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Haematology > Lynpmohoid Disease > Flashcards

Lynpmohoid Disease Flashcards

1
Q

What is acute lymphoblastic leukaemia?

A

Accumulation of lymphoblasts in Bone marrow

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2
Q

What is the most malignancy in children?

A

ALL

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3
Q

What is the occurrence of T-ALL and B-ALL?

A
  1. 3 per 100,000 T-ALL

0. 9 per 100,000 B-ALL

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4
Q

What is the median age for t-all and b-all?

A

T-all median age is 20

B-all median age is 11

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5
Q

What % of all is of B cell lineage?

A

85%

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6
Q

How do you distinguish between b-all and t-all?

A

Based on clonallity testing? Immunoglobulin rearrangement = B cell
TCRs rearrangement = t-all

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7
Q

What are two common changes associated childhood all?

A

Hyperdiploidy (50+) chromosomes good prog

TEL-AML1

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8
Q

What is the affect of TEL-AML1?

A

AML1 plays important part in regulation of haematopoiesis but TEL represses its function

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9
Q

What is the genotype found in the majority of infants?

A

11q23/MLL

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10
Q

What is the prognosis of BCR-abl in all in adults?

A

Poor

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11
Q

What are the clinical features of ALL?

A

BM failure
Anaemia (normochromic, normocytic)
Neutropenia
Thrombocytopenia

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12
Q

What percentage blast is seen in BM and PB in ALL?

A

> 20% in BM but variable in PB

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13
Q

What drugs are used to treat childhood ALL?

A

Daunarubican, etopside, ara-c, vincristine

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14
Q

What is prognosis of ALL in infants less than 1 yr old?

A

20-50% cure rate

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15
Q

What is prognosis for ALL in over 1 yr olds?

A

V. Good over 95%

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16
Q

Which has a worse prognosis b-all or T-all?

A

t-all

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17
Q

How do you treat adult ALL?

A

Same drugs as children but use lower doses

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18
Q

What percentage of adult all is BCR-abl positive?

A

25%

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19
Q

What is chronic lymphoid leukaemia?

A

Accumulation of mature lymphocytes (usually B-lymphocytes) decrease in apoptosis

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20
Q

What is the incidence of CLL?

A

6.5 per 100,000

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21
Q

What is the peak age for CLL and is it more common in males or females?

A

60-80 yrs. more common in men

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22
Q

What is ZAP70 and is it a good or bad prognostic marker?

A

It is a tyrosine kinase and is a good prognostic marker in CLL
Good if you have low levels
Bad if you have high levels

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23
Q

What percentage of CLL I’d t-cll? And what is it associated with?

A

2%. Associated with HTLV-1 (human t-cell leukaemia lymphoma virus) prevalent in Asia and Caribbean

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24
Q

What is the effect of a 17p abnormality?

A

Poor prognosis in CLL

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25
Q

What is the effect of 11q23?

A

Bad prognosis in CLL

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26
Q

What is the prognosis of trisomy 12?

A

Intermediate prognosis in CLL

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27
Q

What is a good prognosis in CLL?

A

13q14 deletions

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28
Q

What are the clinical features of CLL?

A

Cells accumulate in BM, liver, spleen and lymph nodes

Symmetrical enlargement of lymph nodes

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29
Q

What is associated with late stage CLL?

A

Anaemia (normocytic, normochromic)

Thrombocytopenia

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30
Q

What would you see on a blood film of CLL?

A

Lymphocytes seen as bigger than rbcs with little cytoplasm, irregular nuclei and clumped chromatin
Smudged cells

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31
Q

What is rituximab?

A

Anti-cd20 antibody used to treat CLL

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32
Q

What is lymphoma?

A

Accumulation of malignant lymphocytes in lymph nodes

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33
Q

What is characteristic of lymphoma?

A

Lymphadenopathy

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34
Q

What are Reed-Sternberg cells?

A

Abnormal giant cells from b- cell lineage, neoplastic cells (mass tumour)

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35
Q

What is also associated with RS cells?

A

Abnormal mononuclear cells and infiltrating reactive inflammatory cells

36
Q

What virus is hodgekins lymphoma associated with?

A

Epstein-Barr virus

37
Q

Is HL common in children?

A

No, peak in young adults mean age 41 years

38
Q

What is the incidence of HL?

A

3.1 per 100,000

39
Q

95% of HL is divided into 4 types:

A

1) nodular sclerosis
2) mixed cellularity
3) lymphocyte depleted
4) lymphocyte rich

40
Q

What is the other 5%of HL?

A

Nodular lymphocyt predominate HL ( non RS cells)

41
Q

What people are most susceptible to nodular sclerosis?

A

Women in their 20s and 30s - diagnosed usually from swollen neck nodes

42
Q

What group of patients are most affected by mixed cellularity HL?

A

Older patients- is usually a more widespread disease

43
Q

What is stage 1 HL?

A

Involvement of 1 lymphnode

44
Q

What is stage 2 HL?

A

Involvement of 2 or more lymph nodes

45
Q

Stage 3 HL?

A

Lymphnodes above and below diaphragm

46
Q

Stage 4 HL?

A

Involvement outside lymph nodes

47
Q

What does a and b added onto the stages of HL mean?

A 
B= presence of night sweats, fever or weight loss
A= not there
48
Q

What chromosomal abnormality is associated with HL?

A

To date - no non random. Karyotypes usually complex

49
Q

What would you expect to see in a HL tumour section?

A

RS cells, inflammatory cells and possibly fibrosis

50
Q

Is there BM involvement in HL?

A

Usually only occurs in late stage

51
Q

What type of anaemia is associated with HL

A

Normochromic normocytic

52
Q

Adriamycin, bleamycin, vinblastine, decarbazine are used to treat what?

A

HL

53
Q

What percentage of HL is cured?

A

85%

54
Q

What is a bad sign in HL?

A

If you are male and you have low haemoglobin and if you have stage 4

55
Q

What is the most favourable group of patients with HL?

A

Lymphocyte rich

56
Q

Why is there blurred lines between CLL and nm hodgekins lymphoma?

A

Lymphoma cells can circulate and be found in PB

57
Q

What is the incidence of NHL?

A

16.9 per 100,000

58
Q

What virus is associated with adult t- cell lymphoma?

A

HTLV-1

59
Q

What virus associated with Burkitt lymphoma?

A

EBV and Malaria

60
Q

What virus is associated with gastric lymphoma (MALT)

A

Heliocobacter pylori

61
Q

What percentage of NHL are T or NK cells

A

15%, 85% b-cells

62
Q

What 3 groups can B cell lymphomas (NHL) be divided into?

A

BM precursor B cells
Germinal centre B cells
Post GC B- cells in lymph nodes

63
Q

What 3 groups can t-cell lymphomas be split into?

A

BM t-cells
Thymus T cells
Peripheral mature T cells

64
Q

How does WHO 2008 classify NHL

A

According to the cell from which they are derived

65
Q

What are the most common NHL lymphomas?

A

Diffuse large B-cells

Follicular Lymphoma

66
Q

What are he common karyotypes for diffuse large B cell lymphoma?

A

30% have a 3q27 rearrangement that affects BCL - 6 gene
T(14;18)
T(8;14) effects c-Myc gene

67
Q

90% of follicular B cell lymphoma have what karyotype?

A

T(14;18) BCL-2 regulation disrupted

68
Q

100% of mantle cell lymphomas have what mutation.

A

T(11;14) aberrant BCL-1 expression

69
Q

Marginal zone B cell lymphoma is associated with what karyotypes?

A

+3 and + 18

70
Q

Burkitts lymphoma is associated with what karyotypes?

A

T(8;14) c-myc
T(2;8)
T(11;14)

71
Q

As well as lymphadenopathy what is NHL associated with?

A

Anaemia, neutropenia, thrombocytopenia

72
Q

Are marginal and follicular NHL aggressive or indolent?

A

Indolent

73
Q

How are NHL treated?

A

With rituximab

74
Q

What is R-CHOP?

A

Chemotherapy plan that uses other drugs a long side rituximab to treat NHL

75
Q

Is diffuse large B cell indolent or aggressive?

A

Aggressive

76
Q

What is myeloma?

A

Neoplastic accumulation of bone marrow plasma cells

77
Q

In myeloma what antibodies are found in serum and/or urine

A

Usually IgG or IgA

78
Q

What is the incidence of myeloma?

A

7.1 per 100,000

79
Q

What is the median age of myeloma?

A

72

80
Q

What % of plasma cells in BM is diagnostic of MGUS?

A
81
Q

What are common karyotypes of myeloma?

A

Majority have hyperploidy
-13
-13q
11q abnormalities

82
Q

Why is hypercalcaemia associated with myeloma?

A

Osteoclasts are breaking down bone leading to the release of calcium

83
Q

What is CRAB?

A

Hypercalcaemia, renal impairment, anaemia and bone disease

Clinical features for myeloma

84
Q

What would you see in a PB and BM for myeloma?

A 
Rouleaux formation (ESR increase) PB
More than 20% abnormal plasma cells in BM
85
Q

What is the outcome of myeloma?

A

Incurable except with allogeic stem cell transplantation
3-4 years with non intensive chemotherapy
5-6 years with autologous transplant

Haematology (9 decks)

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