Haematological Malignancy

Question 1

Describe the epidemiology of haematological malignancies

Answer 1

• They occur in all age groups
• Adult males are more commonly affected than females
• Lymphoma is relatively common amongst the blood cancers
• Acute lymphoblastic leukaemia is the most common cancer of childhood

Question 2

Describe the pathogenesis of haematological malignancy

Answer 2

• Acquired genetic alterations in a long lived cell
• Proliferative/survival advantage to that mutated cell
• This produces the malignant clone
• The malignant clone grows to dominate the tissue

Question 3

Describe how AML develops

Answer 3

• The genetic mutation affects the stem cell and how the myeloid progenitor differentiates into neutrophils etc.
• In AML the bone marrow is replaced by non differentiated blast cells
• Leads to bone marrow failure

Question 4

Describe how myeloproliferative disorders develops

Answer 4

• In this case the differentiation is maintained so you have an excess of the myeloid cells
• Chronic myeloid leukaemia etc.

Question 5

Describe how ALL develops

Answer 5

• The mutation occurs in the young lymphocytes

- Way too many immature lymphocytes are produced

Question 6

Name the types of cancers which can develop in maturer lymphocytes

Answer 6

• CLL (bone marrow makes too many lymphocytes)
• Lymphomas (in the germ centre of lymph nodes)
• MM (in plasma cells)

Question 7

What is the main difference between leukaemia and lymphoma?

Answer 7

• Leukaemia starts in the bone marrow

- Lymphoma: in the lymphoid and extramedullary tissue

Question 8

Describe the features of acute leukaemia

Answer 8

• Leukaemic cells do not differentiate
• Bone marrow failure
• Rapidly fatal if untreated
• Potentially curable e.g. childhood ALL
• AML has good prognosis if treated with chemotherapy

Question 9

Describe the features of chronic leukaemia

Answer 9

• Leukaemic cells retain the ability to differentiate
• Proliferation without bone marrow failure
• Survival for a few years
• Potentially curable with modern therapy e.g. tyrosine kinase inhibitors in CML

Question 10

Describe the structure of a lymph node

Answer 10

• B cells in the follicles: those undergoing expansion and selection in the germ centres and naive cells in the mantle zone
• T cells in the paracortex
• Plasma cells in the medulla

Question 11

List the causes of lymphadenopathy

Answer 11

• Localised and painful: bacterial infection in draining site
• Localised and painless: rare infections, metastatic cancer from draining site, lymphoma and reactive
• Generalised and painful/tender: viral infections (EBV, CMV, hepatitis and HIV)
• Generalised and painless: lymphoma, leukaemia, connective tissue diseases, reactive and drugs

Question 12

How do lymphomas present?

Answer 12

• Nodal disease: lymphadenopathy
• Extranodal: NHL
• Systemic symptoms: fever, drenching sweats, loss of weight, pruritis and fatigue

Question 13

List the major groups of haematological malignancies

Answer 13

• Acute leukaemias: acute lymphoblastic leukaemia and acute myeloid leukaemia
• Chronic leukaemias: chronic myeloid leukaemias and chronic lymphocytic leukaemias
• Malignant lymphomas: non-Hodgkins and Hodgkins
• Multiple myeloma
• Myelodysplastic syndromes
• Chronic myeloproliferative diseases