Hematologie-Neoplazii mieloproliferative cronice, Summaries of Hematology

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Neoplasi midoproliferative rouice Leucencia mieloda cronica Tablow elinic : -majritatio saciensler must diagnostical, inJazacrovica a bali simptomatologia : asimptomatici in aza eronica- 50% pacienti not pr T - pacieluti 11 in aza cronicaau simpioussimptomatic T respecific : askuir fizica, inapatenta , scader pooderato,HeLanspira ↑ y - in a tele acceleraGlassin totolarva simptomatic , la adaugande-se ats de eitopeniExauenl - in Jaza splira splecoeg aliei este samuel /cel mai Monica,prezentadine,palpabilonaur . de leuereits deposplenomegalia in LM2 zste de radwere/i- 9 9 -I so doub & ta F - i tatourgaliaa ticastore - - her Fpra, mustprezeenteI Tara sau blas chimozepallrea Legume apurpura I kuopati sou infiltratio entanatepure reveuta in yousele Pl-in pezeleaccelerata s blastical I Explanparadivia: - Hb N - leneocitize > 100. 000 /was - houbocit N/↑ uveau > 1000000/um3 - Tourla~ leucocitaroriatsta please latwielblast , e prezeu&de natural davada I a matural norale in / series gravelocitara - mieloblastics" in rangele periferic - bazafilie 5-15% si rozinofili Saccola breocitara exista'svar :Mielocit +-intouielits i sequent Justatet resegmentatebi in fazele accelerata's blastica : - anzuie Howbotosaur,accenturepoubotaT creste proceutul de wirloblastawela intermediar/mirlite , matamiesoods surrental n' be so se conturze unhiatus leacemicrate celui div LA est proceea bazafile investigati biochimice :I- ac , ulic si LDHP d .pee masa humorala - hiperhistaminemie - uit Bi24 - test ol wag vlare alterate secundar tombopatien - Posfataza lealiva leneocitarav , ↑ in feza& accelerat san blastica 3) Exauveur madures goase haratogene:edutaobligatorio pt. diagu. pandinasa etageneticse efctuenza exauver Policiteria Uzra Tablou Clinic : Sicptomatologic : must def. d brea visitatides song ales acuf asteui : auxpli , al , ditaade ina & evomeux eur , if Raynard,auf hidrade obes fars. Debutul prote Ji print-- complicate , in queeral complicati hombotice.I & prurit & eneralizat , accentrat o baile fier birtylat- Vasodilatatiei : / oseufaIncidenaza a vlceruleigastaderadeval fizica, hiper his faniseries Asteria conseciuta excesului de citokive , silresponsabil I de hauspirati nocturne, durer' otoat,prurit , Jebra Examenl ob - : dz . exitazei generalizat, en↑tuta' violacer , van accentrata la wi . xhemita ause la soarea a teguantelo exdrauregalie9 I geDatarita susitueri , jonaro a livingratag Explaini saraclinice :1)HLG : - 46 wi Her 24 >68%) ↓ Leudocituza (120.000/wmB) ↓ Trombocitara /1000 .000- 100 .000 i) 2) liperuric erpretent I poate atof a 3) ere 4/exauenl madures asoase heratogene : s realize pli peaa biopsie astourdulaba" ;bogatoliporelulare ra itoats limits wieloids , des F est eous soo If lea in evolutietautprezenta, I S apaBeopsion oskouedular m este obligatorie docatati Jack-2 Kinazei este prezenta siNee a 2 scabut card I val a persistents ll t entreprenea si > 49 ,5% #) 'Fiblaza wedulares extinca - factor predictiv de evolute note MMM. & 51 Analiza moleculata e ident lustatifie / a aakiazei adobligatin a st M .J & za- diag alsercop splenomeg Diagnostic positiv : Criteri Majores-Hb > 16 ,51B) six/ (F) SAUHBring (f)SAU o cresters a masesentroids 215% - biopria astanedular ca evidentiazaL hipercelularitate (paumieloza) ~ proliferar↑ critoida, samsloctara si ocitara 9 / deal ↳ regacoriite mature aegdif diversinierezenta anomalici Jackskivazei sou amutation-I ack la viv . exonvlui 12 I JCriteriu neitos : - wil serie sout al critropoieticiAeriae Pt . diagn . + de certitudine Lebrie indeplints toatele majore , sow, in absenta mutati ackzivazei,criteri , ei] privele 2 criteri majore plus criterialvivor . I Principi de Latament : ↑ Preste S ective incurabila, objectivelof S terapies specifice but : -reducerea riscule complicatili homboticeInducerra risculvi erasilossang I -amelioratia touces ii siviiep tolog Ireducera Miscule de evoluti heT 20 mielsfibraza secundar, sid . Wields sou LA plat erst deJudif rupa de rise , pleborentzele repetate9 se rudica & la creetara val . Ht . le o val%pana , Flebazeufez un wai must o 250z epe flebrzen S , AT ef I sing , ↳za · in sarael en hidhafale of a sel accentua so Hh . de Lipervaseria de likePac . or site seabut care par.su HTA ,microtoubote , etc , are indicatiin special eng & ide hat al vaout en daze mic de AspirivaPecienti ei Mise resent an indicatio de not citoreduxtor en Hidroxiure . diagnostic , fire unl diete critrile obligatorie af Nottia positive . 6) Time· pur de sangerar poate fimabit 7Hiperviseiate poo Eco and. Postsea splenomegalia Diagnostic positiv : Cateri major : -houboite Spoodzub -proliferate in special negacariocitarala bropria otoata, en regacalizeite dedim . Mari , das ologie natura,a wat z nuclei solibbal ; crestere mesernit es seril ravelocitara sicritcitara9 sift nar restero fibrelos de reticulina - Excludereca relocate neoplasicrative craice , a sorwielsprolif miclodisplazies san a alter reglazi wieloid-Demonstrated prez . uni worker loual , prezenta anomalies jakive, CALR , MPL Criteriu wind : ~presvaltworkercouna Reactiva Pt . lig↑ & u . Habric indeplimite took ente Leviteri way sa primet 3 wajre + ritecive wivr Princip de Latament: - Te esti o boala incurabili, la ears tat neiflumpto supracti S labala , scopulfind a cela deNa prvovira complicatilor pambotic san hemorgiceavevistators de viato. - La pieti en risaH . scabut , dace me actoi pur .T 1 . de risa . C se recomandarviteria terapies anting - daca mustpres , Yoetow or riss ca , ade . d Sda Aspirina me daze mici si prizounio'previvehowbazele eit S -paciee & It prezie - Ca t ou rese seazut eare to retreimportanH/ ale rol frombrafeler are dat hemoragin , in acaste situati se indica terapiecitareduetoare st . reducered ur . O housocit , pana la reiterea · I estailor .monit -Pacisntri en rise scant o ind. A hat, actiengregatE ↑ &He prizo uniea? iar er case on Rise e .U . Se reau. tat , anting2 - Grupa de riso soari fara's& L- , hat . auf estv , CALr+, Jakz/MP autiague& & suf ·isset iar daca prez . rise case adoga trap aleitoreductors- Grupo of size crescent are ind, de trapis eitoreduct.1 - caz de istoria de houboze art se reow . fergrisS outaregauta inaa d complicati tombotiveI ↓ rist . Hed . Stardustoon inTantiwag vlan Inter L : Hidroxivra , eraul Metaplasia micloida en mielofibrota Tablou Clinic : - cel mai constant seen clinic est splenomegaliairge in timp la dimeupar impresol / care to ,of envi em abdomenl ; se consideraeaunzai occupa integ spline ereste lau Simptomatologies - 14 dis pacienti ·met ·sitomaticS ↑- 20% dir pacien prezintos siptome respecifice : asteir , fizica, harspirati abuudeute, sader pouderalogoate S - avencia det & astruir fisica , fatigabilitate , awebli,fidispues , palpita & I def . Senzatie de restate ,in- Spleromedalia ,Satictate Y 9vipocadral string precoce , constipatio;aretle here revent tainf splenie +T in MF , se maritseire dure intensa & , ta I elipeacl sta · apa e es Susa , iradiato in Umaif ↑ , accentuat o epost si resp auple stasabys wear obsilitate . - in] S de epuizar'wedelara, cand fibrazoafa medelora' este extensiv si apar eitopenile severe,I apar manifestarite bewoagjar Exautmul elima : - avemie - plz . Reportesplanaregalies Diagnostic positio : elivia la un ↑ alie , - respicionat pacient en splenoneg en sect caracteristic al HLG si este zee as couf - pe baza criteritor de diage+ asocitara en atipiCriteri major : -proliferawea h div -dif. de la I ↓ nucleo-citopletmaticla a pants, eu capos i insof I altera ( tode↓, mercleipolitibal ↑ S fibraza reticlinica MI fibrozei.Zu Obs. / retculivice , anomalike wegacarizatore se insotise de crestera celularitati wiederlan ear grauloctares feweutpoliyseaderra - excluderaPepapoizlazi wieloprilrouice ouz . anomalies Jaka san a alter-I marker cloual/ASX1 , EZH2 , TETz! san excluderra fibrazeimedularereactive Criteri mirrore : Anemia Neatibuito' enterior unei alte aurbiditati - Cenwattoza 11000 /nms - tabloo leucocritoblastic - LDH A - splenomegalie important Diag Iecerdempout lolecriteri Principi de Hastament : -este o boala ineurabila - ringuna terapie en intentie errative est allotaupYwed . care se indica unmai in casuri select &,Qua es vostic insalt19 proghiv urwarst- hat & polic ameliorate sirupt. reducerra riscule de Lausfowars in LA P . pacienti en rise secient san intermedied : - cei asiptomatic me an indicatio o tratament; Se upraved Graza Clinic ri biologic la sluvi - daca saeenfi acuza I & nipt! de sol. acemic en est confirmat biolog ic , se crectaza eventualele exeluoabficite , be S ↳ mecanimal imn i' se adve. critoportina & cand soomU/mL) , eienta se apreciate la sluvi& - deca critopoietima >500mU/mL se porteader . Darazal cu anrmie severa necesita hatamentpacient eu wasa critocitola - scienti ↳ simpl . constitutionate & an indicati delkrapist pecifical en inhibitor de JakzkilazaSICruxolitinib Jakavil Principate optiveprapeutice met : - Hedry , nea I ef in reduces vol. slivei - Interferonl , dimineaza fibrota 9wedlara - Juk . de Jakzkinaza' (akavi) Pacienti en rise cresent If intermediar : - timerichiavi al indicatin o allot ansplant medelar [ - sacienti I ↑ simpotinuatefazkie- corticoterapiaGedifica - alopisivolal se end . pt. conectaria val · Cresenteale ac . unia - pacienti un enemie severa si necesitati Ihausfizionale resente , necesits tatamient chelator de fier en Dejeravirax (Exjade)