GAMOPATII Flashcards
Gamopatia monoclonala cu semnificatie necunoscuta
proteina M in absenta unei hemopatii maligne
GMSN - dg
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
GMSN - evol, prognostic
prot M ramane stabila
1/4 -> evol spre GM maligne
urmarire clinico-bio per
MM - def
afect maligna dat prolif Pl -> invad MO, prod lez osteolitice, secr prot M
MM - patogeneza
Pl cu prolif controlata -> anomalii cromosomiale -> + oncogene => prolif necontrolata
MM - clinic
per asimptomatica dureri osoase, fract pe os patologic hipercalcemie - sdr poliuro-polidipsic, greata, varsaturi, constipatie IR manif neuro sdr hipervascozitate insuf medulara
MM - hlg
anemie
leucocite, PLT N/scazute
MM - tablou sg
N/ hematii in fisicuri, Pl maligne
MM - mo
medulograma - infiltrat Pl maligne >10%
BOM - infiltrat nod Pl
MM - biochimie
Prot tot crescute, electroforez P serice si urinare proteinuria Bence Jones Imunelectroforeza - Ig monoclon hiper calcemie, uricemie fct renala LDH, b2 microglobulina coag
MM - Rx
osteoliza - calota, coaste, stern, clavic, bazin, vertebre
osteoporoza
osteocondensare - rar (sdr POEMS)
sdr POEMS
Polineuropatie Osteoscleroza Endocrinopatie prot M Skin - lez cut
MM - dg +
>10% Pl monoclon in mo Prot M in ser/urina crit CRAB hiperCalcemie insuf Renala Anemie Bones
MM - DDx
alte cauze de plasmocitoza, lez osteolitice, meta osoase plasmocitom solitar amiloidoza AL macroglobulinemia Waldenstrom sdr POEMS
MM - clasif ISS
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
MM - fact prognostic
LDH anomalii cr - del 17p, t (4,14), t(14,16) comorbid varsta >70 ani infiltrat pl >50%
MM - tratament mas gen
lez os - evit mic bruste, mobiliz in lim tol, antialgice, irad loc, bifosfonati
hiperuricemie - allopurinol
hipercalcemie - hidrat, diuretice, bifosfonati
IR - dializa
anemie - EPO
MM - tratament citostatic
< 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
Macroglobulinemia Waldenstrom - def
cresterea conc IgM monoclon
Macroglobulinemia Waldenstrom - clinic
semne gen paloare, adenopatii, hepatosplenomegalie manif hrg hipervascozitate polineuropatie SZ-M
Macroglobulinemia Waldenstrom - hlg, tablou perif
anemie, trombocitopenie
leucocite N/scazute/crescute
hematii in fisicuri
Macroglobulinemia Waldenstrom - medulograma
infiltrat LIMFO-plasmocitar
Macroglobulinemia Waldenstrom - biochimie
Prot tot crescute cu IgM monoclonal mult crescut
IR (dep IgM pe fata endo a MBG)
test Coombs, aglut la rece +
Macroglobulinemia Waldenstrom - dg +
organomegalii
infiltrat Ly-Pl (imunofenotipare)
Ig M monoclonal
mutatia MYD1
Macroglobulinemia Waldenstrom - DDx
MM IgM
macroglobulinemii sec - LLC, LPL, LNH
Macroglobulinemia Waldenstrom - evol, prognostic
sv medie - 4-5 ani
fact prognostic neg - >65 ani, alb <4g/dl, citopenii
Macroglobulinemia Waldenstrom - tratament
chimioterapie - clorambucil, ciclofosfamida, corticoterapie
Ac anti CD20 - rituximab
plasmafereza
