Haematological malignancy

Question 1

Can children be affected by haematological malignancy?

Answer 1

Yes

Question 2

What is the commonest cancer in children?

Answer 2

Acute lymphoblastic leukemia

Question 3

What age is the peak incidence for Hodgkin lymphoma?

Answer 3

20-30

Question 4

What time of year do we see Hodgkin lymphoma most?

Answer 4

Spring

Question 5

What is the pathogenesis of haematological malignancy?

Answer 5

Multi step process
Acquired genetic alterations in a long lived cell
Proliferative/survival advantage to that mutated cell
This produces a malignant clone
Malignant clone grows to dominate the tissue

Question 6

What cell does malignancy originate in?

Answer 6

Haematopoietic stem cell

Memory cells

Question 7

What is important about haematopoietic stem cells?

Answer 7

They are pluripotent

They are self renewing

Question 8

How are the stem cells self renewed?

Answer 8

One daughter cell is given up to become a specific cell, one remains as a stem cell

Question 9

What malignancies are considered myeloid malignancies?

Answer 9

Red cells
Platelets
Granulocytes
Monocytes

Question 10

What happens if there is excessive proliferation without differentiated cells leaving the marrow?

Answer 10

The bone marrow fails - acute myeloid leukemia

Question 11

What happens in the blood as a result of acute myeloid leukemia?

Answer 11

Cytopenia

Question 12

What is a myeloproliferative disorder?

Answer 12

Excessive proliferation leading to differentiated myeloid cells

Question 13

What is acute lymphoblastic leukemia?

Answer 13

Excessive proliferation of immature lymphoid cells without differentiation

Question 14

Where do mutation events occur in Mature lymphoid malignancy?

Answer 14

In the mature lymphoid cell

Question 15

What are differences between leukemia and lymphoma?

Answer 15

Leukemia is in blood and bone marrow

Lymphoma is in lymphoid tissue

Question 16

How does chronic lymphocytic luekemia usually present?

Answer 16

Raised white cell count over a period of time without other symptoms

Question 17

What are differences between acute and chronic leukemia?

Answer 17

Acute cells do not differentiate, chronic cells do
Acute leads to marrow failure, chronic has proliferation without marrow failure
Acute is rapidly fatal if untreated, chronic is survivable for a few years
Both are potentially curable, but chronic required more modern therapy

Question 18

What is used to treat chronic myeloid leukemia?

Answer 18

Tyrosine kinase inhibitors

Question 19

What are different areas of lymph nodes?

Answer 19

Medulla
Cortex
Paracortex
B-cell follicle - germinal centre, mantle zone, marginal zone

Question 20

Where do B cells mature?

Answer 20

Germinal centre in lymph node

Question 21

What is somatic hypermutation?

Answer 21

B cell divides rapidly to mature and be able to destroy antigens correctly

Question 22

What is the origin of most lymphomas?

Answer 22

B-cell mutations

Question 23

How do lymphomas present?

Answer 23

Lymphadenopathy - not always malignant
Systemic symptoms - fever, drenching sweat. loss of weight, pruritis, fatigue
Extranodal disease

Question 24

What should be considered with lymphadenopathy presentation?

Answer 24

Localised and painful - likely bacterial infection in draining site
Localised and painless - rare infections ie TB, Metastatic carcinoma from draining site (hard), Lymphoma (rubbery), reactive (no cause identified)
Generalised and painful - viral infections ie glandular fever, HIV, Hep
Generalised and painless - Lymphoma, Leukemia, connective tissue diseases, reactive with no cause identified, drugs