MGUS |
Serum monoclonal protein (non-IgM type) <30 g/l |
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Clonal bone marrow plasma cells <10% |
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Absence of end-organ damage such as hypercalcaemia, renal insufficiency, anaemia, and bone lesions (CRAB) or amyloidosis that can be attributed to the plasma cell proliferative disorder |
SMM |
Both criteria must be met: |
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• Serum monoclonal protein (IgG or IgA) ⩾30 g/l or urinary monoclonal protein ⩾500 mg per 24 h and/or clonal bone marrow plasma cells 10–60% |
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• Absence of myeloma defining events or amyloidosis |
MM |
Clonal bone marrow plasma cells ⩾10% or biopsy-proven bony or extramedullary plasmacytoma |
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Evidence of any of myeloma defining events |
PCL |
Presence of >20% of clonal plasma cells in peripheral blood and/or the absolute number of circulating plasma cells exceeding 2 × 109/l in peripheral blood |
Solitary |
Biopsy-proven solitary lesion of bone or soft tissue with evidence of clonal plasma cells |
Plasmacytoma |
Normal bone marrow with no evidence of clonal plasma cells |
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Normal skeletal survey and MRI (or CT) of spine and pelvis (except for the primary solitary lesion) |
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Absence of end-organ damage such as hypercalcaemia, renal insufficiency, anaemia, or bone lesions (CRAB) that can be attributed to a lymphoplasma cell proliferative disorder |
Light-chain |
Abnormal FLC ratio (<0·26 or >1·65) |
MGUS |
Increased level of the appropriate involved light chain (increased κ FLC in patients with ratio >1·65 and increased λ FLC in patients with ratio <0·26) |
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No immunoglobulin heavy chain expression on immunofixation |
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Absence of end-organ damage such as hypercalcaemia, renal insufficiency, anaemia, and bone lesions (CRAB) or amyloidosis that can be attributed to the plasma cell proliferative disorder |
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Clonal bone marrow plasma cells <10% |
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Urinary monoclonal protein <500 mg/24 h |
AL |
Presence of an amyloid-related systemic syndrome (eg, renal, liver, heart, gastrointestinal tract, or peripheral nerve involvement) |
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Positive amyloid staining by Congo red in any tissue (eg, fat aspirate, bone marrow, or organ biopsy) |
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Evidence that amyloid is light-chain-related established by direct examination of the amyloid using mass spectrometry-based proteomic analysis, or immunoelectronmicroscopy |
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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) |
IgM-MGUS |
Serum IgM monoclonal protein <30 g/l |
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Bone marrow lymphoplasmacytic infiltration <10% |
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No evidence of anemia, constitutional symptoms, hyperviscosity, lymphadenopathy, hepatosplenomegaly or other end-organ damage that can be attributed to the underlying lymphoproliferative disorder |
Smoldering WM |
Presence of serum IgM monoclonal protein |
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Bone marrow lymphoplasmacytic infiltration >10% |
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No evidence of anaemia, constitutional symptoms, hyperviscosity, lymphadenopathy, hepatosplenomegaly, or other end-organ damage that can be attributed to the underlying lymphoproliferative disorder |
WM |
Presence of serum IgM monoclonal protein |
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Bone marrow lymphoplasmacytic infiltration >10% |
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Evidence of anaemia, constitutional symptoms, hyperviscosity, lymphadenopathy, hepatosplenomegaly, or other end-organ damage that can be attributed to the underlying lymphoproliferative disorder |
POEMS |
Polyneuropathy |
Syndrome |
Monoclonal plasma cell proliferative disorder (almost always λ) |
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Any one of the following three other major criteria: |
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• Sclerotic bone lesions |
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• Castleman’s disease |
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• Elevated levels of VEGFA |
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Any one of the following six minor criteria: |
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• Organomegaly (splenomegaly, hepatomegaly, or lymphadenopathy) |
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• Extravascular volume overload (oedema, pleural eff usion, or ascites) |
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• Endocrinopathy (adrenal, thyroid, pituitary, gonadal, parathyroid, pancreatic) |
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• Skin changes (hyperpigmentation, hypertrichosis, glomeruloid haemangiomata, plethora, acrocyanosis, flushing, white nails) |
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• Papilloedema |
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• Thrombocytosis/polycythaemia |