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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
What is Waldenstrom Macroglobulinemia?
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
What are the types of non-Hodgkin T cell lymphoma?
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
What causes adult T cell lymphoma?
Etiology is mostly unknown but there does seem to be a link to HTLV1 which infects T cells and causes a mutation
28
What is mycosis fungoides?
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
Whats the neoplastic cell in mycosis fungoides? And what does this look like under a microscope?
CD4+ helper T cell Has a cerebriform nucleus (looks like a brain)
30
What is Sezary syndrome?
When neoplastic CD4+ helper T cells from mycoses fungoides circulate in the blood It causes erythroderma (generalised red rash) or itchy skin
31
What symptoms does non-Hodgkin lymphoma cause?
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
How is non-hodgkin lymphoma diagnosed?
Imaging scans to help establish the stage based on nodal and extranodal involvement Lymph node biopsy
33
How is non-Hodgkin lymphoma treated?
Watch and wait Radiotherapy for localised stage 1 and 2 disease Rituximab-based chemotherapy for symptomatic generalised disease
34
What is rituximabs moa?
It’s a monoclonal antibody that binds CD20 and induces complement-mediated lysis, direct cytotoxicity and apoptosis
35
What are the 3 types of Burkitt lymphoma?
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
What is Sjögren’s syndrome? What is it associated with?
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
What is extranodal marginal zone lymphoma of gastric MALT closely associatedto?
Helicobacter pylori infection
38
What are the 2 most common types of peripheral T cell lymphomas?
Peripheral T cell lymphoma, Not Otherwise Specified Angio-immunoblastic T cell lymphoma
39
When should you refer someone for suspected non-hodgkins lymphoma?
2WW in adults presenting with unexplained lymphadenopathy or splenomegaly
40
How are peripheral T cell lymphomas treated?
Cyclic combination chemotherapy Can’t use rituximab as T cells don’t express CD20
41
How do you treat low grade lymphomas?
Radiotherapy which almost always induces complete remission
42
How do you treat high grade lymphomas?
R-CHOP regimen Rituximab, Cyclophosphamide, Hydroxydaunorubicin, vincristine, prednisolone
43
How do we grade non-Hodgkins lymphoma?
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
Outline the 2 week wait guidelines for non-Hodgkin lymphoma?
Any adults with unexplained lymphadenopathy or splenomegaly
45
How do we treat lymphoplasmacytic lymphoma?
If severe them plasmapheresis to lower paraprotein levels Rituximab chemotherapy regimen
46
How do we treat marginal zone lymphoma?
Helicobacter eradication if this is cause Rituximab chemotherapy or radiotherapy
47
What are the complications of lymphoplasmacytic lymphoma?
Autoimmune haemolysis Raynaud phenomenon Amyloidosis of heart, kidney, liver, lungs and joints
48
Whats the ‘hyperviscocity syndrome triad’? What causes it?
Retinopathy (venous congestion of retinal veins) Neurological symptoms (venous congestion of cerebral veins) Mucosal bleeding (IgM antibodies interfere with coagulation) Lymphoplasmacytic lymphoma
49
Whats the most likely cause of tumour lysis syndrome?
occurs in hematological malignant patients particularly non-Hodgkin's lymphoma and acute leukemia due to chemotherapy or spontaneously
50
Which clusters of differentiation are associated with Reed-Sternberg cells?
CD15 and CD30
51
Which clusters of differentiation are associated with mantle cell lymphoma?
CD5
52
What are the risk factors for non-Hodgkin lymphoma?
Family history HIV EBV H pylori hep B or C Exposure to pesticides and or trichloroethylene
53
On average, what’s the prognosis of Non-Hodgkin lymphoma for 5 years after diagnosis?
65% will survive their cancer for 5 years or more after diagnosis
54
What causes basophilic vacuolated lymphoma cells and nucleated red cells with left shift?
Burkitts lymphoma
55
What are poor prognostic factors?
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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