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Boli mieloproliferative cronice Flashcards

1
Q

Baza patologiei MC

A

mutatii la nivelul genelor care codifica TIROZIN KINAZE

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2
Q

Mutatie LMC

A

t(9,22) => cr Philadelphia (22 mic) cu proteina mutanta BCR-ABL cu activitate tirozin-kinazica crescuta

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3
Q

LMC - Hlg

A

Leucocitoza >100.000
Anemie moderata
Trombocitoza >1.000.000

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4
Q

LMC - frotiu sg perif

A

Neutrofilie, bazofilie

Deviere la stanga

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5
Q

LMC - BOM

A

hiperplazia seriei G
deviere la stanga
bazofilie

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6
Q

LMC - scoruri de risc faza cronica

A

scor SOKAL

scor HASFORD

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7
Q

LMC - scor Sokal

A

Varsta, PLT, splina, blasti in sg perif

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8
Q

LMC - scor Hasford

A

Varsta, PLT, splina, blasti, Eo, Ba in sg perif

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9
Q

LMC - faza accelerata

A 
Splenomegalie +/- leucocitoza sub tratament
Bazofilie >20%
Trombocitopenie nelegata de tratament
Trombocitoza refractara la tratament
Cresterea proc de blasti (10-19%)
Anomalii citogenetice noi
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10
Q

LMC - faza blastica Hlg + frotiu

A

anemie, trombocitopenie, BLASTI >20%

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11
Q

LMC faza cronica - tratament

A

INHIBITORI DE TIROZIN-KINAZE - imatinib mesilat, dasatinib, nilotinib, bosutinib, ponatinib
TRANSPLANT ALOGENIC DE CEL STEM - posibilitate de vindecare

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12
Q

LMC faza blastica - tratament

A

Chimioterapie combinata - scheme tip LAM/LAL
Transplant alogenic de cel stem
ITK - dasatinib

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13
Q

POLICITEMIA VERA - mutatie

A

V617F JAK2 (gena care codifica Janus kinaza 2) => activ tirozin-kinazica crescuta

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14
Q

PV - Hlg

A

RBC, HCT, Hb crescute
EPO N/ scazuta
leucocitoza <50.000 + deviere la stanga
trombocitoza >1.000.000

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15
Q

PV - criterii majore de dg

A

Hb > 16.5g/dl la barbati si >16 g/dl la femei
Mieloproliferare triliniara -panmieloza
mutatia JAK2

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16
Q

PV - criterii minore de dg

A

nivele scazute de EPO

17
Q

PV - algoritm diagnostic

A

3M/ primele 2M + m

18
Q

PV - tratament

A 
emisii sanguine
medicatie antiplachetara
HIDROXIUREE - I: >50 ani, manif trombotice, PLT >1,5 mil
antihistaminice
alfaIFN
inhib specif JAK2
19
Q

PV - evolutie, complicatii

A

prognostic bun
complicatii trombotice
evolutie spre LAM secundara, MMM

20
Q

TROMBOCITEMIA ESENTIALA - mutatii

A

V617F JAK2
CALR
MPL

21
Q

TE - sdr hemoragipar patogeneza

A

Deficit functional al PS

Deficit cantitativ si calitativ de FvW - prin fixarea FvW la PS in exces, liza multimerilor de FvW

22
Q

TE - Hlg

A

PLT >450.000, uneori >1 mil
leucocitoza moderata
FARA policitemie

23
Q

TE - criterii dg majore

A

PLT > 450.000
BOM - prolif exagerata Mgk, Mgk mari, hiperlobulate
Excluderea altor BMC (LMV, PV, MMM, SMD)
V617F JAK2, CALR, MPL

24
Q

TE - criterii dg minore

A

1 marker clonal/ absenta trombocitozei reactive

25
Q

TE - algoritm dg

A

4M/ 1+2+3+m

26
Q

TE - evolutie

A

LAM 7/4/bifenotipica

mielofibroza

27
Q

TE - tratament risc scazut

A
  • varsta < 50 ani, fara tromboze –> eventual ASS
28
Q

TE - tratament risc crescut

A
  • varsta >50 ani, istoric tromboze, PLT >1,5 mil –> trat CITOREDUCTOR - hidroxiuree, anagrelid
29
Q

TE - tratament sarcina

A

oprire trat citoreductor (hidroxiuree, anagrelid)
TOATE gravidele - ASS
APP de tromboze, PLT >1-1,5 mil, trombofilie, risc CV - citoreductie cu IFN
APP de complicatii fetale - ENOXAPARINA

30
Q

MMM - caracteristici

A

Mielofibroza
Hepatosplenomegalie
Citopenii progresive

31
Q

MMM - mutatii

A

V617F JAK2
CALR
MPL

32
Q

MMM - patogeneza

A

Mgk displazice –> sint de citokine aberante => PDGF -> + fibroblastii => FIBROZA

33
Q

MMM - Hlg

A 
anemie
DACRIOCITE
\+/- AHAI
leucocitoza + dev la stg
dev la stg + eritroblasti = TABLOU LEUCOERITROBLASTIC
trombocitopenie
34
Q

MMM - criterii majore dg

A

Prolif Mgk atipica + MF
excluderea altor neo mieloide
prez mutatiilor/ marker de clonalitate/ excluderea mielofibrozei reactive

35
Q

MMM - criterii minore dg

A 
tablou leucoeritroblastic
LDH crescut
anemie
splenomegalie
L> 11.000
36
Q

MMM - algoritm dg

A

3 M + minim 1 m

37
Q

MMM - tratament

A 
anemie severa - transfuzii
AHAI - corticoizi
splenomegalie - HXU/IFN
splenomegalie giganta, dureroasa - splenectomie
post PV si TE - inhib JAK2 (ruxolitinib)
allotransplant cel stem
38
Q

MMM - prognostic

A

5-6 ani

acutizare in 10-20% din cazuri

Hematology (12 decks)

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