Haematological Malignancies 3: lymphoma and myeloma Flashcards

1
Q

How is lymphoma different to infection?

A

Tenderness in lymph nodes indicates infection.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What are malignant lymphomas?

A

Replacement of normal lymphoid tissue by abnormal cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What are the main types of lymphoma?

A

Non-hodgkin lymphoma (many sub-types)

Hodgkin lymphoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What is the difference between leukaemias and lymphomas?

A

Leukaemias involve the bone marrow and the blood.

Tissue sites are affected by lymphomas.

There is some overlap; CLL starts in the bone marrow and spreads to lymphoid tissues making it look identical.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

How is NonHodgkin’s Lymphoma classified?

A

B vs T cell (WHO)

Cells: morphology and immunology and stage of normal lymphoid cell maturation.

Grade: low vs high (aggressive)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What virus can cause NHL to develop?

A

Ebstein Barr virus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What bacterial infection can cause NHL?

A

Helicobacter Pylori

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What is a possible reason for the rising incidence of NHL?

A

The aging population (because NHL correlates with age)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What are the clinical features of NHL?

A

Enlarged lymph nodes.

Hepato-splenomegaly

Systemic symptoms (Fever, night sweats, weight loss) [cytokine release by cells of lymphoma]

Lymphadenopathy

Interference with normal organ function

Bone marrow failure

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

How is NHL diagnosed?

A

Pathology of tissue involved:

Pattern

Cell size: small, large

Cell differentiation: well or poorly defined

Cell phenotype: B / T cells

Genetics: karyotyping and FISH

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

How is NHL staged?

A

Extent of disease (radiological imaging to look for spread to other tissue from the source)

Imaging: CT or PET scan of neck, chest, abdomen, and pelvis

BM sample

Lumbar puncture (CSF indicates potential spread to brain)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What is follicular lymphoma?

A

A low grade B cell NHL which has is caused by a t(14,18) translocation.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What is Burkitt lymphoma?

A

A NHL lymphoma commonly caused by Ebsteinn Barr Virus which is endemic to Africa and is quite aggressive.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What is diffuse large B cell lymphoma?

A

A type of NHL that is increasing in number and is quite aggressive

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

How common is follicular lymphoma?

A

2nd most common type of NHL

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

How does follicular lymphoma look at diagnosis?

A

Usually widely disseminated at diagnosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

How is follicular lymphoma diagnosed?

A

CD20, CD10, and BCL2 positive on flow cytometry.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What is the 5-year survival rate of follicular lymphoma?

A

70 - 80%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What is follicular lymphoma growth like?

A

It can be slow growth and then transform to an aggressive NHL

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

How is follicular lymphoma treated?

A

Watch and wait if indolent

Options:

Radiotherapy

Chemotherapy (CHOP regimen)

Antibody-based therapy: anti-CD20 (rituximab)

Combination therapy with antibody + chemotherapy

Transplant options

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

What indicates treatment of NHL?

A

Constitutional symptoms such as painful lymph nodes.

Anatomical obstruction or organ dysfunction

Cytopenias

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

What pattern is typical of follicular lymphoma trephine?

A

Follicular pattern

Predominantly small cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

What translocation is typical of follicular lymphoma?

A

t(14;18)(q32,q21)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

What protein expression is up-regulated by follicular lymphoma?

A

Up-regulates expression of anti-apoptotic protein Bcl-2

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

What is Burkitt lymphoma?

A

A Non-Hodgkin lymphoma derived from Germinal center of lymphatic tissue

26
Q

What causes Burkitt lymphoma?

A

In African populations it is commonly associated with EBV whereas in adults it is not.

27
Q

What changes are characteristic of Burkitt’s lymphoma?

A

Swollen face and enlarged lymph nodes

In adults it commonly affects the eyes.

It grows very fast

28
Q

What gene is activated in Burkitt’s lymphoma?

A

there is a translocation on chromosome 14 where the immunoglobulin heavy chain sits. As a result the oncogene MYC-IGH is activated on chromosome 8 due to the translocation causing the MYC-IGH gene to be continuously expressed resulting in cells constantly being in mitosis stage.

29
Q

What characteristic blood slides are seen in people with Burkitt’s lymphoma?

A

Monotonous B cell infiltrate which looks like a highly vacuoled (fat filled) starry-sky pattern

30
Q

What is the treatment for Burkitt’s lymphoma?

A

Aggressive chemotherapy which can completely cure the disease

31
Q

How common is diffuse large B cell lymphoma?

A

The most common lymphoma which is 30% of all NHLs

32
Q

Who gets large B cell lymphoma most commonly?

A

Adult elderly people

33
Q

How does large B cell lymphoma present?

A

Rapidly enlarging masses

34
Q

What is going wrong during diffuse large B cell lymphoma?

A

diffuse infiltration by large cells (B cells)

Diverse histology with high proliferation rate

35
Q

How is diffuse large B cell lymphoma treated?

A

Combination chemotherapy (eg CHOP)

36
Q

What is the rate of effectiveness of combination chemotherapy in treating diffuse large B cell lymphoma?

A

60 - 70% complete remission rate and approximately 30% curable.

37
Q

What factors are highly influential on prognosis for NHL? (not assessed)

A

International prognostic index

Clinical features used include:

Age <60 vs >60

Performance status

LDH: lactate dehydrogenase is elevated in people with lymphoma

Disease stage:

Extra-nodal involvement:

(not assessed) 1 point for each bad feature

38
Q

What is hodgkin’s lymphoma/

A

Reed-Sternberg cell: minor cell component found in appropriate microscopic cellular background. (pathognomonic of this disease)

Bimodal age incidence: Young adults and after age 50

39
Q

What does pathognomonic mean?

A

Characteristic of the disease

40
Q

What are the clinical features of Hodgkin’s lymphoma?

A

Painless, non-tender, rubbery lymph nodes. (more common in cervical than axillary lymph nodes)

Dyspnoea (when mediastinum is involved)

Splenomegaly (rare)

Constitutional symptoms (B cell symptoms) fever, pruritis and weight loss.

41
Q

What tests are commonly done for Hodgkin’s lymphoma diagnosis?

A

Immunophenotyping: Reed-Sternberg cells express CD15 and CD30.

Clinical staging: CT, MRI, PET

42
Q

How is hodgkin’s lymphoma staged?

A

In 1 location (usually cervical region) is stage I

In 2 locations but both above or below diaphragm ios stage II

Above and below the diaphragm is stage III

If the liver or the bone marrow is involved it is considered stage IV

The higher the stage the more treatment required and the less likely for illness to be completely cured.

43
Q

How is hodgkin’s lymphoma treated?

A

Chemotherapy and radiotherapy to site of disease.

44
Q

What percentage of people with hogkin’s lymphoma are completely cured?

A

80 - 90% especially stage I and II

45
Q

What is the danger associated with hogkin lymphoma treatment?

A

It can result in other forms of leukemia and lymphoma (lung and breast cancers common which is dose - response related)

Damage to bone marrow resulting in acute myeloid leukaemia

46
Q

What is multiple myeloma caused by?

A

Disease of end stage B cells

Homogeneous immunoglobulin are produced from a single clone (paraproteins)

47
Q

What is the prognosis like of Hodgkin’s lymphoma?

A

It is a curable malignancy

Cure rate is approximately 80%

Prognosis based on staging and bulk of disease

Infections: Reduced cell-mediated immunity

Relapsed disease: difficult to treat so it has a more poor prognosis especially if it results in secondary malignancies in lungs and breast

48
Q

What immunoglobins are most common in multiple myeloma?

A

IgG (but it isn’t always IgG it can be others)

49
Q

What are the clinical features of multiple myeloma?

A

Free light chains can be seen known as Bence Jones proteins which are small enough to be cleared by kidneys.

IgG antibodies present in large quanitities in the blood.

Lytic bone lesions which cause pathological fractures and renal failure is also common

Clinical problems from organ infiltration by neoplastic plasma cells and this is common in bone marrow (for obvious reasons)

Production of excess immunoglobulin

calcium loss and bone wasting

Recurrent infections are common

50
Q

What is the common demographic associated with multiple myeloma?

A

People that are >60 years of age

51
Q

What happens to haemoglobin in people with multiple myelomas?

A

It drops

52
Q

What happens to Mean Cell Volume in people with multiple myeloma?

A

it is elevated

53
Q

What happens to white blood cells in multiple myelomas?

A

Decreased

54
Q

What happens to platelet count in people with multiple myelomas?

A

They decrease dramatically

55
Q

What does the bone marrow look like in multiple myeloma?

A

Bone marrow is filled with activated B cells

56
Q

What x-rays are performed in people with multiple myelomas?

A

Head and neck x-rays; damage to bone is common in these people

57
Q

What is the result of lytic lesions?

A

The bone can be broken as a result of small amount of trauma

58
Q

What kidney problem is associated with multiple myeloma?

A

Large amount of protein cleared in the urine.

Lambda light chains (Bence Jones protein) are toxic to renal epithelium

Amyloid deposits in the glomeruli

Hypercalcemia

59
Q

What is the median survival of multiple myeloma?

A

5 years.

Very low survival rate

60
Q

How do people die from multiple myeloma?

A

Kidney failure

Haemorrhage

Infections