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2022-10-21T11:58:25.000Z

Visual abstract | Key updates to the classification of plasma cell neoplasms and paraprotein disorders: The 5th WHO Classification

Oct 21, 2022
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Learning objective: After reading this article, learners will be able to describe the latest changes in the 5th edition of the WHO classification for plasma cell neoplasms and other diseases with paraproteins.

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The Multiple Myeloma Hub is pleased to present a visual abstract highlighting key updates and changes in the 5th edition of the World Health Organization (WHO) Classification of Haematolymphoid Tumours,1 focussing on plasma cell neoplasms and paraprotein-producing disorders.

The latest update reflects changes in the reclassification of some conditions.2 Several new conditions have been added, including AESOP syndrome, TEMPI syndrome (which was a provisional entry in the 4th edition), cold agglutinin disease, and monoclonal gammopathy of renal significance (the subtypes of which are detailed in Table 1).

Furthermore, a number of sections have been reorganized, for example, IgM and non-IgM monoclonal gammopathy of unknown significance and monoclonal gammopathy of renal significance have been grouped as monoclonal gammopathies, diseases with abnormal monoclonal immunoglobulin deposits have been classified together, and heavy chain diseases are now included as plasma cell disorders.



Table 1. MGRS subtypes according to the 5th edition of the World Health Organization Classification of Haematolymphoid Tumours*

Subtype

Ig deposits

Immunological and ultrastructural characteristics

Extrarenal manifestations

Always associated with MGRS

Crystal storing histiocytosis

LC

Intracellular LC crystals in interstitial histiocytes (with or without crystals in tubular and glomerular cells

Yes

Crystalglobulin-induced nephropathy

Ig

Extracellular MIg crystals within glomerular and vessel lumina (with or without TMA)

Yes

Ig-related amyloidosis

LC, Ig, HC

Extracellular deposition of Congophilic randomly-oriented fibrils

Yes

Light chain proximal tubulopathy

LC

Crystalline or non-crystalline LC inclusions within proximal tubular cells

Yes

Monoclonal immunoglobulin deposition disease

LC, Ig, HC

Finely granular “punctate” MIg deposits in tubular and glomerular basement membranes and mesangium

Yes

Proliferative glomerulonephritis with monoclonal immunoglobulin deposits

Ig, LC

Amorphous MIg deposits in mesangium and  subendothelial zone (and occasionally in subepithelial zone)

No

Frequently associated with MGRS

C3 glomerulopathy with MG

Only C3

C3 deposits in mesangium, subendothelial and subepithelial zones (and the lamina densa of the glomerular basement membranes in dense deposit disease)

No

Cryoglobulinemic glomerulonephritis  (Type I and most II)

Ig

Short microtubular and annular deposits composed of MIg only or MIg and polyclonal Igs

Yes

Monoclonal immunotactoid glomerulonephritis

Ig

Microtubular deposits composed of MIg

No

Thrombotic microangiopathy with MG

None

Chronic endothelial cell injury, no MIg or C3 deposits

Yes

Rarely associated with MGRS

Monotypic membranous nephropathy

Ig

Subepithelial deposits of MIg

No

Monotypic anti-GBM disease

Ig

Linear deposits of MIg along the glomerular basement membranes, no electron dense deposits ultrastructurally

No

Monotypic IgA nephropathy/ Henoch Schönlein purpura nephritis

Ig

Monoclonal IgA deposits in the mesangium ((and occasionally in subepithelial and/or subepithelial zones)

No/yes

GBM, glomerular basement membrane; HC, heavy chain; Ig, immunoglobulin; LC, light chain; MG, monoclonal gammopathy; MIg, monoclonal immunoglobulin; MRGS, monoclonal gammopathy of renal significance; TMA, thrombotic microangiopathy.
*Adapted from WHO Classification of Tumours Online. Haematolymphoid Tumours (5th ed.).1

Visual Abstract

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  1. World Health Organization. WHO Classification of Tumours Online. Haematolymphoid Tumours (5th ed.). https://tumourclassification.iarc.who.int/chapters/63. Accessed Sep 30, 2022.
  2. Alaggio R, Amador C, Anagnostopoulos I, et al. The 5th edition of the World Health Organization Classification of Haematolymphoid Tumours: Lymphoid Neoplasms. Leukemia. 2022;36(7):1720-1748. DOI: 1038/s41375-022-01620-2

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