Skip to main content
. 2017 Oct 20;7(10):e617. doi: 10.1038/bcj.2017.90

Table 1. Definitions of plasma cell related disorders (adopted from Rajkumar et al., 3 ).

Title Definition
MGUS Serum monoclonal protein (non-IgM type) <30 g/l
  Clonal bone marrow plasma cells <10%
  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:
   • 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%
   • Absence of myeloma defining events or amyloidosis
MM Clonal bone marrow plasma cells ⩾10% or biopsy-proven bony or extramedullary plasmacytoma
  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
  Normal skeletal survey and MRI (or CT) of spine and pelvis (except for the primary solitary lesion)
  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)
  No immunoglobulin heavy chain expression on immunofixation
  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
  Clonal bone marrow plasma cells <10%
  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)
  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
  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
  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
Smoldering WM Presence of serum IgM monoclonal protein
  Bone marrow lymphoplasmacytic infiltration >10%
  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
  Bone marrow lymphoplasmacytic infiltration >10%
  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)
  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 (oedema, pleural eff usion, or ascites)
   • Endocrinopathy (adrenal, thyroid, pituitary, gonadal, parathyroid, pancreatic)
   • Skin changes (hyperpigmentation, hypertrichosis, glomeruloid haemangiomata, plethora, acrocyanosis, flushing, white nails)
   • Papilloedema
   • Thrombocytosis/polycythaemia