Haematological Malignancy

Question 1

Define acute myeloid leukaemia

Answer 1

Clonal proliferation of primitive myeloblasts
- i.e. all derived from one abnormal progenitor cell

Cells fill up bone marrow and spill into blood resulting in marrow failure

Question 2

What is the presentation of AML?

Answer 2

Anaemia
Neutropaenia
Thrombocytopaenia

Question 3

What is the prupose of treatment in AML and ALL?

Answer 3

Chemo used to destroy blastic cells and allow normal bone marrow to grow back

Question 4

Define acute lymphoblastic leukaemia

Answer 4

Clonal proliferation of primitive lymphoblasts

Fill up bone marrow and spill into blood resulting in marrow failure

Question 5

What is the most common malignant disease of childhood?

Answer 5

Acute lymphoblastic leukaemia

Question 6

Define chronic myeloid leukaemia

Answer 6

Genetic mutation (philadelphia translocation) codes for abnormal tyrosine kinase
 - produces clonal proliferation and reduced apoptosis of granulocytes which enter bloodstream

Question 7

Which cancer is associated with a genetic mutation (Philadelphia translocation) coding for abnormal tyrosine kinase?

Answer 7

Chronic Myeloid Leukaemia

Question 8

What are the features of CML?

Answer 8

Hypercatablic Sx
Leukocytosis
Philadelphia chromosome (from chromosome 9 to 22)

Question 9

Define Chronic Lymphoblastic Leukaemia

Answer 9

Clonal proliferation of (usally) B cells which accumulate in blood
- abnormal B cell maturation may lead to reduced Ig and reduced immunity

Question 10

Which group of people does CLL usually affect?

Answer 10

The elderly - doesn’t usally require treatment

Question 11

Describe the presentation of CLL

Answer 11

May present incidentally on FBC
Lymphadenopathy +/- organomegaly
Immune suppression

Question 12

What is lymphoma?

Answer 12

Proliferation of malignant lymphocytes originating in lymph organs which result in solid depositions of lymphocytes in lymph glands, spleen and organs
- Most commonly affect B cells

Question 13

What are the features of lymphoma?

Answer 13

Fever, nightsweats, and weight loss
- due to cytokine release

Lymphadenopathy/Splenomegaly
- due to lymphocyte masses

Spread to other organs esp. blood and bone marrow

Question 14

What is Hodgkins’ Lymphoma?

Answer 14

Reed Sternberg Cells (giant transformed B cells with double nuclei) = diagnostic

Question 15

What are the features specific to Hodgkins lymphoma?

Answer 15

Pruritis, pain in affected lymph nodes on drinking alcohol

Question 16

What is Non-Hodgkins’ lymphoma?

Answer 16

More disseminated at presentation usally

Question 17

What are myelodysplasias?

Answer 17

Malignant dyspalstic changes in blood and marrow myeloid lines which may become AML, e.g.

• hypogranular neutrophils
• ring sideroblasts

Question 18

What are myeloproliferative disorders?

Answer 18

Overproduction of relatively normal blood and bone marrow cells

• usually due to mutations, e.g. of EPO receptor
• may present as polycythaemia, thrombocythaemia, myelofibrosis

Question 19

What is myelofibrosis?

Answer 19

Increased collagen in marrow cavity and sclerosing of it