Definition | Progression rate | Primary progression events | |
Non-IgM monoclonal gammopathy of undetermined significance | Serum monoclonal protein (non-IgM type) <30 g/L Clonal bone marrow plasma cells <10%* Absence of end-organ damage such as hypercalcemia, renal insufficiency, anemia, and bone lesions (CRAB) or amyloidosis that can be attributed to the plasma cell proliferative disorder | 1% per year | Multiple myeloma, solitary plasmacytoma, immunoglobulin-related amyloidosis (AL, AHL, AH) |
IgM monoclonal gammopathy of undetermined significance | Serum IgM monoclonal protein <30 g/L Bone marrow lymphoplasmacytic infiltration <10% No evidence of anemia, constitutional symptoms, hyperviscosity, lymphadenopathy, hepatosplenomegaly, or other end-organ damage that can be attributed to the underlying lymphoproliferative disorder | 1.5% per year | Waldenström macroglobulinemia, immunoglobulin-related amyloidosis (AL, AHL, AH) |
Light-chain monoclonal gammopathy of undetermined significance | Abnormal FLC ratio (<0.26 or >1.65) Increased level of the appropriate involved light chain (increased kappa FLC in patients with ratio >1.65 and increased lambda FLC in patients with ratio <0.26) No immunoglobulin heavy chain expression on immunofixation Absence of end-organ damage such as hypercalcemia, renal insufficiency, anemia, and bone lesions (CRAB) or amyloidosis that can be attributed to the plasma cell proliferative disorder Clonal bone marrow plasma cells <10% Urinary monoclonal protein <500 mg/24 hours | 0.3% per year | Light chain multiple myeloma, immunoglobulin light-chain amyloidosis |
Solitary plasmacytoma | Biopsy-proven solitary lesion of bone or soft tissue with evidence of clonal plasma cells Normal bone marrow with no evidence of clonal plasma cells Normal skeletal survey and MRI (or CT) of spine and pelvis (except for the primary solitary lesion) Absence of end-organ damage such as hypercalcemia, renal insufficiency, anemia, or bone lesions (CRAB) that can be attributed to a lymphoplasma cell proliferative disorder | About 10% within three years | Multiple myeloma |
Solitary plasmacytoma with minimal marrow involvement¶ | Biopsy-proven solitary lesion of bone or soft tissue with evidence of clonal plasma cells Clonal bone marrow plasma cells <10% Normal skeletal survey and MRI (or CT) of spine and pelvis (except for the primary solitary lesion) Absence of end-organ damage such as hypercalcemia, renal insufficiency, anemia, or bone lesions (CRAB) that can be attributed to a lymphoplasma cell proliferative disorder | 60% (bone) or 20% (soft tissue) within three years | Multiple myeloma |
POEMS syndromeΔ | Polyneuropathy Monoclonal plasma cell proliferative disorder (almost always lambda) Any one of the following three other major criteria: Sclerotic bone lesions Any one of the following six minor criteria: Organomegaly (splenomegaly, hepatomegaly, or lymphadenopathy) | NA | NA |
Systemic AL amyloidosis¥‡ | Presence of an amyloid-related systemic syndrome (eg, renal, liver, heart, gastrointestinal tract, or peripheral nerve involvement) Positive amyloid staining by Congo red in any tissue (eg, fat aspirate, bone marrow, or organ biopsy) Evidence that amyloid is light-chain-related established by direct examination of the amyloid using mass spectrometry-based proteomic analysis, or immunoelectronmicroscopy, and Evidence of a monoclonal plasma cell proliferative disorder (serum or urine monoclonal protein, abnormal free light-chain ratio, or clonal plasma cells in the bone marrow) | NA | Some patients might develop multiple myeloma |
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