Term
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Definition
malignant neoplasm of lymphoblastic cell lineage. 80% - B cell 20% - T Cell |
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Term
what is the peak onset for ALL? |
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Definition
4yrs Gradually increasing risk as age increases so second peak in elderly |
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Term
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Definition
Morphologically: FAB system L1: small, uniform blasts, L2: Large blasts with dec nucleus:cytoplasm. L3: Basophilic, vacuolated blasts.
Immunologically: According to cell surface antigens. e.g pre B cell etc etc. |
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Term
What are the clinical features of ALL? |
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Definition
anaemia - tiredness/weakness/pallor/breathless Recurrent infections Bruise/bleed easily lymphadenopathy/splenohepatomegaly (dissemination of leukaemic cells) wt. loss/anorexia CNS involvement = N&V Bone pain = sore legs |
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Term
On what evidence is a diagnosis of ALL made? |
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Definition
Blood count: dec Hb pancytopenia thrombocytopenia neutropenia (althou init increased - infection)
Blood film: abnormal blast cells normochromic normocytic anaemia
aspirate/trephine increased cellularity leaukaemic cells seen
cytochemistry - staining
immunohistochemisty - determine lineage
cytogenetics
Others: U+E's CXR LFT's Coagulation screen Lumbar Puncture Urate Viral titres |
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Term
On what evidence is a diagnosis of ALL made? |
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Definition
Blood count: dec Hb pancytopenia thrombocytopenia neutropenia (althou init increased - infection)
Blood film: abnormal blast cells normochromic normocytic anaemia
aspirate/trephine increased cellularity leaukaemic cells seen
cytochemistry - staining
immunohistochemisty - determine lineage
cytogenetics
Others: U+E's CXR LFT's Coagulation screen Lumbar Puncture Urate Viral titres |
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Term
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Definition
chemo = mainstay. 1. Induction - vincristine, L-asparginase & prednisolone. aimed at eradicating blast from bone marrow.
2. Consolidation - destroy any remaining blasts and prevent resistance.
3. Maintanence - cycles of chemo to complete 2yr Rx.
CNS Protection - cranial irradiation or intrathecal MTX. |
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Term
In ALL what are the differences in response to treatment between children and adults? |
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Definition
Children - 90% respond 10% need BMT
Adults - 20-30% (often palliative) |
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Term
what are the immediate s/e of chemo in ALL? |
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Definition
1. Infection esp if indwelling catheters avoid live vaccines/contact with infection Short term: Bacterial (Vancomy/teicoplanin) Long term: Viral/fungal - corsadyl mw
2. Bleeding (thrombocytopenia) need prophylactic transplant
3. Tumour lysis synd. chemo causes lysis of blast cells = massive release of cellular breakdown products. Hyperkalcaemia (salbutamol/dialysis) HyperPO4- but hypocalcaemia
4. Decreased cell turnover = alopecia and mouth ulcers. |
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Term
What are the late s/e of chemo in ALL? |
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Definition
Cognition - cranial erad/CNS inv Osteoporosis - corticosteriod Rx Growth - pit damage/dec GH Cardiac toxicity Also - Hep C pre 1991 (blood prod) Tumours - 2ry to chemo - meningioma |
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Term
Describe the impact of ALL |
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Definition
Disrupts family unit younger pts = more accepting/afraid of tmnt older = want to be with friends/alopecia a prob siblings feel neglected financial difficulties childs education suffers |
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Term
What are the ethical issues surrounding the use of siblings as bone marrow doners? |
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Definition
Risks = anaesthetic/blood trans Benefits = joy of pts survival!
Need to reassure that if transfusion fails its not their fault. |
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