Haematological Malignancies Flashcards

1
Q

What is essential thrombocythaemia?

A

A myloproliferative disorder- too many platelets

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2
Q

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

A

Polycythaemia rubra Vera (PRV)

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3
Q

What causes myelofibrosis?

A

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

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4
Q

Name two myloproliferative disorders involving mast cells and eosinophils

A

Idiopathic hypereosinophilic syndrome

Systemic mastocytosis

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5
Q

What lymphocytes are mostly effected by hodgekins lymphomas

A

B lymphocytes

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6
Q

What is underlying problem in ET and PRV?

A

JAK2 mutation

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7
Q

What 3 clinical features occur with bone marrow failure?

A

Anaemia- tiredness
Thrombocytopenia- bleeding and bruising
Neutropenia - infections

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8
Q

What is myelodysplastic syndrome?

A

Premature ageing of the bone marrow

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9
Q

How do you treat AML?

A

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

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10
Q

How do you treat ALL?

A

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

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11
Q

Why are lymphocytes hardest to kill off?

A

Because they want to last longer- memory B cells

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12
Q

What is the leak incidence of ALL?

A

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

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13
Q

What is the cure rate for ALL?

A

85%

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14
Q

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

A

Testes and CNS

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15
Q

What are the most common leucocytes found in CML?

A

Neutrophils and metamyelocytes

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16
Q

What is priapism?

A

Syrupy blood from too many WBCs disrupts blood flow in penis

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17
Q

What cell surface markers would you find in CLL?

A

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

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18
Q

What is cd5 a marker for?

A

Activated B cells

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19
Q

What is cd19 a marker for

A

B cells

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20
Q

What is cd79b a marker for?

A

Immature B cells

21
Q

What is stage A of the Binet system for CLL?

A

Lymphocytosis only - 10 years

22
Q

What is stage B of the Binet system?

A

Lymphocytosis and 3+ areas of lymphadenopathy - 5 years

23
Q

Stage C of the Binet system

A

Lymphocytosis and LYmphodenopathy and hb

24
Q

What are non hodgekins lymphomas?

A

Tumour growth of lymphoid cells

25
Q

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

A

30%

26
Q

What are b symptoms of lymphoma?

A

Weight loss >10% in 6 mts, night sweats? Fever above 37

All caused by cytokines

27
Q

What causes hodgekins lymphoma?

A

EPV virus

28
Q

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

A

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

29
Q

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

A

False

30
Q

Low grade lymphoma

A

Slow and grumbling

31
Q

What type of disorder is myeloma?

A

An immunosecretory disorder

32
Q

What is MGUS?

A

Monoclonal gammopathy of uncertain significance - abnormal antibodies but nothing else

33
Q

What is IgD normally expressed on?

A

B lymphocytes

34
Q

What does IgM normally do?

A

Clears pathogens from circulation

35
Q

What does IgG normally do?

A

Clears pathogens from tissue fluid

36
Q

What does IgA normally do?

A

Prevents pathogens invading mucosal surfaces

37
Q

What does IgE normally do?

A

Protects against parasites

38
Q

True or false: IgG can switch to IgA and IgE

A

True

39
Q

What is a paraproteins and how would I detect it?

A

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

40
Q

What is bence-Jones protein?

A

Isolated light chain

41
Q

What criteria must be met for myeloma?

A

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

42
Q

What is a skeletal survey?

A

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

43
Q

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

A

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

44
Q

Is monoclonal IgM a sign of lymphoma?

A

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

45
Q

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

A

Waldenstroms

46
Q

What is CHAD? What paraproteins is it associated with?

A

Cold haem agglutinin disease p. IgM

47
Q

What is hyperviscosity syndrome?

A

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

48
Q

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

A

No- dangerous. Anaemia reduces the viscosity

49
Q

What cell lines can be affected by myelodysplasia?

A

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