Plasma cell neoplasms Flashcards Preview

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Flashcards in Plasma cell neoplasms Deck (12)
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1
Q

Immunophenotype of normal and abnormal plasma cells

A

Normal: CD45+, CD138+, bright CD38, CD19+, CD56-
Abnormal: Absent/dim CD45, weakened CD38, brighter CD138, CD19-/CD56+ or CD19-/CD56+, may have aberrant expression of other antigens e.g. CD20, CD200

2
Q

Risk factors for progression of MGUS to symptomatic myeloma, plasmacytoma or amyloidosis

A
  1. Predominance (>90%) of aberrant plasma cells by flow cytometry.
  2. Abnormal serum free light chain ratio
  3. Type of paraprotein: IgA paraprotein (~1.5% per year) is higher than IgG or light chain MGUS. IgM paraprotein has highest risk.
  4. High paraprotein > 15 - 25g/L
3
Q

Diagnostic criteria for asymptomatic/smouldering myeloma

A

Paraprotein > 30g/L or urinary M protein > 0.5g/24hrs AND/OR clonal plasma cells in bone marrow 10-60%
No end-organ damage including amyloidosis

4
Q

Diagnostic criteria for MGUS

A

Paraprotein < 30g/L
Plasma cells <10%
No end organ damage
No lymphoproliferative disorder

5
Q

Diagnostic criteria for symptomatic plasma cell myeloma

A

Clonal plasma cells in BM ≥ 10% or plasmacytoma
End-organ damage / myeloma-defining events:
- Hypercalcaemia
- Renal impairment (CrCl <40ml/min or Creat >177umol/L)
- Anaemia (<100g/L or >20g/L below LLN
- Bone lesions (osteolytic, seen on imaging)

OR ≥1 biomarker of malignancy if end-organ damage is not present

  • Clonal BM plasma cells ≥ 60%
  • SFLC ratio ≥100 (involved to uninvolved)
  • > 1 focal lesion on MRI
6
Q

Diagnosis of plasma cell leukaemia

A

Plasma cells > 20% of WBCs or plasma cells > 2 x E+9/L

Immunophenotype: Usually CD56- and CD20+

7
Q

Name two paraneoplastic syndromes associated with myeloma

A

POEMS syndrome and TEMPI syndrome

8
Q

What is TEMPI syndrome?

A
Telangiectasia
Elevated erythropoietin and erythrocytosis
Monoclonal gammopathy
Perinephric fluid collections
Intrapulmonary shunting
9
Q

Diagnostic criteria for POEMS syndrome

A
Mandatory:
Polyneuropathy and monoclonal gammopathy.
Major criteria (1 or more required)
- Castleman disease (plasma cell variant)
- Osteosclerotic bone lesions
- VEGF elevation
Minor criteria (1 or more required)
- Organomegaly
- Endocrinopathy
- Skin changes
- Papilloedema
- Thrombocytosis
- Extravascular volume overload
10
Q

How is light chain deposition disease different from primary amyloidosis (4)

A
  1. No beta-pleated sheets
  2. No serum amyloid P protein
  3. No staining with Congo red
  4. Usually kappa (80%)
11
Q

Name the common coagulopathy found in amyloid disease

A

Acquired factor X deficiency (factor X binds to the amyloid)

12
Q

t(11;14) in myeloma is associated with:

A
Associated with:
Plasma cell leukaemia, 
CD20 expression,
Lymphoplasmacytic morphology
Hyposecretory disease
Lambda light chains