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Flashcards in Haematological Malignancies Deck (49):
1

What is essential thrombocythaemia?

A myloproliferative disorder- too many platelets

2

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

Polycythaemia rubra Vera (PRV)

3

What causes myelofibrosis?

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

4

Name two myloproliferative disorders involving mast cells and eosinophils

Idiopathic hypereosinophilic syndrome
Systemic mastocytosis

5

What lymphocytes are mostly effected by hodgekins lymphomas

B lymphocytes

6

What is underlying problem in ET and PRV?

JAK2 mutation

7

What 3 clinical features occur with bone marrow failure?

Anaemia- tiredness
Thrombocytopenia- bleeding and bruising
Neutropenia - infections

8

What is myelodysplastic syndrome?

Premature ageing of the bone marrow

9

How do you treat AML?

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

10

How do you treat ALL?

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

11

Why are lymphocytes hardest to kill off?

Because they want to last longer- memory B cells

12

What is the leak incidence of ALL?

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

13

What is the cure rate for ALL?

85%

14

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

Testes and CNS

15

What are the most common leucocytes found in CML?

Neutrophils and metamyelocytes

16

What is priapism?

Syrupy blood from too many WBCs disrupts blood flow in penis

17

What cell surface markers would you find in CLL?

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

18

What is cd5 a marker for?

Activated B cells

19

What is cd19 a marker for

B cells

20

What is cd79b a marker for?

Immature B cells

21

What is stage A of the Binet system for CLL?

Lymphocytosis only - 10 years

22

What is stage B of the Binet system?

Lymphocytosis and 3+ areas of lymphadenopathy - 5 years

23

Stage C of the Binet system

Lymphocytosis and LYmphodenopathy and hb

24

What are non hodgekins lymphomas?

Tumour growth of lymphoid cells

25

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

30%

26

What are b symptoms of lymphoma?

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

27

What causes hodgekins lymphoma?

EPV virus

28

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

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

29

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

False

30

Low grade lymphoma

Slow and grumbling

31

What type of disorder is myeloma?

An immunosecretory disorder

32

What is MGUS?

Monoclonal gammopathy of uncertain significance - abnormal antibodies but nothing else

33

What is IgD normally expressed on?

B lymphocytes

34

What does IgM normally do?

Clears pathogens from circulation

35

What does IgG normally do?

Clears pathogens from tissue fluid

36

What does IgA normally do?

Prevents pathogens invading mucosal surfaces

37

What does IgE normally do?

Protects against parasites

38

True or false: IgG can switch to IgA and IgE

True

39

What is a paraproteins and how would I detect it?

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

40

What is bence-Jones protein?

Isolated light chain

41

What criteria must be met for myeloma?

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

42

What is a skeletal survey?

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

43

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

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

44

Is monoclonal IgM a sign of lymphoma?

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

45

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

Waldenstroms

46

What is CHAD? What paraproteins is it associated with?

Cold haem agglutinin disease p. IgM

47

What is hyperviscosity syndrome?

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

48

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

No- dangerous. Anaemia reduces the viscosity

49

What cell lines can be affected by myelodysplasia?

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