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Table 3 Comparison of WHO-HAEM4R, ICC and WHO-HAEM5 classification of plasma cell neoplasms and related disorders

From: Mature B, T and NK-cell, plasma cell and histiocytic/dendritic cell neoplasms: classification according to the World Health Organization and International Consensus Classification

WHO HAEM4R

ICC

WHO HAEM5

Plasma cell neoplasms and related disorders

Monoclonal gammopathies

Not included

Primary cold agglutinin disease

Cold agglutinin disease

IgM monoclonal gammopathy of uncertain significance (MGUS)

IgM MGUS

-Lymphoplasmacytic lymphoma type

-Plasma cell neoplasm type

Non-IgM MGUS

IgM MGUS

Non-IgM MGUS

Monoclonal gammopathy of renal significance

Plasma cell neoplasms

Plasmacytoma

-Solitary plasmacytoma of bone

-Extraosseous plasmacytoma

-Solitary plasmacytoma of bone

-Extraosseous plasmacytoma

Plasmacytoma

Plasma cell myeloma

Plasma cell myeloma variants

-Smoldering plasma cell myeloma

-Non-secretory myeloma

-Plasma cell leukemia

Multiple myeloma (plasma cell myeloma)

-Multiple myeloma NOS

- Multiple myeloma with recurrent genetic abnormality

- Multiple myeloma with CCND family translocation

- Multiple myeloma with MAF family translocation

- Multiple myeloma with NSD2 family translocation

- Multiple myeloma with hyperdiploidy

Plasma cell myeloma/multiple myeloma (PCM/MM)

PCM/MM subtypes:

-Smoldering plasma cell myeloma

-Non-secretory myeloma

-Plasma cell leukemia

Plasma cell neoplasms with associated paraneoplastic syndrome

-POEMS syndrome

-TEMPI syndrome (provisional)

Plasma cell neoplasms with associated paraneoplastic syndrome

-POEMS syndrome

-TEMPI syndrome

Plasma cell neoplasms with associated paraneoplastic syndrome

-POEMS syndrome

-TEMPI syndrome

-AESOP syndrome

Heavy chain diseases

Mu heavy chain disease

Gamma heavy chain disease

Alpha heavy chain disease

Mu heavy chain disease

Gamma heavy chain disease

Alpha heavy chain disease

Mu heavy chain disease

Gamma heavy chain disease

Alpha heavy chain disease

Diseases with monoclonal immunoglobulin deposition

Primary amyloidosis

-Immunoglobulin light chain amyloidosis (AL)

-Localized AL amyloidosis

Immunoglobulin-related amyloidosis (AL amyloidosis)

Light chain and heavy chain deposition diseases

Light chain and heavy chain deposition disease

Monoclonal immunoglobulin deposition disease