GAMOPATII Flashcards

1
Q

Gamopatia monoclonala cu semnificatie necunoscuta

A

proteina M in absenta unei hemopatii maligne

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2
Q

GMSN - dg

A
proteina M <3g/dl
prot BJ absenta
plasmocitoza med <10%
abs anmie, hipercalcemie, lez osteolitice
fct renala N
niv stabil al prot M
abs alto limfoprolif maligne
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3
Q

GMSN - evol, prognostic

A

prot M ramane stabila
1/4 -> evol spre GM maligne
urmarire clinico-bio per

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4
Q

MM - def

A

afect maligna dat prolif Pl -> invad MO, prod lez osteolitice, secr prot M

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5
Q

MM - patogeneza

A

Pl cu prolif controlata -> anomalii cromosomiale -> + oncogene => prolif necontrolata

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6
Q

MM - clinic

A
per asimptomatica
dureri osoase, fract pe os patologic
hipercalcemie - sdr poliuro-polidipsic, greata, varsaturi, constipatie
IR
manif neuro
sdr hipervascozitate
insuf medulara
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7
Q

MM - hlg

A

anemie

leucocite, PLT N/scazute

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8
Q

MM - tablou sg

A

N/ hematii in fisicuri, Pl maligne

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9
Q

MM - mo

A

medulograma - infiltrat Pl maligne >10%

BOM - infiltrat nod Pl

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10
Q

MM - biochimie

A
Prot tot crescute, electroforez P serice si urinare
proteinuria Bence Jones
Imunelectroforeza - Ig monoclon
hiper calcemie, uricemie
fct renala
LDH, b2 microglobulina
coag
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11
Q

MM - Rx

A

osteoliza - calota, coaste, stern, clavic, bazin, vertebre
osteoporoza
osteocondensare - rar (sdr POEMS)

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12
Q

sdr POEMS

A
Polineuropatie
Osteoscleroza
Endocrinopatie
prot M
Skin - lez cut
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13
Q

MM - dg +

A
>10% Pl monoclon in mo
Prot M in ser/urina
crit CRAB
hiperCalcemie
insuf Renala
Anemie
Bones
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14
Q

MM - DDx

A
alte cauze de plasmocitoza, lez osteolitice, meta osoase
plasmocitom solitar
amiloidoza AL
macroglobulinemia Waldenstrom
sdr POEMS
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15
Q

MM - clasif ISS

A

b2microglob, alb
I - b2 <3,5 mg/dl, alb >=3,5 g/dl
II - b2 <3,5 mg/dl, alb <3,5 g/dl/ b2 3,5-5,5 mg/dl
III - b2 >5,5 mg/dl

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16
Q

MM - fact prognostic

A
LDH
anomalii cr - del 17p, t (4,14), t(14,16)
comorbid
varsta >70 ani
infiltrat pl >50%
17
Q

MM - tratament mas gen

A

lez os - evit mic bruste, mobiliz in lim tol, antialgice, irad loc, bifosfonati
hiperuricemie - allopurinol
hipercalcemie - hidrat, diuretice, bifosfonati
IR - dializa
anemie - EPO

18
Q

MM - tratament citostatic

A

< 65 ani - bortezomib (inhib proteazom) + DXM + thalidonmida (antiangiogenetic, imunomodulator)
> 65 ani - ag terap noi
salvare (refract, resuta) - transplant CS, alti ag terap, CAR T cell

19
Q

Macroglobulinemia Waldenstrom - def

A

cresterea conc IgM monoclon

20
Q

Macroglobulinemia Waldenstrom - clinic

A
semne gen
paloare, adenopatii, hepatosplenomegalie
manif hrg
hipervascozitate
polineuropatie SZ-M
21
Q

Macroglobulinemia Waldenstrom - hlg, tablou perif

A

anemie, trombocitopenie
leucocite N/scazute/crescute
hematii in fisicuri

22
Q

Macroglobulinemia Waldenstrom - medulograma

A

infiltrat LIMFO-plasmocitar

23
Q

Macroglobulinemia Waldenstrom - biochimie

A

Prot tot crescute cu IgM monoclonal mult crescut
IR (dep IgM pe fata endo a MBG)
test Coombs, aglut la rece +

24
Q

Macroglobulinemia Waldenstrom - dg +

A

organomegalii
infiltrat Ly-Pl (imunofenotipare)
Ig M monoclonal
mutatia MYD1

25
Q

Macroglobulinemia Waldenstrom - DDx

A

MM IgM

macroglobulinemii sec - LLC, LPL, LNH

26
Q

Macroglobulinemia Waldenstrom - evol, prognostic

A

sv medie - 4-5 ani

fact prognostic neg - >65 ani, alb <4g/dl, citopenii

27
Q

Macroglobulinemia Waldenstrom - tratament

A

chimioterapie - clorambucil, ciclofosfamida, corticoterapie
Ac anti CD20 - rituximab
plasmafereza