{"id":10446,"date":"2009-08-12T12:51:09","date_gmt":"2009-08-12T09:51:09","guid":{"rendered":"https:\/\/www.synevo.ro\/afectiuni\/profil-boli-mieloproliferative-cronice\/"},"modified":"2020-07-10T12:03:50","modified_gmt":"2020-07-10T09:03:50","slug":"profil-boli-mieloproliferative-cronice","status":"publish","type":"synevo_diseases","link":"https:\/\/old.synevo.md\/ru\/afectiuni\/profil-boli-mieloproliferative-cronice\/","title":{"rendered":"Profil boli mieloproliferative cronice"},"content":{"rendered":"<p><strong><span style=\"color: #000080;\">Bolile mieloproferative cronice (BMPC)<\/span> <\/strong>sunt afec\u0163iuni clonale, distincte, dar \u00een str\u00e2ns\u0103 leg\u0103tur\u0103, cu originea \u00een celula stem pluripotent\u0103 din m\u0103duv\u0103. Ele includ: <strong>leucemia mieloid\u0103 cronic\u0103<\/strong>, <strong>policitemia vera<\/strong>, <strong>trombocitemia esen\u0163ial\u0103<\/strong> \u015fi <strong>mielofibroza<\/strong>.<\/p><!--more--><p>\u00cen toate BMPC, capacitatea de proliferare a celulei stem neoplazice nu este controlat\u0103 corespunz\u0103tor \u015fi ini\u0163ial apare hematopoieza excesiv\u0103. Celula stem neoplazic\u0103 re\u0163ine \u00eens\u0103 abilitatea de diferen\u0163iere complet\u0103 \u015fi ca urmare, apare o cre\u015ftere marcat\u0103 a formelor mature \u015fi imature \u00een s\u00e2ngele periferic \u015fi m\u0103duva osoas\u0103.<\/p><p>BMPC se disting una de alta prin tipul predominant de diferen\u0163iere pe care \u00eel sufer\u0103 celula stem neoplazic\u0103 (ex. linie mieloida \u00een LMC, megacariocitar\u0103 \u00een trombocitemia esen\u0163ial\u0103, eritrocitar\u0103 \u00een policitemia vera). BMPC au o evolu\u0163ie progresiv\u0103, posibil spre insuficien\u0163\u0103 medular\u0103 sau leucemie acut\u0103. Diferen\u0163ele \u00eentre prognosticul \u015fi tratamentul optim din cadrul BMPC explic\u0103 necesitatea distinc\u0163iei acestor entit\u0103\u0163i. Pentru c\u0103 BMPC iau na\u015ftere dintr-o celul\u0103 stem pluripotent\u0103, care se diferen\u0163iaz\u0103 pe toate liniile celulare cu predomina\u0163\u0103 pe o alt\u0103 linie celular\u0103, nu este surprinz\u0103tor faptul c\u0103 ocazional, tr\u0103s\u0103turile clinice \u015fi morfologice se pot suprapune \u015fi clasificarea respectiv diagnosticul devin dificile.<sup>1<\/sup><\/p><p><em><strong>Leucemia mieloid\u0103 (granulocitar\u0103) cronic\u0103 (LGC)<\/strong><\/em>, cu o frecven\u0163\u0103 de 15-20% din leucemiile adultului \u015fi cea mai frecvent\u0103 BMPC, este o afec\u0163iune clonal\u0103 a celulei stem pluripotente caracterizat\u0103 prin cre\u015fterea prolifer\u0103rii elementelor mieloide \u00een toate stadiile de diferen\u0163iere. Prezen\u0163a \u00een num\u0103r foarte mare a acestora \u00een s\u00e2ngele periferic este esen\u0163ial\u0103 pentru stabilirea diagnosticului, al\u0103turi de alte semne importante: cre\u015fterea granulocitelor bazofile, sc\u0103derea fosfatazei alcaline leucocitare (FAL), cre\u015fterea nivelului seric al vitaminei B12 \u015fi a transcobalaminelor I \u015fi III. \u00cen plus, o anomalie citogenetic\u0103, cromozomul Philadelphia, se g\u0103se\u015fte la 95% din cazurile tipice de (LGC) \u00een faza cronic\u0103. El apare \u00een 100% din celulele de diviziune din m\u0103duva osoas\u0103 \u015fi contribuie la diagnostic \u015fi la monitorizarea tratamentului. Eviden\u0163ierea genei de fuziune BCR\/ABL \u015fi a proteinei himerice p210, cu activitate tirozin-kinazica, pe care aceasta o codific\u0103, definesc LGC la nivel molecular. Simptomele \u015fi semnele clinice se dezvolt\u0103 insiduos \u015fi includ pe primul plan astenia \u015fi splenomegalia progresiv\u0103. Dup\u0103 o medie de 3,5 ani, LGC trece dintr-o faz\u0103 cronic\u0103 \u00een una acut\u0103, blastic\u0103, care poate fi mieloid\u0103 sau limfoid\u0103.<sup>2<\/sup><\/p><p><em><strong>Policitemia vera (PV)<\/strong><\/em> este caracterizat\u0103 prin cre\u015fterea predominant\u0103 a eritrocitelor \u015fi masei eritrocitare, cu o satura\u0163ie de O2 arterial\u0103 normal\u0103. Supraproduc\u0163ia este frecvent asociat\u0103 cu niveluri serice sc\u0103zute de eritropoetina. De obicei asociaz\u0103 leucocitoza, trombocitoza, cre\u015fterea scorului FAL (80-100% din pacien\u0163i). M\u0103duva osoas\u0103 este hipercelular\u0103, cu hiperplazia liniilor: mieloid\u0103, eritroid\u0103, megakariocitar\u0103.<br>Manifest\u0103rile clinice principale sunt pruritul agnogenic, colora\u0163ia ro\u015fie-violacee a tegumentelor \u015fi mucoaselor, splenomegalie, complica\u0163ii trombotice \u015fi hemoragice.<br>\u00cen evolu\u0163ia sa natural\u0103, PV poate vira \u00eentr-o alt\u0103 BMPC (frecvent MMM, foarte rar LGC), cu o faz\u0103 terminal\u0103 de leucemie acut\u0103, uneori precedat\u0103 de sindrom mielodisplazic.<sup>3<\/sup><\/p><p><em><strong>Metaplazia mieloid\u0103 agnogenic\u0103 (MMA)<\/strong><\/em> sau metaplazia mieloid\u0103 cu mielofibroz\u0103 este o entitate distinct\u0103 \u00een cadrul BMPC, caracterizat\u0103 prin coexisten\u0163a a trei tulbur\u0103ri citologice fundamentale: proliferare clonal\u0103 a celulelor stem hematopoietice din m\u0103duv\u0103, proliferarea nonclonal\u0103 reactiv\u0103 a celulelor stromale medulare (fibrobla\u015fti \u015fi osteobla\u015fti), care duce la mielofibroz\u0103 (MF) \u015fi osteomieloscleroz\u0103 (OMS) \u015fi hematopoieza extramedular\u0103 (metaplazie meloid\u0103) \u00een splin\u0103, ficat \u015fi alte organe. MF \u015fi OMS au drept consecin\u0163\u0103 o insuficien\u0163\u0103 medular\u0103 progresiv\u0103 cu pancitopenie.<br>Tabloul clinic este dominat de splenomegalia tumoral\u0103. \u00cen diagnosticul de laborator un rol important \u00eel ocup\u0103 aspectul frotiului de s\u00e2nge periferic.<br>Evolu\u0163ia clinic\u0103 este lent progresiv\u0103. Un procent variabil din pacien\u0163ii cu MMA tipic\u0103 fac o transformare \u00een leucemie acut\u0103.<sup>4<\/sup><\/p><p><em><strong>Trombocitemia esen\u0163ial\u0103 (TE) <\/strong><\/em>este ca \u015fi celalalte BMPC o boal\u0103 clonal\u0103 cu origine \u00een celula stem pluripotent\u0103 din m\u0103duva osoas\u0103, av\u00e2nd drept tulburare citologic\u0103 principal\u0103 hiperplazia megacariocitar\u0103 din m\u0103duva osoas\u0103, care are ca urmare cre\u015fterea excesiv\u0103 a trombocitelor din s\u00e2nge.<br>Diagnosticul TE este un diagnostic de excludere. Criteriile utilizate \u00een acest scop sunt arbitrare \u015fi necesit\u0103 o analiz\u0103 atent\u0103.<sup>5<\/sup><\/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\"><p><strong>Teste disponibile \u00een laboratorul Synevo<\/strong>:<\/p><\/div>\n\n<div class=\"wp-block-column is-layout-flow wp-block-column-is-layout-flow\"><div class=\"synevo-shop-products\">\r\n    <div class=\"sysprod\">\r\n        \r\n        <div class=\"sysprod-products\">\r\n                            <div class=\"sysprod-product\">\r\n                    <a class=\"sysprod-product-title\" href=\"https:\/\/old.synevo.md\/ru\/shop\/hemograma-cu-formula-leucocitara-hbhtindici-si-reticulocite-hemoleucograma\/\">Hemogram\u0103 cu formul\u0103 leucocitar\u0103, Hb,Ht,indici \u0219i reticulocite (analiza general\u0103 a s\u00e2ngelui)<\/a>\r\n                    <div class=\"sysprod-product-price\">\r\n                        <span>Pre\u021b:<\/span>\r\n                        <span>145.00 lei<\/span>\r\n                    <\/div>\r\n                                            <div class=\"sysprod-product-addcart\">\r\n                            <a\r\n                                href=\"?add-to-cart=9253\"\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=\"9253\"\r\n                                data-product_sku=\"HE08\"\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\/medulograma\/\">Medulograma<\/a>\r\n                    <div class=\"sysprod-product-price\">\r\n                        <span>Pre\u021b:<\/span>\r\n                        <span>360.00 lei<\/span>\r\n                    <\/div>\r\n                                            <div class=\"sysprod-product-addcart\">\r\n                            <a\r\n                                href=\"?add-to-cart=9269\"\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=\"9269\"\r\n                                data-product_sku=\"HE10\"\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><br><strong>Bibliografie:<\/strong><\/small><\/p><p><small>1. Richard S. Larson and Steven N. Wolff, Chronic Myeloid Leukemia, \u00cen Wintrobe\u2019s Clinical Hematology, 10 Ed., 1999, 2343-2344<br>2. Elena Butoianu, Emilia Niculescu-Mizil, Leucemia Mieloida Cronic\u0103, \u00cen Tratat de Medicin\u0103 Intern\u0103, sub redac\u0163ia Radu P\u0103un, coordonator Dan Coli\u0163\u0103, Hematologie partea II, 1997, pg.133<br>3. S. Nicoar\u0103, Policitemia Vera, \u00cen Tratat de Medicin\u0103 Intern\u0103, sub redac\u0163ia Radu P\u0103un, coordonator Dan Coli\u0163\u0103, Hematologie partea II, 1997,185<br>4. Adriana Coli\u0163\u0103, Elena Butoianu, S. Nicoar\u0103, Metaplazie Mieloid\u0103 Agnogenic\u0103, \u00cen Tratat de Medicin\u0103 Intern\u0103, sub redac\u0163ia Radu P\u0103un, coordonator Dan Coli\u0163\u0103, Hematologie partea II, 1997, 164<br>5. S. Nicoar\u0103, Andreea Moicean, Trombocitemia Esentiala, \u00cen Tratat de Medicin\u0103 Intern\u0103, sub redac\u0163ia Radu P\u0103un, coordonator Dan Coli\u0163\u0103, Hematologie partea II, 1997, 203-208)<\/small><\/p><p><small><br><span style=\"font-size: 12pt;\">Teste utilizate \u00een laboratorul Synevo pentru diagnosticul BMPC:<\/span><\/small><\/p><ul class=\"wp-block-list\"><li><a href=\"\/shop\/hemograma-cu-formula-leucocitara-hbhtindici-si-reticulocite-hemoleucograma\/\" class=\"rank-math-link\"><span style=\"font-size: 12pt;\"><small>Hemogram\u0103 cu formul\u0103 leucocitar\u0103 cu Hb, Ht, indici \u015fi reticulocite<\/small><\/span><\/a><\/li><li><a href=\"\/shop\/frotiu-de-sange-periferic\/\" class=\"rank-math-link\"><span style=\"font-size: 12pt;\"><small>Frotiu s\u00e2nge periferic<\/small><\/span><\/a><\/li><li><a href=\"\/shop\/medulograma\/\" class=\"rank-math-link\"><span style=\"font-size: 12pt;\"><small>Medulogram\u0103<\/small><\/span><\/a><\/li><li><a href=\"\/shop\/concentrat-leucocitar\/\" class=\"rank-math-link\"><span style=\"font-size: 12pt;\"><small>Concentrat leucocitar<\/small><\/span><\/a><\/li><li><span style=\"font-size: 12pt;\"><small><a href=\"\/shop\/fosfataza-alcalina-leucocitara-indice-fal\/\" class=\"rank-math-link\">Indice FAL<\/a><\/small><\/span><\/li><\/ul><p><\/p>","protected":false},"featured_media":0,"template":"","synevo_diseases_category":[893],"class_list":["post-10446","synevo_diseases","type-synevo_diseases","status-publish","hentry","synevo_diseases_category-diabet-zaharat-nutritie-si-boli-metabolice"],"_links":{"self":[{"href":"https:\/\/old.synevo.md\/ru\/wp-json\/wp\/v2\/synevo_diseases\/10446","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/old.synevo.md\/ru\/wp-json\/wp\/v2\/synevo_diseases"}],"about":[{"href":"https:\/\/old.synevo.md\/ru\/wp-json\/wp\/v2\/types\/synevo_diseases"}],"wp:attachment":[{"href":"https:\/\/old.synevo.md\/ru\/wp-json\/wp\/v2\/media?parent=10446"}],"wp:term":[{"taxonomy":"synevo_diseases_category","embeddable":true,"href":"https:\/\/old.synevo.md\/ru\/wp-json\/wp\/v2\/synevo_diseases_category?post=10446"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}