sindroamele mieloproliferative cronice studenti
DESCRIPTION
hemato fundeniTRANSCRIPT
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SINDROAMELE MIELOPROLIFERATIVE
CRONICE
Conferenţiar Dr. DANIEL CORIU
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SINDROAME LIMFOPROLIFERATIVE
SINDROAME MIELOPROLIFERATIVE
Acute:-LAM
Cronice - PV- TE-_LGC- MMM
Acute:-LAL
Cronice - LLC- HCL- LNH - BH - BW- MM
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SINDROAMELE MIELOPROLIFERATIVE CRONICE:
Leucemia Mieloida Cronica (LMC) Policitemia Vera (PV) Metaplazia Mieloida cu Mielofibroza (MMM) Trombocitemia Esentiala (TE)
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LEUCEMIA MIELOIDA CRONICA
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LMC: Definitie
• Boala mieloproliferativa clonala a celulei stem pluripotente proliferare, apoptoza– Marker citogenetic: Cromozom Philadelphia (Ph)– Marker Molecular: oncogena Bcr-Abl
• 7% - 15% din totalul leucemiilor la adult• Incidenta: 1-2 cazuri noi / 100 000 loc / an• Varsta mediana la diagnostic 55 ani (20% - 30% din
pacienti ≥ 60 ani)
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LMC: Patogeneza
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Cromozom Philadelphia Cromozom Philadelphia
p210BCR-ABL
p190BCR-ABL
p230BCR-ABL
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Hematology 2003;2003:597-618
Structure of the p210Bcr-Abl fusion protein and mechanisms of leukemogenesis
BCR-ABL codifica proteina p210 cu activitate tirozin- kinazicaBCR-ABL codifica proteina p210 cu activitate tirozin- kinazica
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Enzima care transfera gruparea fosfat de la ATP la un tirozina din structura unei proteine.
Fosforilarea proteinelor de catre kinaze este un mecanism important in transductia semnalului pentru reglarea activitatii enzimatice.
Tirozin kinazele sunt impartite in doua grupe; kinaze citoplasmatice si kinaze membranare (receptori).
Ce sunt ‘tyrosine kinazele’?
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Src: Activitate in CML
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Diagnosticul leucemiei mieloide cronice
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Diagnostic clinic
10% dintre pacienţi: nu prezintă simptome, iar boala este diagnosticată accidental
La 10% dintre pacienţi, boala apare în faza blastică (stadiul terminal)
80% au - Simptome generale: oboseală, anorexie, scădere în greutate, transpiraţii şi - Simptome specifice
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Simptome legate de mărirea de volum a splinei şi ficatului:
- balonări,- dureri abdominale, - senzaţie de apăsare în partea stângă a abdomenului
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LMC: diagnostic de laborator
• Hemograma
• FAL (Fosfataza Alcalina Leucocitara)
• Medulograma
• Examen citogenetidc
• Examen Molecular
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LMC: evolutie
• Faza Cronica: durata mediana 4-5 ani
• Faza Accelerata: durata mediana 6- 9 luni
• Faza Blastica (criza blastica): supravietuire mediana 3-6 luni
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LMC faza cronica: criterii de diagnostic
• Hemograma:
- leucocitoza importanta cu neutrofilie
- deviere la stanga a formulei leucocitare (curba de distributie cu doua varfuri la Mi / Mt, Ns/ S)
- Bazofilie 5-10%
- Monocite sub 3%
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Dg diferential: LMC faza cronica vs reactie leucemoida
• Reactia leucemoida:
- leucocitoza importanta cu neutrofilie (rar peste 50 000/mmc)
- deviere la stanga a formulei leucocitare, dar fara Mbl
- Bazofilie absenta
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Dg diferential: LMC faza cronica vs reactie leucemoida
• Tabloul clinic:
- cancer
- inflamatii
- sepsis
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LMC: evolutie
• Faza Cronica: blasti 0%
• Faza Accelerata: absenta raspunsului la terapie, febra, splenomegalie, anemie, bazofilie, anomalii cromozomiale aditionale
blasti peste 10%
• Faza Blastica (criza blastica):
blasti peste 30%
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LMC: dg citogenetic
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BCR/ABL t(9;22)(q34;q11)
FISHFluorescent In Situ Hybridisation
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CML: CML: Investigare molecularaInvestigare moleculara
Metodologia Metodologia Light CyclerLight Cycler
PCR CALITATIV RQ-PCRMultiplex Multiplex
PCRPCR
Numarul de copii Bcr-
Abl
Tipul de transcript Bcr-Abl
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IDENTIFICAREA TRANSCRIPTILOR IDENTIFICAREA TRANSCRIPTILOR BCR-ABL PRIN PCR CALITATIVBCR-ABL PRIN PCR CALITATIV
Multiplex PCR – probe migrate în gel de agaroză 2%. Imagine captată cu cameră foto digitală (sistem BioCaptTM).
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IDENTIFICAREA TRANSCRIPTILOR IDENTIFICAREA TRANSCRIPTILOR BCR-ABL PRIN PCR CALITATIVBCR-ABL PRIN PCR CALITATIV
Multiplex PCR – probe migrate în gel de agaroză 2%. Imagine captată cu cameră foto digitală (sistem BioCaptTM).
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METODOLOGIA LightCyclerTM
FRET= Fluorescence Resonance Energy Transfer
•Rasmussen R. Quantification on the LightCycler Instrument. Indano Technilogy•http://www.idahotech.com/pdfs/LCU/HybridizationProbeChemistry.pdf
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CURBA STANDARD ABLCURBA STANDARD ABL
4000000 400000 40000 4000 400 copii
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CURBA STANDARD BCR-ABLCURBA STANDARD BCR-ABL
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J.C., ♂, 41 ani, dg-LMC-FC din 2007, din 2007 IM 400 mg
Centru de Hematologie si Transplant Medular Centru de Hematologie si Transplant Medular FundeniFundeni Laborator de Biologie Moleculara Laborator de Biologie Moleculara
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B.N., ♂, 54 ani, dg-LMC-FC din 2003, 2004-2008 IM 400-800 mg, oct 2008
P ac ient 2
1220
30
4448
30
0
10
20
30
40
50
60
1 2 3 4 5 6 19.06.2007 20.09.2008 14.02.2008 19.03.2008 17.04.2008 10.02 2009
%
BCR-ABL
Centru de Hematologie si Transplant Medular Centru de Hematologie si Transplant Medular FundeniFundeni Laborator de Biologie Moleculara Laborator de Biologie Moleculara
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LMC: tratamentLMC: tratament
agenti citostatici ( busulfan , hydroxiuree) Interferon Transplant medular Inhibitori de tirozin kinaze
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0.8
Survival in Early Chronic-Phase CML
Imatinib 276 141990-2000 960 3571982-1989 365 2661975-1981 132 1271965-1975 123 122
Year Total Dead1.0
0.6
0.4
0.2
0.0
Pro
po
rtio
n S
urv
ivin
g
Years From Referral
0 3 6 9 12 15
90%
The University of Texas M. D. Anderson Cancer Center database.
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STI 571; 2- phenylaminopyrimidina (IMATINIB, GLIVEEC)
Competitor de ATP in legarea specifica din situsul domeniului kinazic
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LMC: pana in 2000
• Boala fatala cu supravietuire mediana : 3-5 years• Transpant medular allogeneic curativ in 40% - 70% din
pacienti– mortalitate si toxicitate mare
• Interferon alfa ± cytarabine: – supravietuirea mediana : 6-7 ani– efecte adverse
• Alte optiuni: hydroxyurea, busulfan
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LMC: dupa 2000
• Boala indolenta cu prognostic excelent• Supravietuire la 5-ani : 90%
– supravietuire mediana: ≥ 25 ani
• Terapie de prima linie: imatinib mesylate• Terapie de a doua linia: inhibitori TK gen II,
transplant medular
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SINDROAMELE MIELOPROLIFERATIVE CRONICE:
Leucemia Mieloida Cronica (LMC) Policitemia Vera (PV) Metaplazia Mieloida cu Mielofibroza (MMM) Trombocitemia Esentiala (TE)
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POLICITEMIAVERA
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Incidenta
• Varsta mediana la diagnostic este 60 ani ( 20 – 85)
• B > F
• Incidenta 1,3 – 2,8 cazuri noi /100,000 / an
• Supravietuire PV netratat 6 – 18 luni
tratat > 10 ani
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Policitemia Vera: patogeneza
Mutatia punctiforma (JAK2 V617F)Tirozin kinaza constitutiv activata
Identificata in SMPC : PV (60-90%), TE si MMM (30-50%)
gena kinazei JAK2
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Policitemia Vera: patogeneza
Janus Kinase 2 (JAK2) => activitate tirozin kinazica si este implicata in transductia semnalului de la nivelul EPOR (erythropoietin receptor) la nucleu pentru expresia genica
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Cauza de deces in PV
• Tromboza - 29%
• Boli maligne hematologice : LAM sau SMD 23%
• Alte cancere 16%
• Hemoragie 7%
• Metaplazia mieloida cu mielofibroza - 3%
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PV: tablou clinic
• Prurit (in special dupa baie calda)• Eritromelalgia (tromboze in microcirculatie)• Tromboze ( ex sindr Budd Chiari….)• Eritroza tegumentelor• Splenomegalie
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PV: diagnostic de laborator
- Hemograma: Hb, Ht, RBC crescute. trombocitoza - Masa Eritrocitara Totala ( MET ) : ♂ 36 ml / kg ; ♀ 32 ml / kg - crescuta - Medulograma/ Biopsia osteomedulara : hiperplazie eritroida - Eritopoietina serica : scazuta - Sa O2 (arterial) > 92% - Colonii eritroide “ endogene” - Biologie moleculara: identificare mutatiei JAK 2
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EPO: N POLICITEMIA VERA
EPO: - POLIGLOBULII SECUNDARE:
A. Hipoxie tisulara generalizata: - incarcare redusa cu O2 a eritrocitelor: hipoxia de altitudine pneumopatii cronice – BPOC boli congenitale de cord met-Hb.emie; sulf-Hb.emie - sunt A-V: cord, pulmon, vase mari - eliberarea redusa de O2 catre tesuturi: Hb.patii eritrogene scaderea ereditara a 2.3-DPG carboxiHb. B. Hipoxie renala localizata: - hidronefroza - chisturi - stenoza arterei renale - tumori - transplant renal.
C. Productie autonoma de EPO sau de substante EPO-like: - hepatom - feocromocitom - fibrom uterin - hemangiom cerebelos.
- POLIGLOBULII RELATIVE Sdr. Gaisböck
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CRITERII PROPUSE PENTRU PV
(PJ Campbell, AR Green, 2005)
A1. Masa eritrocitara crescuta (> 25% fata de cea prezisa sau Ht >/= 60% la barbati si Ht >/= 56% la femei A2. Absenta cauzelor de eritrocitoza secundara (SaO2 normala; valori normale ale EPO)A3. Splenomegalie (palpare)A4. Prezenta mutatiei JAK2 V617F sau a altor anomalii citogenetice in celulele hematopoietice ( excluzand BCR-ABL)
B1. Trombocitoza ( Tr > 400.000/mmc)B2. Neutrofilie ( Neutrofile > 10 x 109/L; 12,5 x 109/L la fumatori)B3. Splenomegalie (investigatii imagistice)B4. Colonii eritroide endogene sau nivel al EPO scazut.
PV = A1 + A2 + PV = A1 + A2 + alt criteriu alt criteriu AA sau doua criterii sau doua criterii B B
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PV
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PV
Evolutie: faza asimptomatica faza eritroida activa faza de epuizare MMM post PV; LA
Complicatii: tromboembolii, hemoragii
DMS: 10 – 20 ani
Tratament: Flebotomii, antiagregant Mielosupresive : Hyo, BUS, 32P IFN Anagrelide – pt. trombocitoza
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SINDROAMELE MIELOPROLIFERATIVE CRONICE:
Leucemia Mieloida Cronica (LMC) Policitemia Vera (PV) Trombocitemia Esentiala (TE) Metaplazia Mieloida cu Mielofibroza (MMM)
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TROMBOCITEMIAESENTIALA
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TE
Clinic: -varsta: 50 – 70 ani, B/F = 1
- ~ 2/3 asimptomatici
- simptomatici:
- Tromboze:
a) manifestari vaso-oclusive ale microcirculatiei:
eritromelalgie
accidente ischemice cerebrale tranzitorii
b) tromboze arteriale – a. splenica – atrofie
c) tromboze venoase – v. splenica
- Hemoragii : a) trombopatie
b) tromboza + infarct hemoragic
- sangerari cutaneo-mucoase, post-operatorii,
post-ingestie de Aspirina
- splina +/-
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TE
Hematologie: Tr > 600.000 – 1.200.000/mmc L ( moderat) < 30.000/mmc, FL Mc+/- Ba Er normocromie hipocromie (sangerari) (Er. in semn de “tras la tinta”, corpi Jolly, Ebl = atrofie spenica) MO: hipercelulara, Mgk (gramezi), Fe +
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Trombocitozelor secundare
Hemoragii acute
Status post-splenectomie
Hiposplenism
Boli maligne
Boli inflamatorii cronice
Infectii cronice
Anemii hemolitice
Anemia feripriva
Medicamente
Rebound dupa refacerea unei trombocitopenii
Efort fizic
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CRITERII DE DG. PROPUSE PENTRU TE (PJ Campbell, AR Green, 2005)
A1. Nr. de trombocite > 600.000/mmc ( cel putin doua luni)
A2. Mutatie JAK2 castigata
B1. Fara cauze de trombocitoza reactiva (probe inflamatorii normale)
B2. Fara evidenta de deficit de fier ( HSM in Mcf. prezenta si VEM = N )
B3. Fara evidenta de PV ( Ht < media nivelului normal sau
MEr = N cu rezerve de Fier = N )
B4. Fara evidenta de LMC ( fara cromozom Ph sau rearanjare bcr/abl )
B5. Fara evidenta de mielofibroza ( fara fibroza colagenica si
</= 2 fibroza reticulinica - scara 0-4 )
B6. Fara evidenta de SMD (fara displazie semnificativa,
fara anomalii citogenetice sugestive de
SMD)
TE = A1 + A2 + B3 – 6 ( TE V617F + )TE = A1 + A2 + B3 – 6 ( TE V617F + )
A1 + B1 – 6 ( TE V617F - )A1 + B1 – 6 ( TE V617F - )
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TE
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TE
Evolutie: LA, MMM, PV
DMS: 10ani
Tratament: Trombocitofereza, Hyo, BUS, 32P IFN Anagrelide ! Aspirina !
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SINDROAMELE MIELOPROLIFERATIVE CRONICE:
Leucemia Mieloida Cronica (LMC) Policitemia Vera (PV) Trombocitemia Esentiala (TE) Metaplazia Mieloida cu Mielofibroza (MMM)
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METAPLAZIE MIELOIDACU MIELOFIBROZA
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MFI
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MMM: evolutieFaza de proliferare=> insuficienta medulara1=> 15 ani
MMM : tratamentSubstitutie MEAndrogeniInterferonAnagrelidaThalidomida