Lynpmohoid Disease Flashcards by leona Connolly

What is acute lymphoblastic leukaemia?

Accumulation of lymphoblasts in Bone marrow

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What is the most malignancy in children?

ALL

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

3 per 100,000 T-ALL

0. 9 per 100,000 B-ALL

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

T-all median age is 20

B-all median age is 11

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What % of all is of B cell lineage?

85%

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

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

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What are two common changes associated childhood all?

Hyperdiploidy (50+) chromosomes good prog

TEL-AML1

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What is the affect of TEL-AML1?

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

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What is the genotype found in the majority of infants?

11q23/MLL

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

Poor

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What are the clinical features of ALL?

BM failure
Anaemia (normochromic, normocytic)
Neutropenia
Thrombocytopenia

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

> 20% in BM but variable in PB

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What drugs are used to treat childhood ALL?

Daunarubican, etopside, ara-c, vincristine

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

20-50% cure rate

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What is prognosis for ALL in over 1 yr olds?

V. Good over 95%

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

t-all

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How do you treat adult ALL?

Same drugs as children but use lower doses

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What percentage of adult all is BCR-abl positive?

25%

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What is chronic lymphoid leukaemia?

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

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What is the incidence of CLL?

6.5 per 100,000

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

60-80 yrs. more common in men

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

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

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

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What is the effect of a 17p abnormality?

Poor prognosis in CLL

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What is the effect of 11q23?

Bad prognosis in CLL

What is the prognosis of trisomy 12?

Intermediate prognosis in CLL

What is a good prognosis in CLL?

13q14 deletions

What are the clinical features of CLL?

Cells accumulate in BM, liver, spleen and lymph nodes | Symmetrical enlargement of lymph nodes

What is associated with late stage CLL?

Anaemia (normocytic, normochromic) | Thrombocytopenia

What would you see on a blood film of CLL?

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

What is rituximab?

Anti-cd20 antibody used to treat CLL

What is lymphoma?

Accumulation of malignant lymphocytes in lymph nodes

What is characteristic of lymphoma?

Lymphadenopathy

What are Reed-Sternberg cells?

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

What is also associated with RS cells?

Abnormal mononuclear cells and infiltrating reactive inflammatory cells

What virus is hodgekins lymphoma associated with?

Epstein-Barr virus

Is HL common in children?

No, peak in young adults mean age 41 years

What is the incidence of HL?

3.1 per 100,000

95% of HL is divided into 4 types:

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

What is the other 5%of HL?

Nodular lymphocyt predominate HL ( non RS cells)

What people are most susceptible to nodular sclerosis?

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

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

Older patients- is usually a more widespread disease

What is stage 1 HL?

Involvement of 1 lymphnode

What is stage 2 HL?

Involvement of 2 or more lymph nodes

Stage 3 HL?

Lymphnodes above and below diaphragm

Stage 4 HL?

Involvement outside lymph nodes

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

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

What chromosomal abnormality is associated with HL?

To date - no non random. Karyotypes usually complex

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

RS cells, inflammatory cells and possibly fibrosis

Is there BM involvement in HL?

Usually only occurs in late stage

What type of anaemia is associated with HL

Normochromic normocytic

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

What percentage of HL is cured?

85%

What is a bad sign in HL?

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

What is the most favourable group of patients with HL?

Lymphocyte rich

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

Lymphoma cells can circulate and be found in PB

What is the incidence of NHL?

16.9 per 100,000

What virus is associated with adult t- cell lymphoma?

HTLV-1

What virus associated with Burkitt lymphoma?

EBV and Malaria

What virus is associated with gastric lymphoma (MALT)

Heliocobacter pylori

What percentage of NHL are T or NK cells

15%, 85% b-cells

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

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

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

BM t-cells Thymus T cells Peripheral mature T cells

How does WHO 2008 classify NHL

According to the cell from which they are derived

What are the most common NHL lymphomas?

Diffuse large B-cells | Follicular Lymphoma

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

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

90% of follicular B cell lymphoma have what karyotype?

T(14;18) BCL-2 regulation disrupted

100% of mantle cell lymphomas have what mutation.

T(11;14) aberrant BCL-1 expression

Marginal zone B cell lymphoma is associated with what karyotypes?

+3 and + 18

Burkitts lymphoma is associated with what karyotypes?

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

As well as lymphadenopathy what is NHL associated with?

Anaemia, neutropenia, thrombocytopenia

Are marginal and follicular NHL aggressive or indolent?

Indolent

How are NHL treated?

With rituximab

What is R-CHOP?

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

Is diffuse large B cell indolent or aggressive?

Aggressive

What is myeloma?

Neoplastic accumulation of bone marrow plasma cells

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

Usually IgG or IgA

What is the incidence of myeloma?

7.1 per 100,000

What is the median age of myeloma?

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

What are common karyotypes of myeloma?

Majority have hyperploidy -13 -13q 11q abnormalities

Why is hypercalcaemia associated with myeloma?

Osteoclasts are breaking down bone leading to the release of calcium

What is CRAB?

Hypercalcaemia, renal impairment, anaemia and bone disease | Clinical features for myeloma

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

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

What is the outcome of myeloma?

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