TROMBOTIČNA MIKROANGIOPATIJA pogled hematologa. Polona Novak, Samo Zver Zreče, april 2015
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1 TROMBOTIČNA MIKROANGIOPATIJA pogled hematologa Polona Novak, Samo Zver Zreče, april 2015
2 Klinični primer 64-letni pacient Zbolel s splošno slabim počutjem so mu začele dreveneti roke in noge je težko prijel kozarec, težje je govoril znaki poškodbe glave, padca se ne spomni, postane nemiren SB MS CT glave bp Infekcijski oddelek SB MS: LP bp, Tr 14, Hb 100, MCV 82, Tr 14, retikulociti 7 %, abs 235, bili 35/10, seč 9, krea 113, CRP pod 5, LDH 15 Svojci: prekomerno uživanje alhohola, krvava driska
3 Diferencialna diagnoza sepsa + sindrom DIK + krvavitev (testi hemostaze) imunska trombocitopenija + krvavitev (LDH, hemoliza) megaloblastna anemija (retikulociti) Evansov sindrom: AIHA + ITP (Coombs test) mielodisplastični sindrom bolezni krvi z bicitopenijo (PKM) anemija in trombocitopenija v sklopu napredovale bolezni jeter HIT s krvavitvijo PNH: paroksizmalna nočna hemoglobinurija TMA: TTP, HUS, ahus Hemoragična mrzlica
4 Trombotična mikroangiopatija Skupina stanj za katero je značina mikrovaskularna tromboza, ki vodi v trombocitopenijo in hemolitično anemijo zaradi fragmentacije eritrocitov Je patofiziološka osnova TTP, HUS, ahus, maligne hipertenzije, skleroderme, AFS, SLE, preeklampsije, nefropatije po obsevanju, TMA po presaditvi, HIV, diseminiranega karcinoma Napredek v razumevanju patogeneze namesto klinične slike (prekrivanje) etiologija Scully M J. Guidelines on the diagnosis and management of thrombotic thromboccytopenic purpura and other thrombotic microangiopathies: 2012.
5 George JN, Nester CM. N Engl J Med 2014;371: Časovnica TMA
6 Trombotična trombocitopenična purpura Incidenca 3-6/milijon TMA pri kateri je akutna ledvična odpoved zelo redka (registri!) pentada le v 5-10%, 1/3 nima nevrološke prizdetosti Kongenitalna in pridobljena (idiopatska, sekundarna-hiv, nosečnost, zdravila, transplantacija, malignomi, pankreatitis-adamts!) Diagnoza TTP-klinični in laboratorijski znaki: plazmafereza (preživetje iz 10 na 80%) Tveganje za relaps 20-50% pri pridobljeni TTP Dolgoročno-večja prevalenca kognitivnih motenj, depresije, SLE, AH in večja smrtnost ADAMTS 13 (a disintegrin and metalloprotease with eight thrombospondin-1-like domains): aktivnost, antigen in protitelesa v pomoč pri potrditvi diagnoze
7 Patofiziologija TTP Moake JL. Thrombotic microangiopathies. NEJM 2002
8 HUS Triada: mikroangiopatska hemolitična anemija, trombocitopenija, akutna ledvična odpoved D+ (E.coli O157:H7 idr.- šiga toksin, verotoksin) in D- ( S. pneumoniae) Koprokultura+serologija in/ali PCR V 33% primerov prisotna nevrološka simptomatika Podporno zdravljenje (antibiotiki!) SYN-SORB Pk, monoklonska protitelesa proti Stx
9 PATOFIZIOLOGIJA HUS
10 ATIPIČNI HUS Zelo redka bolezen Akutna ledvična odpoved in AH zelo pogosta Disregulacija komplementa alternativne poti, ki vodi v TMA Poškodba endotelija in disregulacija kompelemta na površini Tr Plazmafereze, % relaps po transplantaciji ledvice Kljub plazmaferezi visok odstotek končne ledvične odpovedi (50%) umrljivost 25% Ekulizumab, presaditev jeter zelo redko Campistol et al. Update for atypical haemolytic uraemic sindrome: 2013
11 Patofiziologija ahus
12 Table I. ahus diagnostic criteria. Exclusion Shiga toxin-associated HUS Secondary causes Drugs Infection (HIV, Streptococcus pneumonia) Transplantation (bone marrow, liver, lung, cardiac but not denovo renal) Cobalamin deficiency Systemic lupus erythematosus Antiphospholipid syndrome Syndrome Scleroderma ADAMTS13 antibodies or deficiency Inclusion Renal biopsy showing a TMA And/or The classic triad of microangiopathic haemolytic anaemia, thrombocytopenia, renal failure
13 ahus terapevtske možnosti Pred plazmaferezami: ELISA za protitelesa proti faktorju H Nivo faktorjev I, B in H v krvi imunološki testi IMI (kvantitativno, kvalitativno več) Nivoji C3, C4 IMI CD46 mutacije pretočna citometrija Funkcionalno testiranje: faktor H
14 Genetsko testiranje mutacij (več mesecev) dokončna diagnoza Nizek nivo C3 v serumu in normalen C4 kaže na porabo in aktivacijo alternativne poti komplementa pri CFH, CFI, CD46, (C3 je v 50% normalen ) C3 depoziti v glomerulih in arteriolah kažejo na aktivacijo komplementa
15 TMA Imunska reakcija po zdravilih (kinin)- edina substanca, ki ima potrjeno drug-dependent antibodies (kjer so protitelesa dokumentirana) Druga zdravila: kvetiapin, gemcitabin (edina zdravila, pri katerih se je TMA pojavila ob ponovnem izpostavljanju) Akutna ledvična odpoved, lahko po letih po 1. izpostavitvi antigenu (tudi pijače) TOKSIČNOST ZDRAVIL Kemoterapija, imunosupresivi METABOLISM-MEDIATED (kobalamin C) KOAGULATION MEDIATED (trombomodulin )
16 Diferencialna diagnoza TMA Campistol et al. Update for atypical haemolytic uraemic sindrome: 2013
17 Algoritem zdravljenja Laboratorijski testi PRED plazmaferezo: HIV, HBV, HCV, presejalni avtoimunski testi, ščitnični hormoni, beta hcg pri ženskah, hemogram, DKS in krvni razmaz, shizociti, haptoglobin, testi hemostaze, retikulociti, elektroliti, troponin, jetrni testi, kalcij, LDH, TSH, urin, direktni antiglobulinski test, krvna skupina, ADAMTS 13 (antigen, protitelesa, aktivnost), koprokultura, serologija na šiga toksin ter tumorski markerji.
18 Odvzem krvi za ADAMTS 13 Odvzem vzorcev plazme za preiskavo določitve ADAMTS 13 aktivnosti, ADAMTS 13 antigen, protitelesa ADAMTS 13 pri sumu na TMA: Kri, odvzeta v 4,5 ml epruveto z natrijevim (Na) citratom (svetlo modri zamašek, 0,105-0,109 M natrijev citrat). Dostava v laboratorij v 2 urah po odvzemu ali vzorec shranimo: Kri centrifugiramo pri 1942 x g (3000 obratov/min), 15 minut pri +4ºC ali sobni temperaturi. Plazmo odpipetiramo v plastično epruveto z zamaškom in pri tem pazimo, da ne odpipetiramo eritrocitov. Plazmo ponovno centrifugiramo pri 2600 x g (3500 obratov/min) v centrifugi, 15 minut pri 4ºC ali sobni temperaturi. Vzorec zamrznemo - po 0,5 ml plazme (supernatant) prenesemo v novo plastično epruveto in vzorec zamrznemo pri 20ºC. Za daljše obdobje shranimo vzorce pri 80ºC. Vzorce v našem laboratoriju na Polikliniki, I.nadstropje E hodnik, sprejemamo vsak delovni dan od 7 30 do
19 Plazmaferezo je potrebno pričeti najkasneje 4-8 h po postavitvi suma na TTP. V primeru izključitve TTP prenehamo s PF, sicer nadaljujemo do odgovora. Pričnemo s kortikosteroidi, če se stanje kljub temu slabša PF 2x/dan, visoke doze kortikosteroidov, Rituximab. V primeru Tr nad dni, lahko prenehamo s PF in nadaljujemo s kortikosteroidi- Ob eksacerbaciji nadaljujemo s PF in Rituximabom. Če so Tr stabilni 1-2 tedna, nižamo kortikosteroide. Remisija: Tr normalni 30 dni po zadnji PF. Pri relapsu TTP zdravimo z rituksimabom ali ciklosporinom A. Algoritem zdravljenja
20 Cataland S R, and Wu H M Blood 2014;123: Diagnostični TMA ALGORITEM.
21 Klinični primer epi napad, premeščen v SIIT, intubiran, sediran, povišan TnI -ADAMTS aktivnost 0% -Od plazmafereze -Klinična slika boljša -Ob odpustu aktivnost ADAMTS upada
22 Zdravljenje z rituximabom pri TTP Indikacija Priporočilo Razlaga Začetno zdravljenje TTP Zdravljenje refraktarne TTP Zdravljenje TTP v remisiji 2 C Podatki kažejo, da aplikacija skrajša hospitalizacijo, manj je relapsov 1 C V primeru, če ni odziva na PF in kortikosteroide 1 C Proti aplikaciji
23 Do l Določen ADAMTS13 antigen in protitelesa (ELISA) v obdobju (140) diagnoze Izključili DIK, sepse, HIT, anemije, porabne trombocitopenije, AIHA, 60 bolnikov z diagozo TTP/HUS: 48 TTP in 12 HUS
24 Klinična slika TTP in HUS TTP (48) HUS (12) Starost 54 (24-85) 47 (30-86) Spol 30 ž, 18 m 9 ž, 3 m Nevrološka simptomatika 20 (42%) 3 (25%) Vročina 11 (23) 3 (25%) Driska 7 (15%) 8 (6x izolirana e.coli O157H7, VTX2) 66% Bruhanje 7 (21%) 6 (50%)
25 TTP HUS ADAMTS13 antigen ( ng/ml) 24/48 (50%) znižan 4/12 (33%) znižan ADAMTS13 protitelesa (poz. nad 9,6 E/mL) 32/48 (66%) povišana, 16 normalna* 3/8 (37,5%), 5 normalna * Od l le 15% odvzemov pred plazmaferezo
26 Št. Bolnikov 9 (2 m, 7 ž) Aktivnost ADAMTS 13 0 ADAMTS protitelesa Nad 60 pri 6, nad 50 pri 3 Št. trombocitov 29 (9-67) Hemoglobin 90 (66-119) Shizociti 6 % (1-11) LDH 15 (3-20) Bilirubin celokupni 46 (8-70) Bilirubin direktni 13 (3-23) Sečnina 7 (3-10) Kreatinin 78 (56-107) Nevrološki simptomi 2 Vročina 3 Driske 2 Relapsi 4 (2 v enem letu) Splenektomija 1 Rituksimab 2
27 Primerjava ADAMTS13 pri idiopatski in sekundarni TTP ADAMTS13 antigen ( ng/ml) ADAMTS13 protitelesa (poz. nad 9,6 E/mL) TTP idiopatska (38) TTP sekundarna (10) Relaps 6 0 Idiopatična TTP 38 (imunska, avtoimunska, zdravila: tiklopidin, klopidogrel) Sekundarna TTP 10 (pečatnocelični Ca želodca, AML po nesorodni alo PKM, SLE, MALT limfom, maligna hipertenzija, CMV, po fetocidu, adenokarcinom želodca, influenca B)
28 Zaključek V zadnjem desetletju izjemen napredek v razumevanju patofizioloških mehanizmov TMA Trenutno vodilo v terapiji po sklepu vseh mednarodnih priporočil je: hitra in empirična plazmafereza Vključevanje pacientov s TMA v registre in klinične raziskave je potrebno za bolj z dokazi podprto medicino in premik k bolj specifični in učinkoviti terapiji Še veliko raziskav in kliničnega dela bo potrebnega tudi pri nas za usmerjeno, učinkovito, dostopno in varno zdravljenje
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30 ? HVALA ZA POZORNOST
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