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Path Y > Haematology - Plasma cell myeloma and Monoclonal Gammopathy of Uncertain Significance > Flashcards

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

1
Q

What immunoglobin is produced by myeloma cells?

A

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

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2
Q

What are Bence Jones proteins?

A

Urine monoclonal free light chains

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3
Q

What is -Lymphoplasmacytic lymphoma?

A

A type of non-Hodgkin lymphoma with IgM paraprotein that causes visual disturbances

aka Waldenstrom’s macroglobunlinaemia

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4
Q

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

A

Monoclonal gammaopathy of uncertain significance (MGUS)

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5
Q

What are the two most significant risk factors for myeloma?

A

Obesity
Black > causasians/ asians

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6
Q

What are the diagnostic criteria for MGUS?

A

Serum Monoclonal-protein (often IgG)<30g/L
BM clonal plasma cells <10%
Asymptomatic

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7
Q

What is smouldering myeloma?

A

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

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8
Q

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

A

>10%

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9
Q

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

A

Produce RANK ligand which stimulates osteoclasts to cause bone resorptions

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10
Q

What is the incidence of IgM myeloma?

A

Very rare (<1% of myelomas)

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11
Q

What does CRAB stand for in myeloma diagnosis?

A

Calcium (hypercalcaemia, >2.75)

Renal (creatinine >177/ eGFR <40)

Anaemia

Bone disease (see lytic lesions)

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12
Q

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

A
  1. Hyperdiploid karyotype
  2. IgH gene rearrangement
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13
Q

What are the 3 2014 Myeloma Defining Events

A

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

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14
Q

Which part of the skeleton is affected by myeloma?

A

Proximal skeleton (spine, skull, knees)

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15
Q

Where are myeloma patients most likely to feel pain?

A

Back, chest wall, pelvis

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16
Q

What % of myeloma patients present with bone disease?

A

80%

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17
Q

What scan is necessary to detect bone lesions in myeloma? What is gold standard

A

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

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18
Q

What are the 2 most likely emergency presentations of myeloma?

A

Cord compression

Hypercalcaemia

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19
Q

What 5 investigations to diagnose myeloma?

A

Serum protein electrophoresis (dense bands of IgG or IgA monoclonal proteins)
blood film - rouleaux stacks
bence jones proteins in urine
high ESR
more than 10% plasma cells in BM biopsy

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20
Q

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

A

Dexamethosone
Radiotherapy

21
Q

What risk does myeloma present to the kidney?

A

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

22
Q

How should myeloma kidney disease be treated in an emergency?

A

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

23
Q

How does myeloma affect immunity?

A

Serum levels of normal Igs reduced

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

24
Q

What is tested for in BM biopsy in suspected myeloma?

A

Immunohistochemistry for CD138 - specific for myeloma cells in BM

25
Q

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

A

FISH

26
Q

How does AL amyloidosis link to myeloma?

A

Misfolded free light chains aggregate into amyloid in target organs

27
Q

How does amyloidosis affect the kidney?

A

Nephrotic syndrome

28
Q

Give 5 features of the clinical presentation of AL amyloidodis?

A

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

29
Q

What is MGRS?

A

Monoclonal gammaopathy of Renal significance

30
Q

How is MGRS defined?

A
  1. One or more kidney lesions caused by mechanisms related to the produced monoclonal immunoglobulin (Ig)
  2. The underlying B cell clone does not cause tumor complications or meet current hematological criteria for immediate specific therapy
31
Q

How do proteasome inhibitors work in multiple myeloma

A

Proteasomes are involved in removing misfolded proteins

If you inhibit this, you get accumulation of misfolded proteins in myeloma cell leading to their death

32
Q

Which monoclonal antibody is used in myeloma treatment?

A

Anti-CD38 (daratumumab)

33
Q

What are the 3 risk factors for MGUS progression?

A
  1. Non-IgG M spike
  2. Monoclonal spike >15g/L
  3. Abnormal serum free light chain ratio
34
Q

What is the cause of de novo angiogenesis in myeloma?

A

CD34 expression on myeloma cells promotes angiogenesis

35
Q

What type of nephropathy does myeloma cause?

A

Cast nephropathy

36
Q

What are the first investigations to be done in suspected myeloma?

A

Serum protein electrophoresis
Serum free light chains

37
Q

What condition can produce MGUS/ myelomas part of its progression?

A

B cell leukaemia

38
Q

Recall 5 options for myeloma therapy

A
  1. Alkylating agents - melphalan
  2. Steroids (eg dex + pred)
  3. Immunomodulatory drugs -Thalidomide
  4. Proteosome inhibitors (bortezomib)
  5. Anti-CD38 monoclonal antibody (daratumumab) cause apoptosis through complement cascade
39
Q

6 features of myeloma

A

monoclonal plasma proteins (IgG, IgA)
paraprotein - bence jones
osteolytic lesions
anaemia
infections
kidney failure

40
Q

MM is a cancer of what cells specifically

A

transformed plasma cells (terminally differentiate B cells)

41
Q

median survival of MM

A

4-7 years
debilitating and incurable

42
Q

outline the 4 steps of progression of myeloma

A
  1. MGUS
  2. Smoudlering myeolma
  3. MM
  4. B cell leukaemia
43
Q

which step of myeloma has CRAB symptoms

A

MM does
not in MGUS or smouldering

44
Q

median age diagnosis of myeloma

A

old - 60-70

45
Q

what staging system in MM

A

durie salmon

46
Q

2 genes implicated in MM

A

p16 methylation - 40%
N-RAS, K-RAS - 30%

47
Q

plasma and myeloma cells have what highly developed organelle

A

secretory apparatus - large endoplasmic reticulum & (golgi)

48
Q

list 5 myeloma markers seen in MM

A

CD 38
CD138
CD56/58
monotypic cytoplasmic Ig
light chain restriction (kappa or lambda +ve)

49
Q

list 3 markers NOT seen in MM (negative)

A

CD 19
CD 20
Surface Ig

Path Y (81 decks)

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