Haematological Malignancies

Question 1

Which haemotological malignancies involve lymphoid cells?

Answer 1

Lymphoblastic leukaemia.

Lymphomas

Question 2

Which haemotological malignancies involve myeloid cells?

Answer 2

Myeloid leukaemia

Myeloproliferative disorders

Question 3

Who is most likely to get ALL?

Answer 3

Children

Question 4

What is the peak age of onset of CML?

Answer 4

25-45 years

Question 5

Which gene is normally present in CML?

Answer 5

BCR-ABL fusion gene

Question 6

What does the BCR-ABL fusion gene do?

Answer 6

Causes abnormal expression of myeloid cells in the bone marrow and peripheral blood.

Question 7

Which chromosomes are translocated in the Philadelphia chromosome?

Answer 7

22 and 9

Question 8

What is the treatment for CML?

Answer 8

Imatinib or bone marrow transplant

Question 9

What is CLL?

Answer 9

B-cell lymphoproliferative disease

Question 10

What is the peak age of onset of CLL?

Answer 10

72 years

Question 11

What would be found on blood in CLL?

Answer 11

High lymphocyte count

Question 12

What is the management of CLL?

Answer 12

Chemotherapy
Tyrosine kinase inhibitors
Monoclonal antibodies
Bone marrow transplant in young patient

Question 13

What are smudge cells?

Answer 13

Remnants of cells that lack any identifiable cytoplasmic membrane or nuclear structure. Also called basket cells.

Question 14

What are smudge cells associated with?

Answer 14

Abnormally fragile lymphocytes in chronic lymphocytic leukaemia.

Question 15

What is myeloma?

Answer 15

A malignant disorder of plasma cells producing the same abnormal immunoglobulin.

Question 16

Who is myeloma most prevalent in?

Answer 16

Median age 72 years

More prevalent in Afro-Carribean descent.

Question 17

What are the signs and symptoms of myeloma?

Answer 17

Bone pain
Anaemia
Infection
Bone marrow failure
Renal failure
Amyloidosis
Hypercalcaemia

Question 18

Which investigations would be done to test for myeloma?

Answer 18

Blood film (rouleaux formation)
Bone marrow aspirate (<10% plasma cells)
Serum protein electropheresis
Bence Jones protein in urine
Bone profile - hypercalcaemia
Imaging - skeletal survey

Question 19

What is the treatment for myeloma?

Answer 19

Monitoring
Chemo - thalidamide and steroids (or doxorubicin and vincristine)
Bone marrow transplant after induction therapy
Radiotherapy for bone pain
Treatment for hypercalcaemia (fluids and bisphosphonates)
Aspirin for DVT prophylaxis.

Question 20

What is lymphoma?

Answer 20

Cloncal prolfieration of lymphoid cells arising in the lymph nodes.

Question 21

What are the symptoms of lymphoma?

Answer 21

Local or generalised lymphadenopathy
Night sweats
Weight loss
Fevers

Question 22

Which cells are present in Hodgkin lymphoma?

Answer 22

Reed-Sternberg cells

Question 23

What is the epedemiology of lymphoma?

Answer 23

M>F

Age 15-40 years

Question 24

What is the treatment of lymphoma?

Answer 24

Local disease - radiotherapy
Wide spread disease - chemotherapy
+/- bone marrow transplant

Question 25

What is the prognosis for lymphoma?

Answer 25

> 90% stage I-II cured

50-70% stage IV cured.

Question 26

What are myelodysplasias?

Answer 26

Clonal haemopoetic stem cell disorder causing various cytopenias.
Bone marrow is hypercellular due to ineffective haemopoiesis.

Question 27

What can myelodysplasia transform into?

Answer 27

AML

Question 28

What is the treatment for myelodysplasias?

Answer 28

Blood transfusion +/- iron chelation

Chemo/bone marrow transplant in young patients

Question 29

What is myelofibrosis?

Answer 29

Proliferation of an abnormal cell line results in fibrosis in the bone marrow.

Question 30

What are the symptoms of myelofibrosis?

Answer 30

Bone pain
Marrow failure symptoms
Hepatosplenomegaly
Pancytopenia