Določanje klona PNH. Darja Žontar, Specializirani laboratorij KOH, UKC Ljubljana

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1 Določanje klona PNH Darja Žontar, Specializirani laboratorij KOH, UKC Ljubljana

2 Paroksizmalna nočna hemoglobinurija Edina pridobljena hemolitična anemija zaradi napake v eritrocitni membrani Klonska bolezen: eritroci, trombociti in granulociti izvirajo iz iste, z mutacijo spremenjene matične celice; mutacija X-vezanega PIG-A gena povzroči delno ali popolno pomanjkanje proteinov, ki so vezani na celično membrano z glikozilfosfatidilinozitolnim sidrom (GPI) Povečana dovzetnost za litični učinek koplementa-razpad eritrocitov Dve populaciji eritrocitov-normalna in z napako v membrani Stopnja hemolize odvisna od razmerja med njima

3 PNHEU11002/NECMEA-eng01 The Defect in PNH with the courtesy of Alexion Pharmaceuticals The Somatic Mutation of the PIG A gene prevents all GPI anchored proteins from binding to cell surface CD59 Forms a defensive shield for RBCs from complement-mediated lysis1 Inhibits the assembly of the membrane attack complex CD55 Prevents formation and augments instability of the C3 convertases, attenuating the complement cascade2 CD59 GPI-anchor CD55 GPI - Glycosylphosphatidylinositol; PIG A - phosphatidylinositol glycan anchor biosynthesis A 1. Johnson RJ et al. J Clin Pathol: Mol Pathol 2002;55: Brodsky R. Paroxysmal Nocturnal Hemoglobinuria. In: Hematology - Basic Principles and Practices. 4th ed. R Hoffman; EJ Benz; S Shattil et al. eds. Philadelphia, PA: Elsevier Churchill Livingstone; 2005; pp

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5 Laboratorijske preiskave za dokaz PNH klona Hamov test (kisli serum test), Sugar-water test Pretočna citometrija-monoklonska protitelesa Prva: CD55 DAF (decay acceleration factor) CD59-MIRL (membrane inhibitor of reactive lysis) FLAER (Fluorescent Labeled AERolysin) Inaktiviran bakterijski toksin se veže direktno na GPI-sidra ; samo na granulocitih in monocitih

6 Antibodies to transmembrane proteins are used to gate on various WBC populations. PNH clones are then detected with antibodies to GPI-proteins (and FLAER) GPI-AP Antibodies like CD55, CD59, CD24, CD16, CD66b and CD14 bind indirectly to the GPI anchor FLAER FLAER binds directly to the GPI anchor Kumar: Robbins and Cotran: Pathologic Basis of Disease, 7th ed. Fig with the courtesy of Alexion Pharmaceuticals 6

7 Ugotavljaje prisotnosti PNH klona s pretočno citometrijo Granulociti-najzanesljivejši za dokaz PNH klona Monociti-potrjujejo PNH klon na granulocitih (klon pogosto večji kot na granulocitih; natančnost manjša zaradi manjšega števila) Limfociti-niso primerni za testiranje zaradi drugačne življenske dobe; le za notranjo kontrolo protiteles Eritrociti-primerni za oceno velikosti klona, ki pa lahko variira zaradi hemolize ali transfuzije 7

8 Izbor protiteles za PNH analize Lab-specific PNH Panels with GPI-linked antibodies and/or FLAER CD55 CD59 CD16 CD66b CD24 CD14 FLAER RBC - ++ NA NA NA NA NA Gran? NA ++ Monos? - NA NA NA Very good Signal/noise ratio + Moderate signal/noise ratio - not recommended Staining with FLAER has become the method of choice as it appears to be a more sensitive and specific method to detect PNH clones in WBCs 1. Borowitz MJ et al. for the International Clinical Cytometry Society. Cytometry B Clin Cytom 2010;78B: with the courtesy of Alexion Pharmaceuticals 8

9 Priprava vzorcev za analizo 2 epruveti za 4-barvno analizo na nevtrofilnih granulocitih in monocitih 100 µl dobro premešane venske krvi inkubiramo s protitelesi minut v temi pri sobni T. Eritrocite liziramo z Immunoprep reagentom, dvakrat speremo s PBS in resuspendiramo v 0,5 ml PBS. Nevtrofilne granulocite zamejimo s FS/SS/CD45/CD15 in monocite s FS/SS/CD45/CD64 ), analiziramo granulocitov (ciljna občutljivost 0.1%) in določimo PNH klone s FLAER v kombinaciji s CD24 na granulocitih in CD14 na monocitih. Kot klon opredelimo samo dvojno negativno populacijo. 1 epruveta za 2-barvno analizo na eritrocitih: 50 µl predhodno razredčene venske krvi (10 µl polne krvi + 1 ml PBS) inkubiramo s predhodno razredčeno in titrirano kombinacijo protiteles CD235a-FITC in CD59-PE za 30 minut v temi pri sobni T, dvakrat speremo s PBS in resuspendiramo v 0,5 ml PBS Eritrocite zamejimo z logfs/logss/cd235a in analiziramo celic (ciljna občutljivost 0.1%). PNH klon opredelimo kot tip III (povsem negativen CD59) ali tip II (delno negativen CD59).

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11 PNH klon na nevtrofilnih granulocitih

12 PNH klon na monocitih

13 PNH klon na nevtrofilnih granulocitih

14 PNH klon na monocitih

15 Vzorec s pomikom v levo v DKS

16 Določanje PNH klona na eritrocitih CD59:izražen v visoki stopnji, označuje tip I, II in III eritrocitov CD55: slabše izražen, pogosto ne označuje tipa II eritrocitov (uporaba se opušča) Anti-Glycophorin A (CD235a): specifičen za označevanje eritroctov Določanje v polni krvi z predhodno razredčenimi protitelesi, da se izognemo agregatom

17 August_12_2010Gbl 17

18 PNHEU11002/NECMEA-eng01 Count Testing for PNH in Red Blood Cells - CD59 plus CD235a Type III 0.0% Type II 0.0% Type I 100% Normal RBCs with normal CD59 expression (Type I cells) CD59-PE Type III 6% Type II 0.1% Type I 93.9% PNH clone with complete CD59 deficiency (Type III cells) CD59-PE CD59-PE Gating on GPA+ RBCs Type III 11.9% Type II 71.3% Type I 16.8% PNH clone with complete CD59 deficiency (Type III cells) and partial CD59 deficiency (Type II cells) CD59-PE GPA: glycophorin A 1. Borowitz MJ et al. for the International Clinical Cytometry Society. Cytometry B Clin Cytom. 2010;78B:

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20 Različni fenotipi eritrocitov pri PNH SJ Richards et al, Cytometry 42: , 2000

21 PNH eritrociti tipa II in III

22 PNH eritrociti tipa II in III

23 PNHEU11002/NECMEA-eng01 CD24-Granulocytes Important to Monitor Granulocytes and RBCs Over Time 1 Sept 08 Dec 08 Mar 09 May 09 Granulocytes: 3.8% FLAER-GPI Anchor Marker Granulocytes: 7.6% Granulocytes: 14.2% Granulocytes: 23.3% RBCs: 0.8% RBCs : 1.6% RBCs : 1.8% RBCs : 2.4% CD59 GPI Anchor Protein 3 months 6 months 9 months PNH clone expanded in < 1 year 1. Adapted with permission from Dahl-Chase Diagnostic Services. 23

24 Smernice I Guidelines for the diagnosis and monitoring of Paroxysmal Nocturnal Hemoglobinuria and related disorders by flow cytometry Borowitz MJ et al. Cytometry Part B 2010; 78: Poudariti klinični pomen določanja PNH klona s pretočno citometrijo Določiti klinične indikacije za PNH analize Izbrati enoten pristop k PNH analizam s priporočenim izborom protiteles za določanje na granulocitih, monocitih in eritrocitih

25 Smernice II Practical Guidelines for the High-Sensitivity Detection and Monitoring of Paroxysmal Nocturnal Hemoglobinuria Clones by Flow Cytometry Sutherland DR, Keeney M, Illingworth A. Cytometry Part B 2012; 82B: Dokument, ki dopolnjuje smernice ICCS iz leta 2010 Zagotavlja dragocene informacije o GPI vezanih protitelesih in linijsko specifičnih protitelesih za PNH testiranje in priporoča specifične koktajle protiteles za optimalno analizo Zagotavlja zgoščene praktične protokole za visoko občutljivo metodo odkrivanja PNH eritrocitov, nevtrofilnih granulocitov in monocitov

26 Study- part of Alexion s PNH Dx network approach 1. Lab training 2. Normal blood testing 3. PNH blood testing 4. Lab certification FC training (expert) Discuss PNH panel required (expert) Provide PNH testing panel (Alexion) PNH testing with normal blood (lab) Validation of testing results (expert) PNH testing of pathological samples Validation of positive PNH testing results (expert) Interlaboratory testing of PNH samples Validation of interlaboratory testing results (expert) Lab certification for PNH testing (expert)

27 Participants IHBT-Prague: reference center FH-Plzen: center 1 FH-Brno: center 2 IO-Bratislava: center 3 UMC-Ljubljana: center 4 Andrea Illingworth, Dahl-Chase Diagnostic Services, USA: on-site PNH testing workshop following ICCS guidelines and support Alexion Pharma International: coordination and financial support

28 Ponovljivost: PNH kloni na eritrocitih tipa III in tipov II in III skupaj 5x ponovljeno pipetiranje in označevanje v vsakem vzorcu Dr. Iuri Marinov, UHKT, Praga

29 Ponovljivost : PNH kloni na nevtrofilnih granulocitih in monocitih 5x ponovljeno pipetiranje in označevanje v vsakem vzorcu Dr. Iuri Marinov, UHKT, Praga

30 Natančnost: PNH kloni na eritrocitih tipa III in tipih II+III skupaj Dr. Iuri Marinov, UHKT, Praga

31 Natančnost : PNH kloni na nevtrofilnih granulocitih in monocitih Dr. Iuri Marinov, UKHT, Praga

32 Podajanje rezultatov > 1% = PNH klon 0,1% - 1% = mali klon ali populacija PNH celic < 0,1% = posamezne celice z znižanimi GPI označevalci 32

33 Zaključki Metoda določanja PNH klona s pretočno citometrijo je zlati standard Določanje PNH klona na nevtrofilnih granulocitih je najboljša metoda za opredelitev velikosti PNH klona Določanje PNH klona na eritrocitih ni zadostno za oceno velikosti klona, je pa koristna informacija FLAER je verjetno najboljši reagent za dokaz PNH klona na levkocitih

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