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METABOLIC > HAEMATOLOGY - NON-HODGKIN LYMPHOMA > Flashcards

HAEMATOLOGY - NON-HODGKIN LYMPHOMA Flashcards

1
Q

Whats the difference between Hodgkin and Non-Hodgkin lymphoma?

A

Hodgkin lymphoma is marked by the presence of Reed-Sternberg lymphocytes and in non-Hodgkin lymphoma these cells are not present.
Non-Hodgkin lymphoma is derived from lymphocytes

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2
Q

What is CD short for?

A

Cluster of Differentiation

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3
Q

Why are there different subtypes of Non-Hodgkin lymphoma?

A

B cell:
- diffuse large B cell (aggressive)
- follicular (indolent)
- Mantle cell (highly aggressive)
- Burkitt (highly aggressive)
- lymphoplasmacytic (indolent)
- marginal zone (indolent)

T cell:
- adult T-cell
- peripheral T cell
- anaplastic large cell
- extranodal natural killer/T cell
- enteropathy-associated T cell lymphoma

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4
Q

What are nodal lymphomas?

A

When lymphomas develop in the lymph nodes

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5
Q

What are extranodal lymphomas?

A

When lymphomas develop somewhere other than the lymph nodes

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6
Q

What happens if lymphomas spread to the GIT?

A

They can cause bowel obstruction

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7
Q

What happens if lymphomas spread to the bone marrow?

A

They crowd out normal cells causing cytopenias

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8
Q

What happens if lymphomas spread to the spinal cord?

A

They can cause spinal cord compression

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9
Q

What are the 2 types of Non-Hodgkin lymphomas?

A

B cell and T cell

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10
Q

Which non-Hodgkin lymphoma is most common?

A

B cell

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11
Q

In B cell non-Hodgkin lymphoma, what surface markers are expressed?

A

CD20

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12
Q

How do we classify non-Hodgkin B cell lymphomas based on how fast they grow?

A

Indolent (slow)
Aggressive
Highly aggressive

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13
Q

What are the types of non-Hodgkin B cell lymphomas?

A

Diffuse large B cell lymphoma
Follicular lymphoma
Burkitt lymphoma
Mantle cell lymphoma
Lymphoplasmacytic lymphoma
Marginal zone lymphoma

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14
Q

Outline the characteristics of diffuse large B cell lymphoma? How aggressive?

A

It’s the most common adult lymphoma worldwide and second most common in children (after Burkitt)
Incidence increases with age
It’s aggressive

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15
Q

Outline the characteristics of follicular lymphoma?

A

Indolent
20% lymphomas worldwide
Occurs middle-late life (rare in childhood)
Median survival exceeds 10 years
Develops from t(14;18)

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16
Q

Whats the pathophysiology of follicular lymphoma?

A

Develops from t(14;18) = overexpression of BCL2 = BCL2 prevents apoptosis

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17
Q

Outline the characteristics of burkitt lymphoma?

A

Highly aggressive - the most rapidly proliferating lymphoma
Most common childhood maliganncy worldwide But occurs at all ages
Male: female 3:1
Results from t(8;14)

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18
Q

Whats the pathophysiology of Burkitt lymphoma?

A

T(8;14) This upregulates the expression of MYC gene
MYC gene is a transcription factor and so stimulates cell division

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19
Q

Outline the difference between burkitt lymphoma in Africa and outside of Africa?

A

In Africa it usually has extranodal involvement of the jaw. Often associated with Epstein Barr virus

Outside Africa it causes extranodal involvement of the abdomen (usually ileocecal junction) and is less associated with EBV

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20
Q

What does Burkitt lymphoma look like histologically?

A

It has a starry sky appearance
This is because there are tangible bodies (macrophages that have eaten neoplastic cells) in between neoplastic lymphocytes

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21
Q

What are the characteristics of Mantle cell lymphoma?

A

Aggressive
Usually presents later in life
Male: female 3:1
Prognosis 8-12 years
Cause is from t(11;14) - bcl1

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22
Q

Outline the pathophysiology behind mantle cell lymphoma?

A

CCND1 gene from chromosome 11 ends up next to the immunoglobulin promoter on chromosome 14. This upregulates expression of CCND1 which encodes cyclin D1 which stimulates cell growth
This results in increased cell division

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23
Q

What are the characteristics of marginal zone lymphoma?

A

Indolent
Uncommon
Associated with chronic infection or inflammation
MALT is the commonest form

24
Q

What ate the characteristics of lymphoplasmacytic lymphoma?

A

Indolent
Usually occurs later in life
Known as waldenstroms macroglobulinaemia when associated with IgM paraprotein and bone marrow infiltration
May be preceded by monoclonal gammopathy of undetermined significance

25
Q

What is Waldenstrom Macroglobulinemia?

A

a type of non-Hodgkin lymphoma aka lymphoplasmacytic lymphoma (a proliferative disease of B lymphocytes)
Characterised by an overproduction of IgM. IgM are Macroglobulin which increase the thickness of blood and they aggregate with one another causing hyperviscocity syndrome. This causes blood vessels to become engorged and leads to hypercoagulability

Note: different from myeloma as doesnt cause bone lytic lesions

26
Q

What are the types of non-Hodgkin T cell lymphoma?

A

Adult T cell lymphoma
Mycosis fungoides
Angioimmunoblastic T cell lymphoma
extranodal natural killer or T cell lymphoma
Enteropathy-associated intestinal T cell lymphoma
Anaplastic large cell lymphoma
Peripheral T cell lymphoma, not otherwise specified (those that don’t readily fit into any of the groups above)

27
Q

What causes adult T cell lymphoma?

A

Etiology is mostly unknown but there does seem to be a link to HTLV1 which infects T cells and causes a mutation

28
Q

What is mycosis fungoides?

A

A T cell lymphoma of the skin that causes patches on the skin that look a bit like a fungal ifnection
The most common cutaneous lymphoma

29
Q

Whats the neoplastic cell in mycosis fungoides?
And what does this look like under a microscope?

A

CD4+ helper T cell

Has a cerebriform nucleus (looks like a brain)

30
Q

What is Sezary syndrome?

A

When neoplastic CD4+ helper T cells from mycoses fungoides circulate in the blood
It causes erythroderma (generalised red rash) or itchy skin

31
Q

What symptoms does non-Hodgkin lymphoma cause?

A

Painless lymphadenopathy
Fever
Drenching night sweats
Weight loss

Extranodal symptoms:
GIT - bowel obstruction
Bone marrow - fatigue, easy bruising, recurrent infections
Spinal cord - loss of sensation usually in the legs

32
Q

How is non-hodgkin lymphoma diagnosed?

A

Imaging scans to help establish the stage based on nodal and extranodal involvement
Lymph node biopsy

33
Q

How is non-Hodgkin lymphoma treated?

A

Watch and wait
Radiotherapy for localised stage 1 and 2 disease
Rituximab-based chemotherapy for symptomatic generalised disease

34
Q

What is rituximabs moa?

A

It’s a monoclonal antibody that binds CD20 and induces complement-mediated lysis, direct cytotoxicity and apoptosis

35
Q

What are the 3 types of Burkitt lymphoma?

A

Endemic Burkitt lymphoma primarily affects African children ages 4 to 7 and is twice as common in boys. Usually presents with a rapidly growing jaw tumour

Sporadic (non-African). Sporadic Burkitt lymphoma occurs worldwide.

Immunodeficiency-associated. This variant of Burkitt lymphoma is most common in people with HIV/AIDS. It accounts for 30% to 40% of non-Hodgkin lymphoma in HIV patients and may be an AIDS-defining disease. It also can occur in people with congenital conditions that cause immune deficiency and in organ transplant patients who take immunosuppressive drugs.

36
Q

What is Sjögren’s syndrome? What is it associated with?

A

a systemic autoimmune, rheumatic disease that affects the entire body. The most common symptoms are dry eyes and dry mout

It has a strong link to extranodal marginal zone lymphoma of MALT of the salivary glands

37
Q

What is extranodal marginal zone lymphoma of gastric MALT closely associatedto?

A

Helicobacter pylori infection

38
Q

What are the 2 most common types of peripheral T cell lymphomas?

A

Peripheral T cell lymphoma, Not Otherwise Specified
Angio-immunoblastic T cell lymphoma

39
Q

When should you refer someone for suspected non-hodgkins lymphoma?

A

2WW in adults presenting with unexplained lymphadenopathy or splenomegaly

40
Q

How are peripheral T cell lymphomas treated?

A

Cyclic combination chemotherapy
Can’t use rituximab as T cells don’t express CD20

41
Q

How do you treat low grade lymphomas?

A

Radiotherapy which almost always induces complete remission

42
Q

How do you treat high grade lymphomas?

A

R-CHOP regimen
Rituximab, Cyclophosphamide, Hydroxydaunorubicin, vincristine, prednisolone

43
Q

How do we grade non-Hodgkins lymphoma?

A

Ann Abor staging
1 - single lymph node region
2 - 2+ lymph nodes regions on the same side of diaphragm
3 - 2+ lymph node regions on either side of diaphragm
4 - widespread disease - multiple organs

44
Q

Outline the 2 week wait guidelines for non-Hodgkin lymphoma?

A

Any adults with unexplained lymphadenopathy or splenomegaly

45
Q

How do we treat lymphoplasmacytic lymphoma?

A

If severe them plasmapheresis to lower paraprotein levels
Rituximab chemotherapy regimen

46
Q

How do we treat marginal zone lymphoma?

A

Helicobacter eradication if this is cause
Rituximab chemotherapy or radiotherapy

47
Q

What are the complications of lymphoplasmacytic lymphoma?

A

Autoimmune haemolysis
Raynaud phenomenon
Amyloidosis of heart, kidney, liver, lungs and joints

48
Q

Whats the ‘hyperviscocity syndrome triad’? What causes it?

A

Retinopathy (venous congestion of retinal veins)
Neurological symptoms (venous congestion of cerebral veins)
Mucosal bleeding (IgM antibodies interfere with coagulation)

Lymphoplasmacytic lymphoma

49
Q

Whats the most likely cause of tumour lysis syndrome?

A

occurs in hematological malignant patients particularly non-Hodgkin’s lymphoma and acute leukemia due to chemotherapy or spontaneously

50
Q

Which clusters of differentiation are associated with Reed-Sternberg cells?

A

CD15 and CD30

51
Q

Which clusters of differentiation are associated with mantle cell lymphoma?

A

CD5

52
Q

What are the risk factors for non-Hodgkin lymphoma?

A

Family history
HIV
EBV
H pylori
hep B or C
Exposure to pesticides and or trichloroethylene

53
Q

On average, what’s the prognosis of Non-Hodgkin lymphoma for 5 years after diagnosis?

A

65% will survive their cancer for 5 years or more after diagnosis

54
Q

What causes basophilic vacuolated lymphoma cells and nucleated red cells with left shift?

A

Burkitts lymphoma

55
Q

What are poor prognostic factors?

A

B sympathomimetic
Lymphadenopathy/organomegaly
Advanced tumour stage
Advanced age
Anaemia
Raised serum LDH (its an indirect indicator of the rate of proliferation of the lymphoma)

METABOLIC (43 decks)

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