Haem: Lymphomas 2 - CLL and quiz Flashcards

1
Q

Why do we need to check LDH and B2 microglobulin in lymphomas?

A

Give an indication about how aggressive the lymphoma is and cell turnover (LDH is a marker of cell turnover

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2
Q

Why do you need a HIV serology in lymphomas?

A

as loss of T cell function can increase the incidence of B cell lymphomas

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3
Q

Why do you need a Hep B serology in lymphomas?

A

If B cell depleting therapy is carried out and the patient is a carrier of Hepatitis B, they may run risk of fatal liver failure due to reactivation of Hepatitis B

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4
Q

How will women normally present with HL?

A

Nodular sclerosing subtype

Cervical and mediastinal lymphadenopathy

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5
Q

How can HL present in general?

A

Patients present with PAINLESS enlargement of lymph node/nodes
This may cause obstructive symptoms/ signs
Great vessels (IVC, SVC, etc.)
Tracheal obstruction
Oesophageal obstruction
Bile duct obstruction
Constitutional symptoms (B symptoms):
Fever
Night sweats (drenching)
Unexplained weight loss (10% in 6 months)
+ pruritis may be present

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6
Q

What are the 4 subtypes of classical HL (cHL)?

A

Nodular sclerosing
Mixed cellularity
Lymphocyte rich
Lymphocyte depleted

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7
Q

How is cHL staged?

A

FDG-PET/CT scan leading to Ann-Arbor staging

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8
Q

How does cHL spread?

A

Contiguously

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9
Q

What are the different stages in cHL?

A

1 - One group of nodes
2 - >1 group of nodes same side of diaphragm
3 - Nodes above and below the diaphragm
4 - Extra nodal spread

A - If no systemic symptoms
B - If you have FLAWS

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10
Q

How is HL treated?

A

Chemotherapy - ABVD

Follow up with PET CT to see how effective treatment has been

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11
Q

What are the long term side effects of cHL?

A

Pulmonary fibrosis

Cardiomyopathy

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12
Q

Why is radio AND chemo not advised in terms of treatment?

A

Combined modality treatment (radiotherapy AND chemotherapy) leads to a VERY HIGH risk of secondary malignancy

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13
Q

Is the risk of relapse a bigger risk than secondary malignancy after treatment?

A

Until 10 years post treatment, risk of relapse is bigger, then secondary malignancy is the bigger risk

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14
Q

What are the 3 subtypes of NHL in terms of prognosis?

A

Very aggressive - Easier to treat lol
Aggressive
Indolent - Harder to treat lmao

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15
Q

What is the main difference in the way HL and NHL present?

A

Hodgkin’s tends to be contiguous and patients present with mediastinal masses. NHL is more widespread.

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16
Q

What are some very aggressive NHL?

A

Burkitt’s

B or T cell lymphoblastic leukaemia/lymphoma

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17
Q

What are some aggressive NHL?

A

Diffuse large B cell (DLBCL)

Mantle cell

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18
Q

What are some indolent NHL?

A

Follicular
CLL
MALT

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19
Q

How is DLBCL treated?

A

6-8 cycle of R-CHOP

If they relapse consider autologous stem cell transplant

20
Q

What is follicular NHL associated with?

A

t 14:18 translocation resulting in over espression of bcl-2

21
Q

How is follicular NHL treated?

A

Watch and wait
Only treat if clinically indicated like:
- compression due to nodes e.g. bowel, ureter, vena cava
- massive painful nodes, recurrent infections

22
Q

What is Extranodal Marginal Zone Lymphoma?

A

A marginal zone NHL involving extranodal lymphoid tissue e.g. MALT

23
Q

What are some conditions associated with Extranodal Marginal Zone Lymphomas?

A

Sjogren’s syndrome- parotid lymphoma
Hashimoto’s thyroiditis- thyroid MZL
Psittaci infection- lacrimal gland
H. pylori- gastric MALToma

24
Q

Where do Hy Pylori gastric MALTs usually arise and how don they present?

A

In the stomach

Usually present with dyspepsia, epigastric pain, ulceration, or bleeding

25
What is Enteropathy Associated T cell Lymphoma (EATL) and how does it present?
T cell NHL associated with Coeliac disease
26
What cells does EATL involve and where does it occur?
Mature T cells | Small intestine
27
How does EATL present?
Abdominal pain, obstruction, perforation, GI bleeding Malabsorption Systemic symptoms Responds poorly to chemotherapy
28
What cells does CLL involve and what kind of mutation is it associated with?
Mature B cells Acquired sporadic mutation
29
What are the lab findings in CLL?
LYMPHOCYTOSIS between 5-300 x 109/L Smear cells present- Artefactual problems as the cells break down (damaged by smearing) Normocytic normochromic anaemia Thrombocytopaenia Bone marrow lymphocytic replacement of normal marrow elements- due to lots of infiltration
30
What cell markers do normal mature B cells express?
CD19
31
What cell markers do normal mature T cells express?
CD 3 CD 5 Cd 4 or 8 depending on whether its a cytotoxic cell or helper cell
32
What cell markers do CLL B cells express?
CD 5 - Should never be expressed by B cells | CD 19
33
What indicated poor prognosis in CLL?
CD 38 expression IgH unmutated Deleted 17p (TP53)
34
What causes increased risk of infection in CLL?
Malignant and non functional mature B cells cannot secrete polyclonal immunoglobulins, leading to hypogammaglobulinaemia
35
What causes bone marrow failure in CLL?
Proliferation of B cells in bone marrow causing effacement of bone marrow
36
What causes lymphadenopathy and splenomegaly in CLL?
B cells circulating to nodes, spleen and blood
37
What is Richter transformation and what causes it in CLL?
Transformation into a higher grade lymphoma Further acquired mutations cause this
38
What other diseases can CLL cause?
Autoimmune diseases such as haemolytic anaemia
39
Which vaccines are not given to CLL patients?
Live vaccines such as VZV vaccine
40
What supportive treatments can be given to CLL patients?
Vaccines Acyclovir Pneumocystis jiroveci pneumonia prophylaxis with pentamidine for those who are immunosuppressed IVIg recommended for those with hypogammaglobulinaemia and recurrent bacterial infections
41
What are the treatment options for CLL
Chemo if possible otherwise watch and wait
42
What is the aim of treatment?
Establish remission
43
What are the indications for treatment?
Progressive lymphocytosis (count doubling < 6 months) Progressive bone marrow failure Hb < 100 Platelets < 100 Neutrophils < 1 Massive or progressive lymphadenopathy/ splenomegaly Systemic symptoms (B symptoms) Autoimmune cytopaenias (treat with steroids)
44
How does venetoclax cure CLL?
BCL 2 protein inhibitor
45
How does Rituximab cure CLL?
Targets CD20 Ag on B cells
46
How does Ibrutinib cure CLL?
BCR kinase inhibitor