- MGUS monoklonale Gammopathie unklarer Signifikanz
- SMM smoldering multiple Myeloma
(asyptomatisches Myelom)
SMM - smoldering multiple myeloma
asymptomatische Myelom
Risiko
Risikostratifizierung des SMM entweder nach Mayo oder Pethema Kriterien.
Pethema erfordert spezielle FACS Analyse, daher Verwendung von Mayo Kriterien
Konsequenz derzeit - Auswirkung auf Beobachtungsintervall
Therapie von high risk derzeit nur in Studien empfohlen.
low risk:
Mayo=1 Faktor
Pethema=0 Faktor
Progression=25%/5Jahre
Beobachtung alle 3-6 Mo
intermediate risk:
Mayo=2 Faktor
Pethema=1 Faktor
Progression=50%/5Jahre
Beobachtung alle 3 Mo
high risk:
Mayo=3 Faktor
Pethema=2 Faktor
Progression=70%/5Jahre
Beobachtung alle 1-3 Mo
Therapie in Studien
Definition:
Mayo Clinic criteria - 3 Faktoren
- Knochenmarkinifiltration >10%
- M-protein >3 g/L
- FLC ratio <0.125 or >8
1 Faktor = low risk SMM
2 Faktoren = intermediate risk SMM
3 Faktoren = high risk SMM
PETHEMA criteria
Voraussetzung FACS Analyse- 95% abnormal plasma cells, including decreased CD38 expression, expression of CD56, and absence of CD19 and/or CD45
- Immunoparesis
0 Faktor = low risk SMM
1 Faktoren = intermediate risk SMM
2 Faktoren = high risk SMM
Other risk factors
- IgA isotype
- the presence of proteinuria
- circulating plasma cells
- high proliferative rate of bone marrow plasma cells
- abnormal MRI findings
chromosomal abnormalities critical for the rate of progression
- deletion 17p
- t(4;14) is associated with the shortest time to progression
- trisomies were a risk factor for progression
- 1q21 were also associated with increased risk for progression
- evolving nature of the M spike
International Myeloma Working Group criteria for SMM:
- Serum M protein >3 g/dL
- Light-chain restricted bone marrow plasma cells >10%
- or urinary monoclonal protein >500 mg per 24 hr
- No end-organ damage
- Serumelektrophorese
- Knochenmarkbiopsie
24h Sammelurin
Niere,Elo,Blutbild, Skelett-Rö, MR Wirbelsäule-Becken