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International Myeloma Working Group diagnostic criteria and classification for monoclonal gammopathy of undetermined significance and related plasma cell disorders

International Myeloma Working Group diagnostic criteria and classification for monoclonal gammopathy of undetermined significance and related plasma cell disorders
  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
Castleman's disease
Elevated levels of VEGFA

Any one of the following six minor criteria:

Organomegaly (splenomegaly, hepatomegaly, or lymphadenopathy)
Extravascular volume overload (edema, pleural effusion, or ascites)
Endocrinopathy (adrenal, thyroid, pituitary, gonadal, parathyroid, pancreatic)§
Skin changes (hyperpigmentation, hypertrichosis, glomeruloid hemangiomata, plethora, acrocyanosis, flushing, white nails)
Papilledema
Thrombocytosis/polycythemia

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
IgM: immunoglobulin M; AL: immunoglobulin light-chain amyloidosis; AHL: immunoglobulin heavy and light-chain amyloidosis; AH: immunoglobulin heavy chain amyloidosis; FLC: free light chain. MRI: magnetic resonance imaging; CT: computed tomography; POEMS: polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes; VEGFA: vascular endothelial growth factor A; NA: not applicable.
* Bone marrow can be deferred in patients with low-risk monoclonal gammopathy of undetermined significance (IgG type, monoclonal protein <15 g/L, normal free light-chain ratio) in whom there are no clinical features concerning for myeloma.
¶ Solitary plasmacytoma with 10% or more clonal plasma cells is regarded as multiple myeloma.
Δ Not every patient meeting these criteria will have POEMS syndrome; the features should have a temporal association with each other and no other attributable cause. Anemia or thrombocytopenia are distinctively unusual in this syndrome unless Castleman's disease is present.
The source data do not define an optimal cutoff value for considering elevated VEGFA level as a major criterion. We suggest that VEGFA measured in the serum or plasma should be at least three to four times higher than the normal reference range for the laboratory that is doing the testing to be regarded as a major criterion.
§ To regard endocrinopathy as a minor criterion, an endocrine disorder other than diabetes or hypothyroidism is required because these two disorders are common in the general population.
¥ Patients with AL amyloidosis who also meet criteria for multiple myeloma are considered to have both diseases.
‡ Approximately 2 to 3% of patients with AL amyloidosis will not meet the requirement for evidence.
Reproduced from: Rajkumar SV, Dimopoulos MA, Palumbo A, et al. International Myeloma Working Group updated criteria for the diagnosis of multiple myeloma. Lancet Oncol 2014; 15:e538. Table used with the permission of Elsevier Inc. All rights reserved.
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