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Path - Haematology > Haemato-oncology 1 > Flashcards

Haemato-oncology 1 Flashcards

1
Q

Chronic lymphocytic leukaemia, CLL?

A

Cancer of the B-cells

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2
Q

Chronic myeloid leukaemia, CML

A

Cancer of the granulocytes at all levels

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3
Q

Polycythaemia Rubra Vera

A

Cancer of the red blood cells

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4
Q

Essential Thrombocythaemia

A

Cancer of the megakeryocytes/platelets

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5
Q

Myelofibrosis

A

Cancer of blood progenitor cells leading to bone marrow scarring

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6
Q

T-cell lymphoma

A

cancer of T cells

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7
Q

Chronic Lymphocytic Leukaemia

which population is it common?

most cases of CLL diagnosed?

• What is CLL?

What is monoclonal B cell lymphocytosis?

A

elderly (median age at diagnosis is 72)

70% are as an incidental finding on a routine blood check

defined as having CLL when you have a clonal B cell lymphocytosis of >5x109 cells per litre for more than 3 months.

where you have a clonal population of B cells that is not >5x109 cells per litre. So you still have a clonal B cell proliferation but it hasn’t passed the threshold to call it CLL yet, although they do have a chance of progressing to it.

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8
Q

• How do you know you have CLL from a blood film?

Once we’ve seen the blood film and suspect CLL, how do we confirm the diagnosis?

A
  1. Lots of mature lymphoid cells
  2. Smear cells – this is because the cancerous B cells are fragile and during the blood film they get smudged

immunophenotyping (looks at cell surface markers). Weirdly, the B cells in CLL express a T-cell marker called CD5 as well as B cell markers, which allows us to say for sure that the cause of the B-cell lymphocytosis is CLL and not some other rare form of B-cell lymphoma

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9
Q

• What things would suggest that your particular CLL is fast-growing, hence worsening your prognosis?

Cytogenetics:

13q deletion
11q deletion
17q deletion of p53 gene

A
  1. Lymphadenopathy of >3 areas
  2. Progressive splenomegaly.
  3. A lymphocyte doubling time of <6 months

i. 13q deletion = good prognosis
ii. 11q deletion = medium prognosis
iii. 17p deletion of the p53 gene = worst prognosis as its resistant to chemotherapy and rapidly progressive (2-3 years survival). Found in 10% of CLL patients.

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10
Q

• When do we treat CLL?

A

When there is a bad prognosis

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11
Q

How do we categorise patients with CLL to inform how to treat them?

o Go-Go’s
o Slow-Go’s
o No-Go’s

• When would you look at the possibility of a bone marrow transplant?

What is ‘chemo-free’ treatment?

A

o Go-Go’s – if the patient is fit they get very intensive chemotherapy (including anti-CD20 mAB called Rituximab), which kills such a big proportion of the clonal B cells that it takes a long time (>10 years) for the B-cell count to creep back up. Hence, a go-go will have one session of very intensive therapy and then be ok for a number of years.

o Slow-Go’s – the patient is slightly less well and would probably die from very intensive chemotherapy. Hence you give little bouts of chemotherapy every year to try and keep the clonal B-cell population under control

o No-Go’s – the patient is so unwell that any amount of chemotherapy would make them ill

In fit, healthy patients with a bad prognosis who has relapsed after initial go-go treatment.

Idelenisib & Ibrutinib) that targets the tyrosine kinase cascade downstream from the B-cell receptor as a means of preventing replication. These drugs also work on p53 mutated individuals!

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12
Q

• What are the associated problems with CLL:

  • what can it transform into?
A
  1. Richter’s transformation – CLL can transform into a high grade non-Hodgkin’s lymphoma = extremely bad news
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13
Q

Chronic Myeloid Leukaemia

  • What is CML?
  • When does CML present

• What does a blood film look like in CML?

A

cancer of the granulocytes leading to a massive accumulation of white blood cells.

40-60 years old

a huge number of mature granulocytes (neutrophils, basophils, eosinophils) as well as immature granulocytes

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14
Q

• What goes wrong in CML?

Philadelphia translocation

A

9-22 translocation

BCR-ABL tyrosine kinase protein
This hybrid is really high in activity → cell growth gets out of control → cancer

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15
Q

A successful tyrosine kinase inhibitor drug for CML?

  • How does it work?
  • How effective is this treatment?
A

Imatinib

  1. It binds to the BCR-ABL tyrosine kinase protein
  2. This prevents ATP from binding to the protein (competitive inhibitor)
  3. The substrate cannot be phosphorylated and the signal is blocked

Outstandingly effective - now they live to same age as normal people

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16
Q

• What is a myeloproliferative neoplasm?

A

An umbrella term for a blood cancer arising from the differentiated myeloid blood cells

o Cancers from the granulocytes = CML
o YES. CML is a type of myeloproliferative neoplasm even though this lecture splits them up because there’s a lot to say about CML
o Cancers from the erythrocytes = polycythaemia rubra vera
o Cancers from the megakaryocytes = essential thrombocythaemia.
o Cancers from reactive fibrosis = primary myelofibrosis

17
Q

• What are the common mutations in the myeloproliferative neoplasms?

Chronic myeloid leukaemia?
Polycythaemia Rubra Vera?
Essential thrombocythaemia?
Myelofibrosis?

• What is the main risk with both PRV and ET?

A

Chronic myeloid leukaemia = philadelphia translocation
Polycythaemia Rubra Vera = JAK2 90%
Essential thrombocythaemia = JAK2 60%
Myelofibrosis = JAK2 60%

  1. Transformation to acute myeloid leukaemia
18
Q

• What does JAK2 do?

A

It’s a tyrosine kinase receptor.

19
Q

• What goes wrong in the JAK2 mutation

A

constitutive activation of the TKR → proliferation

20
Q

• What specific sign do you get in polycythaemia Rubra Vera?

A

Aquagenic pruritus (itching after water)

21
Q
  • What is the main treatment for any myeloproliferative condition
  • So you have given aspirin and are checking up on them and their platelet count won’t get low enough and you are worried. What do you do?
A
  1. Track them over time to make sure it stays stable
  2. Manage lifestyle factors to reduce risk of clotting
  3. Daily aspirin to reduce risk of clotting
  4. Hydroxycarbamide = a non-specific DNA inhibitor to suppress the blood count

In polycythaemia Rubra Vera, you can also do venesection (literally removing blood to reduce blood count)

22
Q

Myelofibrosis

What is myelofibrosis?

  • What do you see on a blood film with someone with myelofibrosis?
  • What is the last line therapy for myelofibrosis?
  • What else can we do?
A

fibrosis in the bone marrow due to a blood cancer.

Tear drop cells + lots of nucleated/progenitor RBC

If your young or survival is less than 5 yrs due to bad prognosis – then transplant.

JAK2 inhibitors – Ruxolitinib (even if you don’t have a JAK2 mutation because JAK2 in involved with all cells).

Path - Haematology (9 decks)

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