Limfoproliferari cronice

Question 1

LLC - definitie

Answer 1

b clonala - acum de Ly mature in MO, sg, ggl limfatici, splina, ficat etc

Question 2

LLC - clinic

Answer 2

asimptomatic
manif gen - astenie, fatig
sdr tu - adenopatii, hepatosplenomegalie
insuf med, osteoporoza
sdr de hipervascozit
simptomele sunt dat infiltrarii cu Ly a tes

Question 3

LLC - hlg

Answer 3

leucocitoza - limfocitoza > 5000/mm3, >3 luni, clonala

anemie, trombocitopenie

Question 4

LLC - tablou sg

Answer 4

umbre Gumprecht

>20% proLy = LEUCEMIE PROLIMFOCITARA

Question 5

LLC - imunofenotipare

Answer 5

caracteristic - LyB CD19+CD5 (marker LyT)
expresie scazuta a Ig de suprafata
ciclina D1 - (DDx cu LNH cu cel de manta)
monoclonalitate: Ig supraf au un singur tip de lant usor

Question 6

LLC - anomalii citogenetice

Answer 6

mutatia genei IgHV (del3q14 - gena portiunii variabile a lantului greu al Ig) - prognstic favorabil
del13q, 12+ - prognostic favorabil
del 17p, 11q, starea nemutata a genei IgHV (asoc cu CD38, ZAP70)- prognostic nefavorabil

Question 7

LLC - diagnostic

Answer 7

limfocitoza >5000/mm3, >3 luni
fenotip CD19, 20, 23 + CD5
expresie redusa a Ig de suprafata
monoclonalitate (flowcitometrie) - un singur tip de lant usor

Question 8

LLC - DDx

Answer 8

LNH leucemizate (cel de manta, limfom splenic cu cel viloase)
HCL var
leucemia prolimfocitara
limfocitoza infectioasa (policlon)

Question 9

LLC - evolutie prognostic, complicatii

Answer 9

Sdr Richter -> LNH cu grad inalt de malignitate
malignitati secundare
infectii, hrg, AHAI

Question 10

LLC - stadializarea RAI

Answer 10

0 - limfocitoza perif >15.000, med >40%
1 - 0 + adenopatii palpabile
2 - 0 +/- 1 + hepato/splenomegalie
3 - 0 +/- 1 +/- 2 + anemie (Hb <10 g/dl)
4 - 0 +/- 1 +/- 2 +/- 3 + tromboctiopenie (PLT <100.000)

Question 11

LLC - stadializarea Binet

Answer 11

A - limfocitoza + <3 arii limfatice afect
B - limfocitoza + >3arii limfatice interesate
C - limfocitoza + anemie/trombopenie

Question 12

LLC - factori de prognostic negativ

Answer 12

dublare Ly <1 an
LDH, beta2 micro glob
CD38, ZAP 70, absenta mutatiei IgHV
del 17p, 11q
morfologie atipica
infiltrar med limfoid difuz

Question 13

LLC tratament - indicatii

Answer 13

semne gen
anemie, trombocitopenie progr
anemie, trombocitopenie imune refract la corticoizi
mase ggl >10cm, hepato/splenomegalie >6 cm sub rebord
infectii frecv
dubl Ly <6luni

Question 14

LLC - tratament

Answer 14

chimioterpie - cura RFC
corticoterapie
imunoteerapia (anti CD20, anti bcl2 - venetoclax)
ibrutinib - del 17p
radioterapie paliativa, splenectomie
leucafereza
CAR-T cell - inhiba CD19

Question 15

HCL - clinic

Answer 15

semne gen
splenomegalie
insuficienta medulara

Question 16

HCL - hlg

Answer 16

pancitopenie

sau HCL varianta cu leucocitoza

Question 17

HCL - tablou sg

Answer 17

cel paroase - linie B, mari, prel citopl, nucl excentric –> secr un inhibitor al hematopoiezei normale

Question 18

HCL - mo

Answer 18

punctie - alba

BOM - fibroza, infiltrat cu mononucl

Question 19

HCL - biopsie splenica

Answer 19

pulpa rosie - infiltrata cu cel paroase

pulpa alba - atrofica

Question 20

HCL - dg +

Answer 20

splenomegalie
pancitopenie
cel paroase
BOM - fibroza med
mutatia BRAF +

Question 21

HCL - DDx

Answer 21

LLC clasica
LPL
anemie aplastica
MMM

Question 22

HCL - complicatii

Answer 22

infectii
hemoragii
vasculite
lez osteolitice

Question 23

HCL - tratament

Answer 23

splenectomie

alfa IFN, 2 Cl deoxiADO