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Path - Haematology > Plasma Cell Myeloma and Amyloid > Flashcards

Plasma Cell Myeloma and Amyloid Flashcards

1
Q

what is multiple myeloma?

A

Cancer of the BM plasma cells, terminally differentiated and Ig secreting B cells

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2
Q

What kind of tumours do myeloma plasma cells produce?

A

Bone expansile / soft tissue tumours

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3
Q

What kind of Ig do myeloma plasma cells produce?

A

MONOCLONAL Ig

either IgA or IgG

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4
Q

What is the most important structure produced by myeloma plasma cells?

A

Excess of monoclonal (K/lambda) serum free light chains

aka BENCE JONES PROTEIN

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5
Q

What condition can predispose to MM?

A

Pre-malignant condition called:

Monoclonal Gammopathy of Uncertain Significance (MGUS)

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6
Q

What is median survival for MM?

A

4-7 years

POOR prognosis

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7
Q

Explain pathophysiology of MM

A

Early in plasma cell, error occurs in genome
This leads to monoclonal accumulation of plasma cells (MGUS)
1% of people every year acquire extra mutations (KRAS, NRAS) which transform MGUS into MM

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8
Q

What is the average age for myeloma?

A

67

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9
Q

What are RF for myeloma?

A

Age
Obesity
Genetic (sporadically familial)
Black

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10
Q

What proportion of patients a year go from MGUS too MM?

A

1%

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11
Q

What disease occurs if from MGUS there is excess IgM production?

A

Lymphoma

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12
Q

What bone marrow plasma cell percentage is required for MGUS classification?

A

<10%

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13
Q

What disease sits between MGUS and MM?

A

Smouldering myeloa

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14
Q

What is the progression of MM?

A

Incurable
Periods of remission-relapse
Becomes refractory to tx
Becomes extramedullary disease, with circulating plasma cells

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15
Q

What is the most common primary event that causes MM?

A

HYPERPLOIDY (additional odd number chromosome)

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16
Q

What is another primary event that causes MM?

A

heavy immunoglobulin chain rearrangement

17
Q

What occurs genetically in each pt with MM’

A

In each patient there are multiple subclones with genetic lesions
As tumour develops, more lesions accumulate in linear /branching fashion
This creates evolutionary pressure
Selects either indolent or aggressive clones

18
Q

What is the aetiology of MM causing bone destruction?

A

MM releases cytokines

cytokines stimulate osteoclasts to induce bone resorptiìon

19
Q

What percentage of plasma cells in bone marrow are required for MM dx?

A

> =10% plasma cells in bone marrow

20
Q

What are the clinical presentations of MM? ACRONYM

A

CRAB

Calcium elevated
Renal impairment
Anaemia
Bone lesions

21
Q

What diagnosis is a good indicator that pt will develop myeloma in coming months?

A

MDE (Myeloma Defining Event)

22
Q

What cellular markers is MM positive for?

A

CD138
CD38
CD56
CD58

23
Q

What cellular markers in MM negative for?

A

CD19
CD20
Surface IgG

24
Q

What does bone disease present like in MM?

A 
Proximal skeleton affected
Spine, chest wall, pelvic pain 
OsteoLYTIC lesions 
Osteopoenia 
Pathological fractures 
Hypercalcaemia
25
Q

What is the key bone emergency in MM?

A

Cord compression

Due to spine fracture > soft tissue enters spinal canal or soft tissue mass forms

26
Q

What is tx for cord compression in MM?

A

Dexamethasone
Radiotherapy
Neurosurgery

27
Q

How do you image MM bone lesions?

A

MRI or CT scan (or PET)

NOT plain X ray film. obsolete as low sensitivity

28
Q

What is the key cause of kidney injury in MM’

A

FLC and Bence Jones Proteins

29
Q

How do light chains cause kidney injury in MM?

A

High quantity of free light chains produced
Proximal tubule can no longer uptake them, as under high stress
FLC travel to distal tubule
Here they bind to THP
This complex obstructs the tubule and eventually destroys the nephron

30
Q

How do you treat kidney disease in MM?

A

BORTEXOIB

31
Q

What marker of mM do you look for in bone marrow aspirate?

A

CD138

32
Q

What does AL amyloidosis occur due to?

A

Misfolded free light chains aggregate into amyloid fibrils in target organs

33
Q

What kind of stain do you use for AL amyloidosis?

A

CONGO red

34
Q

What are 2 common target organs of AL amyloidosis=?

A

Kidney - nephrotic syndrome

Heart - heart failure

35
Q

What are the 4 key domains for MM treatment=?

A

Cytostatic drugs e.g. melphalan, cycophosphamide

Steroids

Immunomodulators e.g. thalidomide

Proeasome inhibitors

36
Q

What are other immunomodulators that are used for M M?

A

Lenalidomide

Pomalidomide

37
Q

What are examples of protease inhibitors?

A

Bortezomib

Path - Haematology (17 decks)

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