{"id":10496,"date":"2007-11-15T14:21:21","date_gmt":"2007-11-15T11:21:21","guid":{"rendered":"https:\/\/www.synevo.ro\/afectiuni\/profil-hemostaza-sangerari-anormale\/"},"modified":"2020-07-10T14:17:25","modified_gmt":"2020-07-10T11:17:25","slug":"profil-hemostaza-sangerari-anormale","status":"publish","type":"synevo_diseases","link":"https:\/\/old.synevo.md\/ru\/afectiuni\/profil-hemostaza-sangerari-anormale\/","title":{"rendered":"Profil hemostaz\u0103 (s\u00e2nger\u0103ri anormale)"},"content":{"rendered":"<p style=\"text-align: justify;\"><strong>Profil hemostaz\u0103: Orice s\u00e2ngerare ap\u0103rut\u0103 spontan este anormal\u0103<\/strong>, cu excep\u0163ia celei din cursul menstrua\u0163iei.<!--more--><br><strong>Evaluarea clinic\u0103<\/strong> atent\u0103 a pacientului cu s\u00e2nger\u0103ri anormale poate furniza adesea informa\u0163ii de valoare pentru localizarea anormalit\u0103\u0163ii la nivel: vascular, tombocitar sau la nivelul procesului coagul\u0103rii, iar datele de istoric pot de obicei s\u0103 stabileasc\u0103 dac\u0103 afec\u0163iunea este mo\u015ftenit\u0103 sau dob\u00e2ndit\u0103. Astfel:<br>\u2013 <strong><span style=\"color: #000080;\">afec\u0163iunile trombocitare \u015fi vasculare<\/span><\/strong> (adesea denumite afec\u0163iuni purpurice) au ca tr\u0103s\u0103turi caracteristice: pete\u015fiile hemoragice, echimozele superficiale, de obicei mici, multiple, s\u00e2nger\u0103rile persistente, adesea profuze din r\u0103ni superficiale (prin t\u0103iere sau zg\u00e2riere); sunt relativ mai frecvent \u00eent\u00e2lnite la femei;<br>\u2013 <strong><span style=\"color: #000080;\">tulbur\u0103rile de coagulare<\/span><\/strong> au ca tr\u0103s\u0103turi caracteristice: hematoamele profunde disecante, echimozele superficiale de obicei mari, solitare, hemartrozele, s\u00e2nger\u0103rile \u00eent\u00e2rziate; 80-90% din formele ereditare apar numai la sexul masculin.<br>Investigarea de laborator a pacien\u0163ilor cu s\u00e2nger\u0103ri anormale trebuie condus\u0103 \u00een func\u0163ie de informa\u0163iile ob\u0163inute din evaluarea clinic\u0103.<sup>1<\/sup><\/p>\n<hr>\n<p style=\"text-align: justify;\"><strong><span style=\"color: #000080;\">Afec\u0163iunile trombocitare pot fi:<\/span><\/strong><\/p>\n<ul>\n<li style=\"text-align: justify;\"><strong>cantitative \u2013 trombocitopeniile<\/strong> \u2013 cele mai frecvente afec\u0163iuni hemoragice ce implic\u0103 hemostaza primar\u0103; ca \u015fi anemia reprezint\u0103 un simptom, nu un diagnostic av\u00e2nd mecanisme multiple \u2013 sc\u0103derea produc\u0163iei trombocitare, cre\u015fterea distruc\u0163iei plachetare de cauz\u0103 imunologic\u0103 sau nonimunologic\u0103, distribu\u0163ia anormal\u0103 (\u201cpooling\u201d) a trombocitelor, trombocitopenia ca artefact de laborator, care necesit\u0103 investiga\u0163ii suplimentare; num\u0103rul trombocitelor se coreleaz\u0103 bine cu severitatea s\u00e2nger\u0103rii;<\/li>\n<li style=\"text-align: justify;\"><strong>calitative \u2013 trombocitopatiile \u2013 formele ereditare<\/strong> (ex. defect de adeziune trombocitar\u0103 \u2013 sd. Bernard-Soulier, defect de agregare trombocitar\u0103 \u2013 trombastenia Glanzmann, defect de secre\u0163ie) sunt adesea asociate cu trombocitopenie u\u015foar\u0103 \u015fi sunt rar \u00eent\u00e2lnite fa\u0163\u0103 de formele dob\u00e2ndite, care pot complica o varietate de afec\u0163iuni (afec\u0163iuni ale sistemului hematopoietic \u2013 sindroame mieloproliferative, sindroame mielodisplazice, paraproteinemii; uremie; disfunc\u0163ia plachetar\u0103 indus\u0103 de medicamente).<sup>1,2,3<\/sup><\/li>\n<\/ul>\n<p style=\"text-align: justify;\"><span style=\"color: #000080;\"><strong>Boala von Willebrand (B.vW) sau pseudohemofilia<\/strong><\/span> \u2013 afec\u0163iune hemoragic\u0103 ereditar\u0103 transmis\u0103 autosomal, are la baz\u0103 o deficien\u0163\u0103 sau o anomalie a complexului factorului VIII cu toate cele trei frac\u0163iuni constitutive ale sale (C, Ag, vW), caracterizat\u0103 clinic prin anormalit\u0103\u0163i \u00een hemostaza primar\u0103 (hemoragii de tip capilar) combinate cu manifest\u0103ri moderate de tip hemofilic4; c\u00e2nd timpul de s\u00e2ngerare este prelungit \u015fi num\u0103rul trombocitelor este normal, investiga\u0163iile de laborator de obicei sunt direc\u0163ionate pentru excluderea BvW pentru c\u0103 ea este mult mai frecvent\u0103 (1% din popula\u0163ie) dec\u00e2t trombopatiile ereditare.<sup>1<\/sup><\/p>\n<hr>\n<p style=\"text-align: justify;\"><span style=\"color: #000080;\"><strong>Afec\u0163iunile vasculare pot fi:<\/strong><\/span><\/p>\n<ul>\n<li style=\"text-align: justify;\"><strong>congenitale<\/strong> ex. teleangiectazia hemoragic\u0103 Rendu-Osler \u2013 afec\u0163iune ereditar\u0103 cu transmitere autosomal dominant\u0103, prezent\u0103 la ambele sexe, cu o frecven\u0163\u0103 de 1-3\/100.000, adesea nediagnosticat\u0103, caracterizat\u0103 prin multiple teleangiectazii la nivelul tegumentelor \u015fi mucoaselor asociate cu epistaxis \u015fi alte complica\u0163ii hemoragice, profilul coagul\u0103rii este de obicei normal, numai \u00een cursul \u015funturilor arteriovenoase (ex. din pl\u0103m\u00e2n) coagularea consumptiv\u0103 cronic\u0103 poate apare cu modificarea corespunz\u0103toare a testelor de hemostaz\u0103 5,6; hemangiomul gigant congenital (sindromul Kasabach-Merritt) poate determina coagulopatia cronic\u0103 de consum; afec\u0163iuni ereditare ale \u0163esutului conjunctiv etc.;<sup>6<\/sup><\/li>\n<li style=\"text-align: justify;\"><strong>dob\u00e2ndite<\/strong> ex. purpur\u0103 reumatoid\u0103 Henoch-Schonlein \u2013 vasculit\u0103 a vaselor mici cu depozite imune predominant IgA, cu etiologie nesigur\u0103, posibil infec\u0163ioas\u0103 (70-100% din cazuri, prezent\u0103 o infec\u0163ie anterioar\u0103 de c\u0103i aeriene superioare, cu streptococ beta-hemolitic) sau datorat\u0103 hipersensibilit\u0103\u0163ii la antigene bacteriene, esen\u0163ial\u0103 este absen\u0163a oric\u0103rei modific\u0103ri a hemostazei, cu testul rezisten\u0163ei capilare pozitiv (Rumpell-Leede);7 microangiopatiile ex. microangiopatia Moschowitz (purpura trombocitopenica trombohemolitica), sindromul hemolitic uremic \u00een ambele sunt posibile semne de coagulare intravascular\u0103 diseminat\u0103 (CID) sau localizat\u0103; <sup>6<\/sup><\/li>\n<\/ul>\n<p style=\"text-align: justify;\"><strong>Sindroamele hemoragice plasmatice<\/strong> \u2013 se datoresc unor deficien\u0163e la nivelul factorilor coagul\u0103rii \u015fi pot fi \u00eemp\u0103r\u0163ite \u00een:<\/p>\n<p style=\"text-align: justify;\"><span style=\"color: #000080;\"><strong>Tulbur\u0103ri la nivelul c\u0103ii intrinseci a coagul\u0103rii <\/strong><\/span>\u2013 caracterizate prin APTT prelungit \u015fi PT normal<\/p>\n<ul>\n<li style=\"text-align: justify;\"><strong>formele congenitale<\/strong>, reprezentate \u00een &gt; 94% din cazuri de hemofilia A, hemofilia B \u015fi boala von Willebrand. Hemofiliile A \u015fi B sunt afec\u0163iuni hemoragice ereditare, cu transmitere recesiv\u0103 X \u2013 linkat\u0103, care au la baz\u0103 deficien\u0163a sau func\u0163ionarea defectuoas\u0103 a factorului VIIIc respectiv factorului IX; transmiterea genetic\u0103 este tipologic\u0103 (A sau B), dar \u015fi cantitativ\u0103 (gradul de inhibi\u0163ie al genei = gradul de severitate al bolii) de obicei hemofilia av\u00e2nd acela\u015fi grad de severitate \u00een cadrul acelea\u015fi familii; clinic, se caracterizeaz\u0103 printr-o simptomatologie asem\u0103n\u0103toare, predominant articular\u0103 (hemartozele) \u00een 75% din cazuri sau muscular\u0103 (hematoame profunde), diferen\u0163ierea celor dou\u0103 tipuri de hemofilie f\u0103c\u00e2ndu-se prin analize specifice.<sup>4<\/sup><\/li>\n<li style=\"text-align: justify;\"><strong>formele dob\u00e2ndite<\/strong> \u2013 includ lupus inhibitor \u015fi anticorpii \u00eempotriva factorului VIII; determinarea APTT dup\u0103 amestecul cu plasm\u0103 normal\u0103 ajut\u0103 la diferen\u0163ierea celor dou\u0103 tipuri de deficien\u0163e (-\/+ anticorpi inhibitori). Prelungirea APTT apare \u015fi \u00een cursul tratamentului cu heparin\u0103.<\/li>\n<\/ul>\n<p style=\"text-align: justify;\"><strong><span style=\"color: #000080;\">Tulbur\u0103ri la nivelul c\u0103ii comune a coagul\u0103rii<\/span> <\/strong>\u2013 caracterizate prin APTT \u015fi PT prelungite la un pacient cu o afec\u0163iune ereditar\u0103, indic\u0103 o deficien\u0163\u0103 a unuia din factorii c\u0103ii comune: factorul X, V, II sau I; asemenea deficien\u0163a izolat\u0103 a unui factor este rar\u0103. Pe de alt\u0103 parte, deficien\u0163a unui sau mai mul\u0163i din ace\u015fti factori este \u00eenso\u0163it\u0103 de anormalit\u0103\u0163i adi\u0163ionale \u00een calea intrinsec\u0103 \u015fi extrinsec\u0103, \u00een multe afec\u0163iuni comune, dob\u00e2ndite ale coagul\u0103rii ex. deficien\u0163a de vitamina K, boli hepatice \u015fi coagularea vascular\u0103 diseminat\u0103 (CID). Un PT prelungit de obicei sugereaz\u0103 o afec\u0163iune dob\u00e2ndit\u0103 (excluz\u00e2nd rarele cazuri de deficien\u0163\u0103 ereditar\u0103 de factor VII) \u015fi de obicei este asociat\u0103 cu un complex de anormalit\u0103\u0163i implic\u00e2nd multiple c\u0103i cum ar fi CID.<\/p>\n<p style=\"text-align: justify;\"><span style=\"color: #000080;\"><strong>Tulbur\u0103ri la nivelul c\u0103ii extrinseci a coagul\u0103rii <\/strong><\/span>\u2013 APTT normal, cu PT prelungit, rareori sugereaz\u0103 o deficien\u0163\u0103 izolat\u0103 de factor VII, ereditar\u0103 sau dob\u00e2ndit\u0103; unele cazuri de CID sau difibrinogenemie se pot prezenta cu un PT prelungit, izolat.<\/p>\n<p style=\"text-align: justify;\"><strong>CID, sindromul de defibrinare sau coagulopatia de consum <\/strong>este un sindrom caracterizat clinic printr-o intricare de manifest\u0103ri trombotice \u015fi hemoragice \u015fi fiziopatologic printr-o generare intravascular\u0103 extins\u0103 de fibrin\u0103, datorit\u0103 activ\u0103rii c\u0103ii extrinseci a coagul\u0103rii produs\u0103 de o eliberare de material coagulant \u015fi a c\u0103ii intrinseci a coagul\u0103rii, datorit\u0103 unor leziuni difuze a endoteliului vascular sau unei agreg\u0103ri generalizate a trombocitelor. Sindromul CID nu este considerat o entitate de sine st\u0103t\u0103toare, ci un mecanism intermediar de boal\u0103, care poate ap\u0103rea \u00een cursul unui num\u0103r \u00eensemnat de afec\u0163iuni primare acute sau cronice (complica\u0163ii obstetricale, infec\u0163ii, neoplasme, afec\u0163iuni sistem hematopoietic, boli vasculare, injurii tisulare masive)4; nivelul fibrinogenului plasmatic, APTT, PT, D-dimeri, num\u0103rul trombocitelor, aspectul frotiului sanguin pentru schizocite, stau la baza diagnosticului de laborator.<sup>9<\/sup><br><small><br><\/small><\/p>\n<p><\/p><div class=\"wp-block-columns is-layout-flex wp-container-core-columns-is-layout-1 wp-block-columns-is-layout-flex\"><div class=\"wp-block-column is-vertically-aligned-center is-layout-flow wp-block-column-is-layout-flow\" style=\"flex-basis:33.33%\"><p><strong>Analize medicale disponibile \u00een laboratoarele Synevo: <\/strong><\/p><\/div>\n\n<div class=\"wp-block-column is-layout-flow 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class=\"sysprod-product-price\">\r\n                        <span>Pre\u021b:<\/span>\r\n                        <span>265 lei<\/span>\r\n                    <\/div>\r\n                                            <div class=\"sysprod-product-addcart\">\r\n                            <a\r\n                                href=\"?add-to-cart=9217\"\r\n                                data-quantity=\"1\"\r\n                                class=\"addToCart button product_type_simple add_to_cart_button ajax_add_to_cart \"\r\n                                data-product_id=\"9217\"\r\n                                data-product_sku=\"HE31\"\r\n                                rel=\"nofollow\"\r\n                            ><div><\/div><\/a>\r\n                        <\/div>\r\n                                    <\/div>\r\n                            <div class=\"sysprod-product\">\r\n                    <a class=\"sysprod-product-title\" href=\"https:\/\/old.synevo.md\/ru\/shop\/fibrinogen\/\">Fibrinogen<\/a>\r\n                    <div class=\"sysprod-product-price\">\r\n                        <span>Pre\u021b:<\/span>\r\n                        <span>120.00 lei<\/span>\r\n                    <\/div>\r\n                                            <div class=\"sysprod-product-addcart\">\r\n                            <a\r\n                                href=\"?add-to-cart=9237\"\r\n                                data-quantity=\"1\"\r\n                                class=\"addToCart button product_type_simple add_to_cart_button ajax_add_to_cart \"\r\n                                data-product_id=\"9237\"\r\n                                data-product_sku=\"HE05\"\r\n                                rel=\"nofollow\"\r\n                            ><div><\/div><\/a>\r\n                        <\/div>\r\n                                    <\/div>\r\n                    <\/div>\r\n        \r\n    <\/div>\r\n<\/div>\r\n\r\n<style>\r\n.synevo-shop-products {\r\n    padding: 24px 0;\r\n    margin: 24px 0;\r\n    border-radius: 10px;\r\n    background: #F6F7F9;\r\n}\r\n.synevo-shop-products .sysprod-product {\r\n    position: relative;\r\n    margin: 0 24px;\r\n    margin-bottom: 24px;\r\n    border-radius: 10px;\r\n    background: #fff;\r\n    display: flex;\r\n    flex-direction: row;\r\n    align-items: center;\r\n}\r\n.synevo-shop-products .sysprod-product:last-child {\r\n    margin-bottom: 0;\r\n}\r\n.synevo-shop-products .sysprod-product-title {\r\n    flex-grow: 1;\r\n    padding: 15px;\r\n    margin-left: 14px;\r\n    font-family: Poppins,sans-serif;\r\n    font-weight: 600;\r\n    font-size: 16px;\r\n    color: #29263F;\r\n}\r\n.synevo-shop-products .sysprod-product-price {\r\n    flex-shrink: 0;\r\n    font-family: Poppins,sans-serif;\r\n    font-weight: 600;\r\n    font-size: 18px;\r\n    color: #FDB813;\r\n}\r\n.synevo-shop-products .sysprod-product-addcart {\r\n    flex-shrink: 0;\r\n    position: relative;\r\n    width: 38px;\r\n    height: 38px;\r\n    margin-left: 24px;\r\n    margin-right: 24px;\r\n}\r\n.synevo-shop-products .sysprod-product-addcart .button {\r\n    top: unset;\r\n    right: unset;\r\n}\r\n<\/style><\/div><\/div><hr class=\"wp-block-separator\"\/><p><small>Bibliografie<\/small><\/p><p><small>1.George M. Rodgers and Thomas C. Bithell, The Diagnostic Approach to the Bleeding Disorders, \u00een Wintrobe\u2019s Clinical Hematology, 10 Ed., 1999, 1557-1578.<br>2.Shirley Parker Levine, Thrombocytopenia Pathophysiology and Classification, \u00een Wintrobe\u2019s Clinical Hematology, 10 Ed., 1999, 1579-1582.<br>3. Shirley Parker Levine, Qualitative Disorders of Platelet Function, \u00een Wintrobe\u2019s Clinical Hematology, 10 Ed., 1999, 1661-1662.<br>4. C. Poppa, Florica Enache, Sindroame Hemoragice Plasmatice, \u00een Tratat de Medicina Interna, sub redactia Radu Paun, coordonator Dan Coli\u0163\u0103, Hematologie partea II, 1997, 779-854.<br>5. Wintrobe\u2019s pg.1634<br>6. Hans D. Bruhn, Hemorrhagic disorders, In Clinical Laboratory Diagnostics- Use and Assessment of Clinical Laboratory Results. Lothar Thomas, TH- Books Verlagsgesellschaft mbH, Frankfurt \/Main, Germany,1 Ed., 1998, 571.<br>7. G. Popa, Sindroame hemoragice de origine vasculara, In Tratat de Medicina Interna, sub redactia Radu Paun, coordonator Dan Colita, Hematologie partea II, 1997, 670-673.<br>8. WI<br>9. Alan B. M. Grosset, George M. 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