Haematology 11 - Plasma cell myeloma and Monoclonal Gammopathy of Uncertain Significance Flashcards Preview

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Flashcards in Haematology 11 - Plasma cell myeloma and Monoclonal Gammopathy of Uncertain Significance Deck (42)
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1

What immunoglobin is produced by myeloma cells?

One single type (eith IgG or IgA) which is known as paraprotein or M spike

2

What are Bence Jones proteins?

Urine monoclonal free light chains

3

What is Waldenstrom’s-Lymphoplacytic lymphoma?

A lymphoplasmocytic lymphoma with IgM paraprotein that causes visual disturbances

4

What is the name of the premalignant condition that always precedes myeloma?

Monoclonal gammaopathy of uncertain significance (MGUS)

5

What are the two most significant risk factors for myeloma?

Obesity
Black > causasians/ asians

6

What are the diagnostic criteria for MGUS?

Serum M-protein <30g/L
BM clonal plasma cells <10%
Asymptomatic

7

What is smouldering myeloma?

Serum M-protein >30g/L
BM clonal plasma cells >10%
Asymptomatic

8

What % of plasma cells is there in symptomatic multiple myeloma?

>10%

9

What is the most notable interaction of myeloma cells with the bone marrow micro-environment?

Produce RANK ligand which stimulates osteoclasts to cause bone resorptions

10

What is the incidence of IgM myeloma?

Very rare (<1% of myelomas)

11

What does CRAB stand for in myeloma diagnosis?

Calcium (hypercalcaemia, >2.75)

Renal (creatinine >177/ eGFR <40)

Anaemia

Bone disease (see lytic lesions)

12

What is the most common and 2nd most common cytogenetic abnormality in myeloma?

1. Hyperdiploid karyotype
2. IgH gene rearrangement

13

What are the 3 2014 Myeloma Defining Events

BM plasma cells >60%
involved:uninvolved FLC ratio >100
>1 focal lesion on MRI

14

Which part of the skeleton is affected by myeloma?

Proximal skeleton (spine, skull, knees)

15

Where are myeloma patients most likely to feel pain?

Back, chest wall, pelvis

16

What % of myeloma patients present with bone disease?

80%

17

What scan is necessary to detect bone lesions in myeloma?

Whole body CT is first line (X ray is obsolete for this use)
PET scan can also be used
Gold-standard = whole body diffusion-weighted MRI as this shows active vs treated disease

18

What are the 2 most likely emergency presentations of myeloma?

Cord compression

Hypercalcaemia

19

What tests should be done to diagnose myeloma?

First:
Serum protein electrophoresis
Serum free light chains
Next:
Bone marrow aspirate and biopsy for immunohistochemistry
FISH (for prognostic)

20

What is the best way to treat cord compression in myeloma?

Dexamethosone
Radiotherapy

21

What risk does myeloma present to the kidney?

Cast nephropathy - FLCs and Bence Jones proteinuria cause proximal tubule cell injury

22

How should myeloma kidney disease be treated in an emergency?

Bortezomib-based therapy is the cornerstone of myeloma kidney disease treatment - once the patients can become independent from dialysis their outcomes improve dramatically

23

How does myeloma affect immunity?

Serum levels of normal Igs reduced

BM micro-environment interference also impairs myeloid, T and NK cells

Chemo also impairs immune response

24

What is tested for in BM biopsy in suspected myeloma?

Immunohistochemistry for CD138 - specific for myeloma cells in BM

25

What test can detect cytogenetic abnormalities of prognostic significance in myeloma?

FISH

26

What are the 3 parameters in the international staging system for myeloma?

Serum beta microglobulin

Cytogenetic risk

LDH

27

How does AL amyloidosis link to myeloma?

Misfolded FLCs aggregate into amyloid in target organs

28

How does amyloidosis affect the kidney?

Nephrotic syndrome

29

Give 5 features of the clinical presentation of AL amyloidodis?

Nephrotic syndrome
Unexplained heart failure
Sensory neuropathy
Abnormal liver function tests
Macroglossia

30

What is MGRS?

Monoclonal gammaopathy of Renal significance

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