIII) by 3 to 5 times for 4\u20136 hours \u2192 IV + SC + or intranasal administration (Octostim\u00ae nasal spray) + mandatory prior therapeutic test to verify individual response + tachyphylaxis after 2\u20133 consecutive doses (depletion of stores) + contraindicated in type 2B (worsens thrombocytopenia) + adverse effects: hyponatremia (mandatory fluid restriction \u00d7 24 h) + flush + headaches<\/li>\n <li><strong>Concentr\u00e9s de facteur VWF (Haemate P\u00ae + Voncento\u00ae + Wilate\u00ae) :<\/strong> indicated in type 2 + type 3 + DDAVP non-responsive type 1 + and during major surgeries + childbirths + or severe bleeding \u2192 provide vWF and FVIII \u2192 management by a specialized hemophilia center<\/li>\n <li><strong>Acide tranexamique (Cyklokapron\u00ae) :<\/strong> antifibrinolytic + inhibits plasmin \u2192 reduces mucosal bleeding (nosebleeds + menorrhagia + mouth bleeds) \u2192 1 g \u00d7 3\u20134\/d PO + or IV \u2192 often used alone or in combination with DDAVP + particularly effective for mucous membranes<\/li>\n <li><strong>Hormone Therapy (Women):<\/strong> combined oral contraceptives \u2192 raise vWF + reduce menorrhagia + first-line gynecological treatment for menorrhagia related to vWD + levonorgestrel IUD (Mirena\u00ae) \u2192 effectively reduces menstrual flow<\/li>\n <li><strong>Before any invasive procedure (surgery + childbirth + tooth extraction):<\/strong> Mandatory preoperative hematological consultation + individualized hemostatic management plan according to the type and level of vWF and the type of procedure<\/li>\n <\/ul>\n\n <div class=\"co-infobox\">\n <span class=\"ico\">\u2119\ufe0f<\/span>\n <span>La maladie de von Willebrand de type 1 est souvent sous-diagnostiqu\u00e9e chez les femmes \u2014 les m\u00e9norragies depuis la m\u00e9narche \u00e9tant attribu\u00e9es \u00e0 tort \u00e0 un \u00abcycle normal abondant\u00bb pendant des ann\u00e9es. Devant toute m\u00e9norragie inaugurale s\u00e9v\u00e8re (premi\u00e8re r\u00e8gles tr\u00e8s abondantes + an\u00e9mie + n\u00e9cessit\u00e9 de transfusion) + tout saignement post-partum important + ou tout ant\u00e9c\u00e9dent familial de diath\u00e8se h\u00e9morragique + un bilan de coagulopathie incluant les dosages de VWF doit \u00eatre r\u00e9alis\u00e9. La MvW est la cause la plus fr\u00e9quente de coagulopathie chez la femme pr\u00e9sentant des m\u00e9norragies inexpliqu\u00e9es.<\/span>\n <\/div>\n\n <div class=\"co-urgence\">\n <div class=\"co-urgence-titre\">Medical consultation recommended<\/div>\n <p>Consulter un m\u00e9decin ou un h\u00e9matologue si des \u00e9pistaxis r\u00e9cidivantes + des m\u00e9norragies importantes depuis la m\u00e9narche + des saignements prolong\u00e9s apr\u00e8s une extraction dentaire ou une chirurgie mineure + ou des ecchymoses multiples sans traumatisme sont pr\u00e9sents \u2014 ces signes peuvent \u00e9voquer une maladie de von Willebrand n\u00e9cessitant un bilan de coagulation sp\u00e9cialis\u00e9. Tout patient connu avec une MvW doit consulter un h\u00e9matologue avant tout geste invasif. Pour le bilan de coagulation et l'orientation vers l'h\u00e9matologie, Clinique Omicron offre des consultations dans ses points de service au Qu\u00e9bec et en t\u00e9l\u00e9m\u00e9decine. Pour prendre rendez-vous, visitez <a href=\"https:\/\/cliniqueomicron.ca\" style=\"color:#c0392b;font-weight:600;text-decoration:none;\">cliniqueomicron.ca<\/a>.<\/p>\n <\/div>\n\n <h2>Consult at Clinique Omicron<\/h2>\n <p>Les m\u00e9decins et infirmiers praticiens sp\u00e9cialis\u00e9s (IPS) de Clinique Omicron \u00e9voquent le diagnostic de maladie de von Willebrand devant tout tableau de saignements muqueux r\u00e9cidivants + m\u00e9norragies depuis la m\u00e9narche + ou score h\u00e9morragique BAT \u00e9lev\u00e9, prescrivent le bilan de coagulation initial (NFS + TP + TCA + PFA-100) et les dosages sp\u00e9cialis\u00e9s VWF:Ag + VWF:RCo + FVIII, orientent vers un centre d'h\u00e9mophilie pour la confirmation diagnostique et le plan de traitement, prescrivent l'acide tranexamique pour les saignements muqueux, et coordonnent la prise en charge pr\u00e9op\u00e9ratoire. Des consultations sont disponibles dans plusieurs points de service au Qu\u00e9bec et en t\u00e9l\u00e9m\u00e9decine. Pour prendre rendez-vous, visitez <a href=\"https:\/\/cliniqueomicron.ca\" style=\"color:#4D6577;font-weight:600;text-decoration:none;\">cliniqueomicron.ca<\/a>.<\/p>\n\n <p class=\"co-disclaimer\">Le contenu de cette page est fourni \u00e0 titre informatif uniquement et ne remplace pas l'avis d'un m\u00e9decin ou d'un h\u00e9matologue sp\u00e9cialis\u00e9 en coagulopathies. La DDAVP est contre-indiqu\u00e9e dans la maladie de von Willebrand de type 2B \u2014 elle aggrave la thrombop\u00e9nie. Tout patient avec une MvW connue doit imp\u00e9rativement consulter un centre d'h\u00e9mophilie avant tout geste chirurgical pour \u00e9tablir un plan h\u00e9mostatique individualis\u00e9.<\/p>\n\n<\/div>\n<\/body>\n<\/html>\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<\/div>","protected":false},"excerpt":{"rendered":"<p>Maladie de von Willebrand : diagnostic, types et traitement | Clinique Omicron H\u00e9matologie & M\u00e9decine interne & M\u00e9decine de famille Maladie de von Willebrand La maladie de von Willebrand (MvW) est la coagulopathie h\u00e9r\u00e9ditaire la plus fr\u00e9quente \u2014 touchant environ 1 % de la population mondiale sous sa forme l\u00e9g\u00e8re + bien que la pr\u00e9valence… <a href=\"https:\/\/cliniqueomicron.ca\/en\/von-willebrand\/\" rel=\"bookmark\">Read More \"<span class=\"screen-reader-text\">Von Willebrand Disease: Diagnosis, Types, and Treatment | Clinique Omicron<\/span><\/a><\/p>","protected":false},"author":1,"featured_media":0,"parent":0,"menu_order":0,"comment_status":"closed","ping_status":"closed","template":"","meta":{"om_disable_all_campaigns":false,"neve_meta_sidebar":"","neve_meta_container":"","neve_meta_enable_content_width":"off","neve_meta_content_width":100,"neve_meta_title_alignment":"","neve_meta_author_avatar":"","neve_post_elements_order":"","neve_meta_disable_header":"","neve_meta_disable_footer":"","neve_meta_disable_title":"","_themeisle_gutenberg_block_has_review":false,"_metasync_otto_title":"Von Willebrand (maladie de) | Brossard | Clinique Omicron","_metasync_otto_description":"Von Willebrand (maladie de) Von Willebrand (maladie de) est une condition m\u00e9dicale qui peut n\u00e9cessiter une \u00e9valuation et un suivi m\u00e9dical appropri\u00e9s. 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VWF:Ag VWF:RCo ratio <0.6 qualitatif, DDAVP type 1 tachyphylaxie contre-indiqu\u00e9 type 2B, concentr\u00e9s VWF Haemate P acide tranexamique m\u00e9norragies m\u00e9narche au Qu\u00e9bec.","_metasync_seo_title":"Von Willebrand (maladie de) | Clinique Omicron","_metasync_seo_desc":"","_metasync_breadcrumb_title":"","_metasync_primary_category":0,"_metasync_primary_product_cat":0,"_metasync_imported_seo_title":"","_metasync_imported_seo_desc":"","_metasync_otto_disabled":"","_metasync_hreflang":"","_metasync_plugin_sync_ts":"{\"aioseo\":\"2026-09-03T07:51:27+00:00\"}","_metasync_robots_advanced":"","_metasync_og_title":"Maladie de von Willebrand : diagnostic, types et traitement","_metasync_og_description":"Von Willebrand (maladie de) Von Willebrand (maladie de) est une condition m\u00e9dicale qui peut n\u00e9cessiter une \u00e9valuation et un suivi m\u00e9dical appropri\u00e9s. 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Medicine<\/span>\n <h1>Von Willebrand disease<\/h1>\n<style>.oc-fiche-cta{margin:8px 0 34px;padding:22px 24px;border:1.5px solid rgba(50,60,82,.15);border-left:4px solid #FF611C;border-radius:12px;background:#F4F6F8;font-family:Poppins,sans-serif}.oc-fiche-cta-t{font-size:18px;font-weight:600;color:#323C52;margin:0 0 4px;line-height:1.35}.oc-fiche-cta-x{font-size:15px;color:#4D6577;margin:0 0 14px;line-height:1.55}.oc-fiche-cta-b{display:flex;flex-wrap:wrap;gap:10px}.oc-fiche-cta-b a{display:inline-flex;align-items:center;justify-content:center;min-height:44px;padding:10px 18px;border-radius:8px;font-size:14px;font-weight:600;text-decoration:none;box-sizing:border-box}.oc-fiche-cta-b .oc-p{background:#FF611C;color:#fff}.oc-fiche-cta-b .oc-p:hover{background:#E04E0C}.oc-fiche-cta-b .oc-s{background:#fff;color:#323C52;border:1.5px solid rgba(50,60,82,.25)}@media (max-width:600px){.co-wrap h1{font-size:24px;line-height:1.25;text-transform:none}.oc-fiche-cta{padding:18px 16px}.oc-fiche-cta-b a{flex:1 1 100%}body .co-wrap > .co-table,body .co-wrap > .co-table tbody,body .co-wrap > .co-table tr,body .co-wrap > .co-table td{display:block;width:100%;box-sizing:border-box}body .co-wrap > .co-table{min-width:0;table-layout:auto}body .co-wrap > .co-table thead{display:none}body .co-wrap > .co-table tr{margin:0 0 10px;border:1px solid rgba(77,101,119,.18);border-radius:8px;overflow:hidden}body .co-wrap > .co-table td{padding:9px 12px;border:0}body .co-wrap > .co-table td:first-child{font-weight:600;color:#323C52;background:rgba(77,101,119,.07)}#ocw{transform:scale(.8);transform-origin:bottom right}}<\/style><div class=\"oc-fiche-cta\" data-oc-cta=\"fiche-haut-consult\"><p class=\"oc-fiche-cta-t\">Obtenir un avis adapt\u00e9 \u00e0 votre situation<\/p><p class=\"oc-fiche-cta-x\">Consultation en clinique ou en t\u00e9l\u00e9consultation, partout au Qu\u00e9bec.<\/p><div class=\"oc-fiche-cta-b\"><a class=\"oc-p\" href=\"https:\/\/cliniqueomicron.ca\/rendez-vous\/\">Make an appointment<\/a><a class=\"oc-s\" href=\"https:\/\/cliniqueomicron.ca\/consultation-en-ligne\/\">Consult online<\/a><a class=\"oc-s\" href=\"tel:+15146063350\">514 606-3350<\/a><\/div><\/div>\n\n\n <div class=\"co-intro\">\n La maladie de von Willebrand (MvW) est la coagulopathie h\u00e9r\u00e9ditaire la plus fr\u00e9quente \u2014 touchant environ 1 % de la population mondiale sous sa forme l\u00e9g\u00e8re + bien que la pr\u00e9valence des formes cliniquement significatives n\u00e9cessitant une prise en charge soit estim\u00e9e \u00e0 1 pour 1 000 \u00e0 1 pour 10 000 selon les crit\u00e8res diagnostiques utilis\u00e9s. Elle est caus\u00e9e par un d\u00e9ficit quantitatif ou qualitatif du facteur von Willebrand (VWF) \u2014 une grande glycoprot\u00e9ine multim\u00e9rique synth\u00e9tis\u00e9e par les cellules endoth\u00e9liales et les m\u00e9gacaryocytes + qui joue un double r\u00f4le essentiel dans l'h\u00e9mostase primaire : d'une part + comme prot\u00e9ine d'adh\u00e9sion plaquettaire au sous-endoth\u00e9lium vasculaire l\u00e9s\u00e9 (via la liaison au collag\u00e8ne et au r\u00e9cepteur plaquettaire GPIb) + permettant la formation du clou plaquettaire \u00e0 haute contrainte de cisaillement (art\u00e8res + art\u00e9rioles) + et d'autre part + comme prot\u00e9ine chaperon du facteur VIII de la coagulation (FVIII) + dont il prot\u00e8ge la d\u00e9gradation pr\u00e9matur\u00e9e dans le plasma + expliquant pourquoi un d\u00e9ficit en VWF entra\u00eene secondairement une r\u00e9duction du FVIII (d'o\u00f9 l'allongement du TCA dans certains types de MvW). La MvW a \u00e9t\u00e9 d\u00e9crite pour la premi\u00e8re fois en 1926 par le m\u00e9decin finlandais Erik Adolf von Willebrand + qui d\u00e9crivit une diath\u00e8se h\u00e9morragique familiale touchant une famille d'\u00eeles \u00c5land + avec un tableau de saignements muqueux r\u00e9cidivants (\u00e9pistaxis + m\u00e9norragies + saignements gingivaux + ecchymoses faciles) \u2014 distinct de l'h\u00e9mophilie classique (saignements musculaires et articulaires) par son si\u00e8ge muqueux et sa transmission autosomique (dominant pour les types 1 et 2 + r\u00e9cessif pour le type 3 s\u00e9v\u00e8re). La grande variabilit\u00e9 ph\u00e9notypique + le chevauchement avec les valeurs normales basses dans le type 1 + et l'influence des groupes sanguins ABO sur les taux de VWF (groupe O \u2192 VWF naturellement 20\u201325 % plus bas que les autres groupes) rendent le diagnostic parfois difficile et n\u00e9cessitent souvent plusieurs dosages r\u00e9p\u00e9t\u00e9s et des tests fonctionnels sp\u00e9cialis\u00e9s.\n <\/div>\n\n <h2>Classification of von Willebrand disease types<\/h2>\n\n <table class=\"co-table\">\n <thead>\n <tr>\n <th>Type<\/th>\n <th>D\u00e9ficit<\/th>\n <th>Frequency<\/th>\n <th>VWF:Ag<\/th>\n <th>VWF:RCo<\/th>\n <th>FVIII<\/th>\n <th>Transmission<\/th>\n <\/tr>\n <\/thead>\n <tbody>\n <tr>\n <td>Type 1 (le plus fr\u00e9quent)<\/td>\n <td>Quantitatif partiel \u2014 r\u00e9duction proportionnelle de toutes les formes multim\u00e9rique du VWF<\/td>\n <td>70\u201380 % des MvW<\/td>\n <td>Bas (20\u201350 UI\/dL)<\/td>\n <td>Proportionately<\/td>\n <td>L\u00e9g\u00e8rement bas ou normal<\/td>\n <td>Autosomique dominant \u00e0 p\u00e9n\u00e9trance variable<\/td>\n <\/tr>\n <tr>\n <td>Type 2A<\/td>\n <td>Qualitatif \u2014 perte des grands multim\u00e8res (les plus actifs) \u2192 d\u00e9faut d'adh\u00e9sion plaquettaire<\/td>\n <td>10\u201315 % des MvW<\/td>\n <td>Normal ou l\u00e9g\u00e8rement bas<\/td>\n <td>Tr\u00e8s bas (ratio RCo\/Ag <0,6)<\/td>\n <td>Normal<\/td>\n <td>Autosomal dominant<\/td>\n <\/tr>\n <tr>\n <td>Type 2B<\/td>\n <td>Qualitatif \u2014 gain de fonction \u2192 VWF anormal \u00e0 affinit\u00e9 augment\u00e9e pour GPIb \u2192 agr\u00e9gation spontan\u00e9e des plaquettes \u2192 consommation des grands multim\u00e8res + thrombop\u00e9nie<\/td>\n <td>5 % of the MvW<\/td>\n <td>Normal or low<\/td>\n <td>Tr\u00e8s bas<\/td>\n <td>Normal<\/td>\n <td>Autosomique dominant + DDAVP contre-indiqu\u00e9 (aggrave la thrombop\u00e9nie)<\/td>\n <\/tr>\n <tr>\n <td>Type 2 meters<\/td>\n <td>Qualitatif \u2014 d\u00e9faut de liaison au collag\u00e8ne ou \u00e0 GPIb sans perte des grands multim\u00e8res<\/td>\n <td>Rare<\/td>\n <td>Normal<\/td>\n <td>Tr\u00e8s bas<\/td>\n <td>Normal<\/td>\n <td>Autosomal dominant<\/td>\n <\/tr>\n <tr>\n <td>Type 2N (Normandy)<\/td>\n <td>Qualitatif \u2014 d\u00e9faut de liaison au FVIII \u2192 d\u00e9gradation rapide du FVIII \u2192 tableau ressemblant \u00e0 l'h\u00e9mophilie A l\u00e9g\u00e8re<\/td>\n <td>Rare<\/td>\n <td>Normal<\/td>\n <td>Normal<\/td>\n <td>Tr\u00e8s bas (5\u201340 %)<\/td>\n <td>Autosomique r\u00e9cessif \u2014 peut mimer une h\u00e9mophilie A \u2192 test de liaison VWF-FVIII<\/td>\n <\/tr>\n <tr>\n <td>Type 3 (le plus s\u00e9v\u00e8re)<\/td>\n <td>Quantitatif complet \u2014 absence quasi-totale de VWF<\/td>\n <td>1\u20135 % des MvW<\/td>\n <td>Ind\u00e9tectable ou <3 UI\/dL<\/td>\n <td>Ind\u00e9tectable<\/td>\n <td>Tr\u00e8s bas (1\u201310 %)<\/td>\n <td>Autosomique r\u00e9cessif \u2014 saignements s\u00e9v\u00e8res muqueux + articulaires + musculaires<\/td>\n <\/tr>\n <\/tbody>\n <\/table>\n\n <h2>Clinical presentation<\/h2>\n <ul class=\"co-list\">\n <li><strong>Saignements muqueux (caract\u00e9ristiques de la MvW) :<\/strong> recurrent epistaxis + menorrhagia (often inaugural in women - very heavy first menstruation) + gingival bleeding + easy bruising + prolonged post-dental extraction bleeding + digestive bleeding (telangiectasias + intestinal angiodysplasias in severe forms)<\/li>\n <li><strong>M\u00e9norragies :<\/strong> touchent 32\u2013100 % des femmes avec MvW + souvent m\u00e9connu et attribu\u00e9 \u00e0 tort \u00e0 un cycle irr\u00e9gulier + score PBAC (Pictorial Blood Assessment Chart) >100 = m\u00e9norragies significatives<\/li>\n <li><strong>Saignements post-op\u00e9ratoires :<\/strong> After surgery + childbirth + dental extractions \u2192 mandatory hematologic planning before any invasive procedure<\/li>\n <li><strong>Type 3 s\u00e9v\u00e8re :<\/strong> can resemble hemophilia with muscle and joint bleeding (hemarthrosis) plus mucosal bleeding<\/li>\n <li><strong>Score h\u00e9morragique (BAT \u2014 Bleeding Assessment Tool ISTH) :<\/strong> standardized tool to quantify bleeding severity + score \u2265 3 (male) or \u2265 5 (female) = significant hemorrhagic diathesis \u2192 mandatory investigation<\/li>\n <\/ul>\n\n <h2>Diagnostic biological assessment<\/h2>\n <ul class=\"co-list\">\n <li><strong>Bilan de premi\u00e8re intention :<\/strong> NFS + platelets (thrombocytopenia in type 2B) + PT + aPTT (prolonged if FVIII is low - type 2N + type 3) + bleeding time or PFA-100 (prolonged occlusion time)<\/li>\n <li><strong>Bilan sp\u00e9cialis\u00e9 (h\u00e9matologie) :<\/strong> VWF:Ag (VWF antigen \u2014 quantitative) + VWF:RCo (ristocetin cofactor activity \u2014 functional) + FVIII:C (coagulant) + multimeric analysis by electrophoresis + VWF-FVIII binding test (type 2N) + RIPA (ristocetin-induced platelet aggregation \u2014 type 2B)<\/li>\n <li><strong>VWF:RCo \/ VWF:Ag ratio<\/strong> ratio <0,6 = d\u00e9faut qualitatif (type 2) \u2192 oriente vers les types 2A + 2B + 2M<\/li>\n <li><strong>R\u00e9p\u00e9ter les dosages :<\/strong> VWF is an acute phase protein that increases during inflammation, stress, pregnancy, exercise, and estrogen therapy. Doses can be falsely normal in these contexts. Repeat at a distance from confounding factors.<\/li>\n <li><strong>ABO Blood Group:<\/strong> Group O is associated with lower VWF 20\u201325 % levels than the other groups \u2192 take this into account when interpreting the reference ranges<\/li>\n <\/ul>\n\n <h2>Treatment<\/h2>\n <ul class=\"co-list\">\n <li><strong>Desmopressine (DDAVP \u2014 Minirin\u00ae ou Octostim\u00ae) \u2014 traitement de choix du type 1 :<\/strong> Synthetic vasopressin analogue \u2192 release of endothelial von Willebrand factor (vWF) stores (Weibel-Palade bodies) \u2192 transient increase in vWF and Factor VIII (FVIII) by 3 to 5 times for 4\u20136 hours \u2192 IV + SC + or intranasal administration (Octostim\u00ae nasal spray) + mandatory prior therapeutic test to verify individual response + tachyphylaxis after 2\u20133 consecutive doses (depletion of stores) + contraindicated in type 2B (worsens thrombocytopenia) + adverse effects: hyponatremia (mandatory fluid restriction \u00d7 24 h) + flush + headaches<\/li>\n <li><strong>Concentr\u00e9s de facteur VWF (Haemate P\u00ae + Voncento\u00ae + Wilate\u00ae) :<\/strong> indicated in type 2 + type 3 + DDAVP non-responsive type 1 + and during major surgeries + childbirths + or severe bleeding \u2192 provide vWF and FVIII \u2192 management by a specialized hemophilia center<\/li>\n <li><strong>Acide tranexamique (Cyklokapron\u00ae) :<\/strong> antifibrinolytic + inhibits plasmin \u2192 reduces mucosal bleeding (nosebleeds + menorrhagia + mouth bleeds) \u2192 1 g \u00d7 3\u20134\/d PO + or IV \u2192 often used alone or in combination with DDAVP + particularly effective for mucous membranes<\/li>\n <li><strong>Hormone Therapy (Women):<\/strong> combined oral contraceptives \u2192 raise vWF + reduce menorrhagia + first-line gynecological treatment for menorrhagia related to vWD + levonorgestrel IUD (Mirena\u00ae) \u2192 effectively reduces menstrual flow<\/li>\n <li><strong>Before any invasive procedure (surgery + childbirth + tooth extraction):<\/strong> Mandatory preoperative hematological consultation + individualized hemostatic management plan according to the type and level of vWF and the type of procedure<\/li>\n <\/ul>\n\n <div class=\"co-infobox\">\n <span class=\"ico\">\u2119\ufe0f<\/span>\n <span>La maladie de von Willebrand de type 1 est souvent sous-diagnostiqu\u00e9e chez les femmes \u2014 les m\u00e9norragies depuis la m\u00e9narche \u00e9tant attribu\u00e9es \u00e0 tort \u00e0 un \u00abcycle normal abondant\u00bb pendant des ann\u00e9es. Devant toute m\u00e9norragie inaugurale s\u00e9v\u00e8re (premi\u00e8re r\u00e8gles tr\u00e8s abondantes + an\u00e9mie + n\u00e9cessit\u00e9 de transfusion) + tout saignement post-partum important + ou tout ant\u00e9c\u00e9dent familial de diath\u00e8se h\u00e9morragique + un bilan de coagulopathie incluant les dosages de VWF doit \u00eatre r\u00e9alis\u00e9. La MvW est la cause la plus fr\u00e9quente de coagulopathie chez la femme pr\u00e9sentant des m\u00e9norragies inexpliqu\u00e9es.<\/span>\n <\/div>\n\n <div class=\"co-urgence\">\n <div class=\"co-urgence-titre\">Medical consultation recommended<\/div>\n <p>Consulter un m\u00e9decin ou un h\u00e9matologue si des \u00e9pistaxis r\u00e9cidivantes + des m\u00e9norragies importantes depuis la m\u00e9narche + des saignements prolong\u00e9s apr\u00e8s une extraction dentaire ou une chirurgie mineure + ou des ecchymoses multiples sans traumatisme sont pr\u00e9sents \u2014 ces signes peuvent \u00e9voquer une maladie de von Willebrand n\u00e9cessitant un bilan de coagulation sp\u00e9cialis\u00e9. Tout patient connu avec une MvW doit consulter un h\u00e9matologue avant tout geste invasif. Pour le bilan de coagulation et l'orientation vers l'h\u00e9matologie, Clinique Omicron offre des consultations dans ses points de service au Qu\u00e9bec et en t\u00e9l\u00e9m\u00e9decine. Pour prendre rendez-vous, visitez <a href=\"https:\/\/cliniqueomicron.ca\" style=\"color:#c0392b;font-weight:600;text-decoration:none;\">cliniqueomicron.ca<\/a>.<\/p>\n <\/div>\n\n <h2>Consult at Clinique Omicron<\/h2>\n <p>Les m\u00e9decins et infirmiers praticiens sp\u00e9cialis\u00e9s (IPS) de Clinique Omicron \u00e9voquent le diagnostic de maladie de von Willebrand devant tout tableau de saignements muqueux r\u00e9cidivants + m\u00e9norragies depuis la m\u00e9narche + ou score h\u00e9morragique BAT \u00e9lev\u00e9, prescrivent le bilan de coagulation initial (NFS + TP + TCA + PFA-100) et les dosages sp\u00e9cialis\u00e9s VWF:Ag + VWF:RCo + FVIII, orientent vers un centre d'h\u00e9mophilie pour la confirmation diagnostique et le plan de traitement, prescrivent l'acide tranexamique pour les saignements muqueux, et coordonnent la prise en charge pr\u00e9op\u00e9ratoire. Des consultations sont disponibles dans plusieurs points de service au Qu\u00e9bec et en t\u00e9l\u00e9m\u00e9decine. Pour prendre rendez-vous, visitez <a href=\"https:\/\/cliniqueomicron.ca\" style=\"color:#4D6577;font-weight:600;text-decoration:none;\">cliniqueomicron.ca<\/a>.<\/p>\n\n <p class=\"co-disclaimer\">Le contenu de cette page est fourni \u00e0 titre informatif uniquement et ne remplace pas l'avis d'un m\u00e9decin ou d'un h\u00e9matologue sp\u00e9cialis\u00e9 en coagulopathies. La DDAVP est contre-indiqu\u00e9e dans la maladie de von Willebrand de type 2B \u2014 elle aggrave la thrombop\u00e9nie. Tout patient avec une MvW connue doit imp\u00e9rativement consulter un centre d'h\u00e9mophilie avant tout geste chirurgical pour \u00e9tablir un plan h\u00e9mostatique individualis\u00e9.<\/p>\n\n<\/div>\n<\/body>\n<\/html>\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<\/div>","protected":false},"excerpt":{"rendered":"<p>Maladie de von Willebrand : diagnostic, types et traitement | Clinique Omicron H\u00e9matologie & M\u00e9decine interne & M\u00e9decine de famille Maladie de von Willebrand La maladie de von Willebrand (MvW) est la coagulopathie h\u00e9r\u00e9ditaire la plus fr\u00e9quente \u2014 touchant environ 1 % de la population mondiale sous sa forme l\u00e9g\u00e8re + bien que la pr\u00e9valence… <a href=\"https:\/\/cliniqueomicron.ca\/en\/von-willebrand\/\" rel=\"bookmark\">Read More \"<span class=\"screen-reader-text\">Von Willebrand Disease: Diagnosis, Types, and Treatment | Clinique Omicron<\/span><\/a><\/p>","protected":false},"author":1,"featured_media":0,"parent":0,"menu_order":0,"comment_status":"closed","ping_status":"closed","template":"","meta":{"om_disable_all_campaigns":false,"neve_meta_sidebar":"","neve_meta_container":"","neve_meta_enable_content_width":"off","neve_meta_content_width":100,"neve_meta_title_alignment":"","neve_meta_author_avatar":"","neve_post_elements_order":"","neve_meta_disable_header":"","neve_meta_disable_footer":"","neve_meta_disable_title":"","_themeisle_gutenberg_block_has_review":false,"_metasync_otto_title":"Von Willebrand (maladie de) | Brossard | Clinique Omicron","_metasync_otto_description":"Von Willebrand (maladie de) Von Willebrand (maladie de) est une condition m\u00e9dicale qui peut n\u00e9cessiter une \u00e9valuation et un suivi m\u00e9dical appropri\u00e9s. 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