Author: Kazunori Murata
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Date: JAN.30.2019
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Source: Trainee Council in English
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What is Multiple Myeloma?
- Hematological Cancer
- Develops in the bone marrow
- Cancer of PLASMA CELLS
Monoclonal Gammopathies
Group of diseases characterized by expansion of a single clone of plasma cells
- MGUS
- Smoldering multiple myeloma
- Waldenstrom's Macroglobulinemia
- Solitary Plasmacytoma
- AL amyloidosis
- POEMS
- Multiple Myelom
Epidemiology of MM
- Incidence of 4-5/100,000
- 1% of all cancers
- 10% of hematologic malignancies in the US
- Median age at diagnosis: 66
- Risk factors
- advanced age
- black race
- Males
- family history
Clinical Aspects of Multiple Myeloma
- Pathophysiology
- Hypercalcemia
- due to bone lytic lesions
- neurological symptoms
- Renal insufficiency
- tubular damage by free light chains
- Anemia
- overgrowth of plasma cells leads to crowding out of cells for RBC production
- Bone lesions
- fractures due to lytic lesions caused by plasma
- Natural Course
- Fatal without treatment
- Incurable, but highly effective treatments available
- Patients generally relapse after treatment
- Patients can undergo multiple treatments
- Need to constantly monitor disease to detect relapse
Tests Vital to Diagnosis/Monitoring of MM
- Clinical examination/history
- Imaging
- Pathology
- bone marrow aspirate/biopsy
- Laboratory Tests
- hematologic
- chemistry
- serum protein electrophoresis/immunofixation
- urine protein
- electrophoresis/immunofixation
- serum free light chains
International Myeloma Working Group (IMWG)
- Offshoot of International Myeloma Foundation
- International consortium of > 200 leading myeloma researchers
- Develop guidelines for diagnosis, management, response criteria, etc
- Constantly evolving and updated
- Last update for diagnostic criteria issued 2014
2014 IMWG criteria for the Diagnosis of MM
- Clonal bone marrow plasma cells ≥ 10% or biopsy proven bony or soft tissue plasmacytoma (clonality must be established by flow, IHC, or IF)
PLUS
- Presence of related organ or tissue impairment (CRAB)
OR
- Presence of a biomarker associated with near inevitable progression to end-organ damage
*Rajkumar et al. Lancet Oncol 2014;15:e538-48.
Presence of related organ or tissue impairment (CRAB)
-
Anemia
- hemoglobin < 10g/dL or
- 2g/dL below normal
- Hypercalcemia
- Renal insufficiency
- eGFR/GFR < 40 ml/min OR
- serum creatinine > 2mg/dL
- Bone lesions
- one or more osteolytic lesions on skeletal radiography, MRI, CT or PET/CT
Presence of a biomarker associated with near inevitable progression to end-organ damage: Myeloma Defining Events (MDE's)
- ≥ 60% clonal plasma cells in bone marrow
- involved/uninvolved FLC ratio of 100 or more (involved FLC must be ≥ 100 mg/L)
- MRI with more than one focal lesion (involving bone or bone marrow)
*Rajkumar et al. Lancet Oncol 2014;15:e538-48.
Other Monoclonal Gammopathies/Plasma Cell Disorders
- Smoldering Multiple Myeloma
- Monoclonal Gammopathy of Undetermined Significance (MGUS)
- Solitary Plasmacytoma
- POEMS Syndrome
- Light Chain Amyloidosis