41 Lymphoma and myeloma Flashcards

1
Q

Describe the process of B cell differentiation:

A

Pro-B gains surface immunoglobulins to become naive B cell in bone marrow.
Then antigens are presented to it in a secondary lymphoid organ.
Last stage is NF-κB pathway.

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2
Q

Name the proteins found during electrophoresis of blood serum: (5).

A
Serum albumin.
Alpha-1 globulins.
Alpha-2 globulins.
Beta globulins.
Gamma globulins.
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3
Q

When is immunofixation performed and what does it detect/identify.

A

When an M spike is seen on protein electrophoresis.

Monoclonal immunoglobulins.

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4
Q

What is myeloma?

A

Malignant disorder of clonal plasma cells.

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5
Q

What is the epidemiology of myeloma?

A

70 years.
Higher in Afro-Carribeans.
Preceeded by asymptomatic MGUS.

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6
Q

What is MGUS?

A

Monoclonal gammopathy of uncertain significance.

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7
Q

What are the diagnostic criteria for a diagnosis of multiple myeloma?

A

Clonal bone marrow plasma cells or plasmacytoma and at least one CRAB/MDE feature.

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8
Q

What are the CRAB features of multiple myeloma?

A

HyperCalcaemia.
Renal insufficiency.
Anaemia.
Bone lesions.

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9
Q

What are MDE’s (myeloma defining events)? (3).

A

> 60% clonal plasma cells on bone marrow biopsy.
SFLC ratio >100mg/L.
Bone marrow lesion >5mm.

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10
Q

Why does renal insufficiency occur in multiple myeloma?

A

Light chains clog the kidney.

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11
Q

What is the treatment for AKI in myeloma?

A

Steroids.

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12
Q

How is myeloma intensively treated? (3)

A

VCD.
GCSF.
Mephalan.

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13
Q

What are the diagnostic criteria for MGUS?

A

Serum M-protein under 30g/L
Under 10% clonal plasma cells in BM
Absence of CRAB features

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14
Q

What are the risks of progression from MGUS? (1,3)

A

1% per year, majority to myeloma.

Others to: Waldenstrom’s macroglobulinaemia, primary AL amyloidosis, lymphoproliferative disorders.

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15
Q

What are the higher risk features for MGUS progressing? (3).

A

High M-protein level.
IgA/IgM rather than IgG production.
Abnormal SFLC ratio.

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16
Q

What is the pathology behind amyloidosis?

A

Light chain fragments misfold and self aggregate into β-pleated fibrils.

17
Q

What does amyloidosis cause? (4).

A

Nephrotic-range proteinuria (mainly albumin).
Cardiac/liver problems.
Peripheral neuropathy.
End-stage renal failure.

18
Q

What type of lymphoma is follicular lymphoma?

A

Non-hodgkin.

19
Q

What is the genetic change in follicular lymphoma?

Effect?

A
Translocation t(14:18).
Brings bcl-2 under influence of immunoglobulin heavy chain gene. Over expression inhibits apoptosis.
20
Q

What features of follicular lymphoma are bad for prognosis? (5).

A
> 60y/o.
Ann Arbor stage III or IV.
LDH above normal limit.
Hb less than 120g/L
Mor than 4 nodal sites.
21
Q

What is Hodgkin lymphoma characterised by?

A

Presence of Hodgkin-Reed Sternberg cells within cellular infiltrate of non malignant inflammatory cells.

22
Q

How do Hodgkin-Reed Sternberg cells evade apoptosis? (3).

A

Activation of NF-κB.
Incorporation of EBV.
Incorporation of latent membrane proteins (LMP1/2).

23
Q

Which chemotherapy is commonly used for Hodgkin lymphoma?

Long term complications? (3).

A

ABVD.

Pulmonary toxicity, CVD, 2o malignancies.

24
Q

What is M-protein?

A

Monoclonal immunoglobulin synthesised by plasma cells in myeloma.

25
Q

What are the common causes of lymphadenopathy? (3)

A

Infections.
Autoimmune disease (SLE, serum sickness).
Neoplasias (lymphoma, leukaemia).