74
amounts to 0.5–1% per year [ 5 ]. MM is a very heterogeneous malignant disorder
with refractory and relapsing course of disease that can be classified as low and
highrisk [ 6 ]. Highrisk MM is characterized by presence of extramedullary disease,
larger than 20% plasma cell in peripheral blood (plasma cell leukemia), unfavorable
genetic alterations (deletion 17p, deletion 1p, gain 1q21, t(14;20), t(14;16)), and
poor clinical outcome and overall survival (OS) [ 6 – 8 ].
The introduction of highdose (HD) chemotherapy and autologous bone marrow
transplantation (ABMT) 30 years ago has revolutionized the treatment of patients
with MM [ 9 ]. It is general consensus among myeloma experts that HDchemotherapy
and autologous stem cell transplantation (ASCT) is the backbone for treatment in
newly diagnosed MM patients [ 10 ]. However, in the era of novel, potent anti
myeloma agents the role of ASCT needs to be redefined. The treatment landscape
has dramatically changed in recent years with the introduction of highly effective
MGUS Multiple Myeloma
Serum M-protein ≥3.0 g/dL
OR
Urine M-protein ≥500 mg/24h
AND / OR
Clonal plasma cells in bone marrow: 10–
60%
Absence of myeloma defining events or
amyloidosis
Clonal plasma cell in bone marrow: ≥10%
OR
Biopsy-proven bony
or extramedullary plasmocytoma
≥1 Myeloma defining events:
- Hypercalcemia: serum calcium >1 mg/dL
higher than the upper limit of normal or
11 mg/dL
- Renal insufficiency: creatinine clearance
<40 mL/min or serum creatinine >2 mg/dL - Anemia: Hemoglobin >2 g/dL below the
lower limit of normal range, or hemoglobin
<10 g/dL - Bone lesions: ≥1 osteolytic lesion(s) on
skeletal radiography, CT, or PET-CT
OR
≥1 New myeloma defining biomarkers: - Clonal plasma cell in bone marrow: ≥60%
- Involved : uninvolved serum free light
chain ratio ≥100
1 focal lesions on MRI studies
Serum M-protein <3.0 g/dL
Clonal plasma cells in bone marrow: <10%
Absence of myeloma defining events or
amyloidosis
Smoldering Myeloma
No treatment
Fig. 5.1. Diagnostic criteria for multiple myeloma, smoldering myeloma and monoclonal gam
mopathy of undetermined significance
S. Thanendrarajan and T.K. Garg