SINDROM MIELODISPLAZIC

Question 1

Definitie

Answer 1

citopenii
mad hipercelulara
semne de dishematopoieza multiliniara
!potential de progresie spre leucemie acuta (stare preleucemica)

Question 2

Etiopatogeneza

Answer 2

eveniment => INSTAB GENOMICA => ANOMALII CROMOSOMIALE -> defecte de prolif si maturare -> clona expansioneaza progr => noi anomalii cr => pot evol spre LA

Question 3

Anomalii cromosomiale

Answer 3

5q, 7q, 17q, 17p - genele factorilor de crestere hematopoietici

Question 4

Clinic

Answer 4

asimptomatic/ insuf medulara

LMMo Cr - hepatosplenomegalie, adenopatii

Question 5

Hlg + tablou sg perif

Answer 5

anemie - dubla pop de eritrocite (normo/macro), ret scazute, uneori eritroblasti, megaloblasti
neutropenie cu anomalii morfo
monocitoza cu anomalii functionale
limfopenie T si NK cu rasp anormal
trombocitopenie cu anomalii morfo si funct

Question 6

Medulograma

Answer 6

seria eritrocitara hiperplazica, megaloblasti, eritroblasti, vacuole, sideroblasti inelari
seria G - proc var de blasti
seria Mgk - microMgk cu nucl hipolob, hipogran

Question 7

BOM

Answer 7

mad normo/hipercel
loc atipica a prec imaturi
edem, exsudat, infiltrat perivascular -> hematopoieza iv, fibroza nod (fb retic)

Question 8

Anemie refractara

Answer 8

Sg perif - anemie, blasti <1%

MO - displazie eritroida, bl <5%, SI <15%

Question 9

ARSI (anemie refractara cu sideroblasti inelari)

Answer 9

Sg perif - anemie, bl <1%

MO - displazie eritroida, Bl <5%, SI >15%

Question 10

CRDML (citopenii refractare cu displazie multiliniara)

Answer 10

Sg perif - citopenii, bl <1%, Mo <1000

MO - displazia mai multor linii, bl <5%, SI <15%

Question 11

CRDML + SI

Answer 11

Sg perif - citopenii, bl <1%, Mo<1000

MO - displazia mai multor linii, bl <5%, SI >15%

Question 12

AREB1 (anemie refractara cu exces de blasti)

Answer 12

Sg perif - citopenii, bl <5%, Mo <1000

MO - displazie, bl 5-9%

Question 13

AREB2

Answer 13

Sg perif - citopenii, bl 5-19%, Mo <1000

MO - displazie, bl 10-19%

Question 14

SMD inclasabil

Answer 14

Sg perif - citopenii, bl <1%

MO - displazie biliniara (G+Mgk), bl <5%

Question 15

SMD cu 5q- izolat

Answer 15

sg perif - anemie, bl >1%, PLT N/crescut

MO - mgk N/mari cu nucl hipolobulati, Bl <5%

Question 16

LMMo cr

Answer 16

sg perif - blasti <5%, Mo > 1000 /mm3

MO - bl <20%

Question 17

Evolutie

Answer 17

stabila
evol lenta spre LA
anomalii cromosomiale - evol rapida spre LA

Question 18

Prognostic

Answer 18

entitatea FAB, anomalii citogen si in culturi

scor international - nr bl, citopenii, cariotip

Question 19

Tratament

Answer 19

chimioterapie/transplant CS
fact crestere hematopoietici
trat antiapoptotic
ag hipometilanti (azacitidina) in SMD cu risc crescut

Question 20

Tratament SMD hipoplazic

Answer 20

imunosupresie

Question 21

Tratament LMMo cr

Answer 21

HXU, azacitidina