Skip to content
GERICLINICS

GeriQBank · Paper III · Q1

Name the various plasma cell dyscrasias

Low Yield
Topic
Haematology, Oncology & Screening
Chapter
Haematology
Years asked
Not recorded in the source
Frequency
Not set in Papers III–IV, 2009–2025

Detailed answer

Pro · free in beta
Attempt it firstOutline your answer before revealing it — definition, causes, evaluation, management and the geriatric angle.Reveal answer

Textbook gap: Pathy's has no dedicated chapter on plasma cell dyscrasias. Only incidental mentions exist in Ch. 24 (paraproteins as a cause of acquired coagulation defects — myeloma, MGUS, amyloid; inhibition of fibrin polymerisation; acquired haemophilia/vWD) and in the Anaemia chapter (MGUS grouped under "anaemia of clonal disorders" with CHIP/MDS). The classification below is sourced from WHO-HAEM5 (2022), ICC (2022) and IMWG consensus.

High-Yield Points

Definition

1.

Plasma cell dyscrasias (monoclonal gammopathies) are disorders in which a single clone of plasma/lymphoplasmacytic cells secretes a monoclonal immunoglobulin (M-protein).

2.

They form a continuous spectrum from harmless MGUS to plasma cell leukaemia. Every myeloma is preceded by an MGUS phase.

3.

Diseases of old age: MGUS in ~3% over 50; median myeloma diagnosis age ~69 years.

A. Premalignant precursor states

4.

MGUS — M-protein <3 g/dL, clonal marrow plasma cells <10%, no CRAB. Progresses ~1%/year.

5.

Three MGUS subtypes: Non-IgM (→ myeloma), IgM (→ Waldenström's), Light-chain (→ light-chain myeloma/AL amyloidosis).

6.

Smouldering (asymptomatic) myeloma — M-protein ≥3 g/dL or plasma cells 10–60%, no end-organ damage.

B. Malignant plasma cell neoplasms

7.

Multiple myeloma (symptomatic) — ≥10% clonal plasma cells or plasmacytoma + a myeloma-defining event (CRAB or SLiM).

8.

Variants: non-secretory, IgD/IgE myeloma (rare, aggressive), IgM myeloma, light-chain (Bence Jones) myeloma.

9.

Solitary plasmacytoma of bone — single lytic lesion, normal marrow, no CRAB.

10.

Extramedullary plasmacytoma — solitary soft-tissue lesion, usually upper aerodigestive tract.

11.

Plasmacytoma with minimal marrow involvement — <10% clonal marrow plasma cells; higher progression risk.

12.

Plasma cell leukaemia — primary or secondary; most aggressive form.

C. Immunoglobulin deposition disorders

13.

AL amyloidosis — misfolded light chains deposit as β-pleated fibrils in heart, kidney, liver, nerve, gut.

14.

Monoclonal Ig deposition disease (MIDD) — non-fibrillar, Congo-red negative: LCDD, HCDD, light-and-heavy-chain forms.

15.

Monoclonal gammopathy of renal significance (MGRS) — small non-malignant clone causing kidney damage; needs clone-directed treatment.

D. Heavy chain diseases

16.

Alpha HCD (IPSID/Mediterranean lymphoma), Gamma HCD (Franklin disease), Mu HCD (with CLL).

E. Lymphoplasmacytic disorders

17.

Waldenström macroglobulinaemia — IgM paraprotein, MYD88 L265P; causes hyperviscosity.

18.

Cold agglutinin disease and cryoglobulinaemia (Type I monoclonal; Type II mixed).

F. Paraneoplastic gammopathies

19.

POEMS syndrome — Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, Skin changes; lambda light chain, sclerotic lesions, high VEGF.

20.

Newer syndromes: TEMPI, AESOP, Schnitzler; umbrella term MGCS (monoclonal gammopathy of clinical significance) — neurological, cutaneous, haemostatic forms.

Latest Updates (2023–Present)

WHO-HAEM5 (2022) restructured the group into four blocks: Monoclonal gammopathies (adds MGRS, cold agglutinin disease); Ig deposition diseases (renamed AL amyloidosis, MIDD); Heavy chain diseases; Plasma cell MD Geriatrics – Haematology Compendium Page 3 of 34 neoplasms (adds TEMPI, AESOP as full entities). ICC 2022 introduced genetic subtyping of myeloma (CCND-family, MAF-family, NSD2 translocations, hyperdiploidy vs MM-NOS). Plasma cell leukaemia threshold lowered to ≥5% circulating plasma cells (from >20%). MGCS is now formally accepted, covering MGRS (renal), MGNS (neurological), cutaneous and multisystem forms. A new IMS–IMWG high-risk myeloma consensus (2025) replaces R-ISS/R2-ISS. AQUILA trial (NEJM 2025): daratumumab in high-risk smouldering myeloma improved 5-yr PFS (63.1% vs 40.8%) and OS (93.0% vs 86.9%) — FDA-approved Nov 2025, the first therapy for a precursor state. The IMWG Frailty Index (and possibly the Rockwood Frailty Index) now guides treatment intensity in the elderly.

Current Practice Update

Domain Pathy's (2022) Current evidence

Coverage No dedicated section Fully classified under WHO-HAEM5/ICC 2022 New entities Not mentioned MGRS, cold agglutinin disease, TEMPI, AESOP formally recognised MGCS concept Absent Established — small clones causing organ damage need clone-directed therapy Plasma cell leukaemia Not mentioned Redefined at ≥5% circulating plasma cells Smouldering myeloma Not mentioned Daratumumab approved (2025) for high-risk SMM MD Geriatrics – Haematology Compendium Page 4 of 34

How was your recall?

Rating marks the question complete. “Need another pass” also adds it to your bookmarks.

References

Spotted an error or something out of date?Report a problem with this page