Haematological Malignancies

Question 1

What is essential thrombocythaemia?

Answer 1

A myloproliferative disorder- too many platelets

Question 2

What is the name of a myloproliferative disorder that results in too many platelets?

Answer 2

Polycythaemia rubra Vera (PRV)

Question 3

What causes myelofibrosis?

Answer 3

It is a myloproliferative disorder from abnormal megakaryocytes that release growth factors in bone marrow causing fibroblasts I’m bone marrow to multiply

Question 4

Name two myloproliferative disorders involving mast cells and eosinophils

Answer 4

Idiopathic hypereosinophilic syndrome

Systemic mastocytosis

Question 5

What lymphocytes are mostly effected by hodgekins lymphomas

Answer 5

B lymphocytes

Question 6

What is underlying problem in ET and PRV?

Answer 6

JAK2 mutation

Question 7

What 3 clinical features occur with bone marrow failure?

Answer 7

Anaemia- tiredness
Thrombocytopenia- bleeding and bruising
Neutropenia - infections

Question 8

What is myelodysplastic syndrome?

Answer 8

Premature ageing of the bone marrow

Question 9

How do you treat AML?

Answer 9

Strong IV chemotherapy in short, sharp, bursts. This is why young people do better

Question 10

How do you treat ALL?

Answer 10

Mix of strong chemotherapy and persisting milder tablets to prevent replapse.

Question 11

Why are lymphocytes hardest to kill off?

Answer 11

Because they want to last longer- memory B cells

Question 12

What is the leak incidence of ALL?

Answer 12

4-5 years. May present with cytopenia or chest masses

Question 13

What is the cure rate for ALL?

Answer 13

85%

Question 14

Where are the sanctuary sites for ALL that can cause relapse?

Answer 14

Testes and CNS

Question 15

What are the most common leucocytes found in CML?

Answer 15

Neutrophils and metamyelocytes

Question 16

What is priapism?

Answer 16

Syrupy blood from too many WBCs disrupts blood flow in penis

Question 17

What cell surface markers would you find in CLL?

Answer 17

Positive for CD5 and CD19
Negative for CD79b
Weak surface immunoglobulins
Negative for FMC7

Question 18

What is cd5 a marker for?

Answer 18

Activated B cells

Question 19

What is cd19 a marker for

Answer 19

B cells

Question 20

What is cd79b a marker for?

Answer 20

Immature B cells

Question 21

What is stage A of the Binet system for CLL?

Answer 21

Lymphocytosis only - 10 years

Question 22

What is stage B of the Binet system?

Answer 22

Lymphocytosis and 3+ areas of lymphadenopathy - 5 years

Question 23

Stage C of the Binet system

Answer 23

Lymphocytosis and LYmphodenopathy and hb

Question 24

What are non hodgekins lymphomas?

Answer 24

Tumour growth of lymphoid cells

Question 25

What percentage of NHL's occur outside of lymph nodes ?

Answer 25

30%

Question 26

What are b symptoms of lymphoma?

Answer 26

Weight loss >10% in 6 mts, night sweats? Fever above 37 | All caused by cytokines

Question 27

What causes hodgekins lymphoma?

Answer 27

EPV virus

Question 28

Why do they think there are two age peaks in hodgekins? Teens and elderly?

Answer 28

Due to where the virus has integrated into the lymphocyte DNA- closer or further from Proto oncogenes

Question 29

True or false: a bone marrow biopsy is diagnostic in lymphoma?

Answer 29

False

Question 30

Low grade lymphoma

Answer 30

Slow and grumbling

Question 31

What type of disorder is myeloma?

Answer 31

An immunosecretory disorder

Question 32

What is MGUS?

Answer 32

Monoclonal gammopathy of uncertain significance - abnormal antibodies but nothing else

Question 33

What is IgD normally expressed on?

Answer 33

B lymphocytes

Question 34

What does IgM normally do?

Answer 34

Clears pathogens from circulation

Question 35

What does IgG normally do?

Answer 35

Clears pathogens from tissue fluid

Question 36

What does IgA normally do?

Answer 36

Prevents pathogens invading mucosal surfaces

Question 37

What does IgE normally do?

Answer 37

Protects against parasites

Question 38

True or false: IgG can switch to IgA and IgE

Answer 38

True

Question 39

What is a paraproteins and how would I detect it?

Answer 39

Electrophoresis. Monoclonal antibody causing a spike in the normal globulin pattern

Question 40

What is bence-Jones protein?

Answer 40

Isolated light chain

Question 41

What criteria must be met for myeloma?

Answer 41

1) plasma cells in marrow>10% 2) detectable paraproteins in blood or urine 3) lytic lesions on Skeletal survey

Question 42

What is a skeletal survey?

Answer 42

Series of plain X Ray's - not like bone scan

Question 43

Think of myeloma when you see elderly with what 5 conditions?

Answer 43

1) anaemia 2) high ESR 3) back pain 4) unexplained renal failure 5) unusually fractures

Question 44

Is monoclonal IgM a sign of lymphoma?

Answer 44

No, IgM is expressed when lymphocytes are maturing not on plasma cells)

Question 45

IgM paraproteins and marrow with lymphoplasmacytoid lymphocytes is found in what disease?

Answer 45

Waldenstroms

Question 46

What is CHAD? What paraproteins is it associated with?

Answer 46

Cold haem agglutinin disease p. IgM

Question 47

What is hyperviscosity syndrome?

Answer 47

Treacle like blood due to excess plasma proteins - fibrinogen, IgM and IgA

Question 48

If a patient has anaemia due to hyperviscosity syndrome should u treat with blood transfusion?

Answer 48

No- dangerous. Anaemia reduces the viscosity

Question 49

What cell lines can be affected by myelodysplasia?

Answer 49

Any or all! Eg pencytopenia- deficieny of red white and platelets