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Haematology > Leukemia > Flashcards

Leukemia Flashcards

1
Q

Leukemias

A

malignant neoplasms of the haemopoietic stem cells, characterised by diffuse replacement of the bone marrow by neoplastic cells

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2
Q

Classification

A

by lineage and degree of maturity of the leukaemic clone

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3
Q

which diseases is the philadelphia (Ph) chromosome associated with?

A

found in 95% of cases with CML and some patients with AML

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4
Q

what happens in the Ph chromosome?

A

the long arm of ch22 is shortened by reciprocal translocation to the long arm of ch9 (t(9;22))

210-kDa fusion protein product of the resulting ‘fusion’ gene, BCR-ABL, has tyrosine kinase activity, and enhanced phosphorylating activity compared with the normal protein –> altered cell growth, stromal attachment and apoptosis

uncontrolled cell division

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5
Q

environmental factors

A

chemical e.g. industrial benzene compounds
drugs e.g AML occurs after Tx with alkylating agents e.g. melphalan
radiation exposure

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6
Q

What are the acute leukaemia characterised by?

A

clonal proliferation of myeloid or lymphoid precursors with reduced capacity to differentiate into more mature cellular elements

accumulation of laekaemic cells in the BM, peripheral blood and other tissues, with a reduction in RBCs, platelets and neutrophils

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7
Q

epidemiology acute leukemia

A

ALL is predominantly a disease of childhood (L for little)

AML seen most frequently in older adults (M for mature)

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8
Q

clinical features acute leukemia

A

resulting from marrow failure:

  • anaemia
  • bleeding
  • infection e.g. sore throat and pneumonia

? peripheral lymphadenopathy and hepatosplenomegaly

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9
Q

Ix acute leukemia

A

peripheral blood film

BM aspirate

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10
Q

what does the blood count show in acute leukemia

A

anaemia and thrombocytopenia

white cell count is usually raised, but may be normal or low

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11
Q

what does the blood film show in acute leukemia

A

characteristic blast cells

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12
Q

what histological feature is characteristic of AML?

A

Auer rods (a rod-like conglomeration of granules in the cytoplasm)

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13
Q

what does BM aspirate show in acute leukaemia?

A

increased cellularity, with a high % of abnormal lymphoid or myeloid blast cells

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14
Q

Mx acute laeukemia

A

induction chemo to return peripheral blood and BM to normal

supportive care prior to treatment: correction of anaemia and thrombocytopenia, treat infection with IV antibiotics

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15
Q

proposed pathogenesis of AML

A

myeoblasts are stopped from differentiating further.

2 mutations?
one –> rapid proliferation of myeoblast and its resistance to apoptose
2nd: blocks its further differentiation

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16
Q

what must BM biopsy show for AML to be diagnosed?

A

20% blast cells

17
Q

Methods of genetic testing

A

FISH

PCR

18
Q

Tx AML

A

walking exercises to reduce fatigue
supportive care

chemo e.g. cytarabine and daunorubicin

HLA sibling matched allogenic bone marrow transplant

19
Q

Why does a bone marrow transplant allow for very aggressive chemo?

A

due to reduced risk of bone marrow failure

20
Q

Acute lymphoblastic leukaemia prognosis

A

Children- excellent: 80% cured at 5yr. Most common paediatric malignancy

Adults- worse with age: 30% being cured

21
Q

How does Tx differ in AML to ALL

A

ALL has the propensity to involve the CNS, so Tx includes prophylactic intrathecal drugs: methotrexate or cytosine arabinoside (cytarabine)

Cranial irradiation sometimes used

22
Q

Significance of Ph chromosome in ALL?

A

It is a poor prognostic factor

Its more common in adults

23
Q

Who does chronic myeloid leukaemia occur in?

+ survival rate

A

middle aged
characterised by the presence of the Philadelphia (Ph) chromosome

survival median: 5-6yrs

24
Q

Chronic phase of CML

A

Lasts 3-4 yrs if untreated
- insidious onset: fever, wt loss, sweating and Sx of anaemia. massive splenomegaly

Followed by blast transformation, with development of AML and commonly, rapid death

Or could transforms to myelofibrosis, death ensuing from BMF

25
Q

Blood count CML

A

anaemia and raised white cell count

26
Q

BM aspirate CML

A

hypercellular marrow

neurtrophilia- with whole spectrum of mature myeloid precursors increased

27
Q

cytogenetics CML

A

translocation t(9;22), presence of Ph chromosome

also shown on reverse transcriptase polymerase chain reaction (RT-PCR)

28
Q

1st line Tx for the chronic phase of CML- response rate, and side effects

A

tyrosine kinase inhibitor Imatinib specifically blocks the enzymatic action of the BCR-ABL fusion protein

95% complete response

SEs: nausea, headache, rashes, cytopenia, cramps, oedema

29
Q

Tx CML in the acute phase (blast transformation)

A

only short-lived response to imatinib

other chemo as for acute leukaemia, to try to achieve a 2nd chronic phase

30
Q

what’s hydroxycarbamide?

+SEs

A

Chemotherapy drug
usually used for sickle-cell disease

Prevents DNA synthesis and repair

SEs: hepatic/renal impairment
headache, dizziness, rash

31
Q

Chronic lymphocytic leukemia

A

incurable disease of older people

uncontrolled proliferation + accumulation of mature B lymphocytes

Escaped programmed cell death
cell cycle arrest in G0-G1 phase

32
Q

Clinical features CLL

A

indolent course

early CLL: asymptomatic, isolated peripheral blood lymphocytosis is frequent

?lymphadenopathy, and in advanced disease, hepatosplenomegaly

33
Q

Blood count CLL

A

raised white cell count with lymphocytosis

may be anaemia and thrombocytopenia

34
Q

Blood film CLL

A

small lymphocytes of mature appearance with ‘smear or smudge cells’- due to cell rupture while film being made

35
Q

What is decision to treat dependent on in CLL?

A

stage of the disease
cytogenetic markers

early-stage: treat expectantly
advanced: treat immediately

36
Q

What Tx successfully palliates the disease in CLL?

A

oral chlorambucil, with or without prednisolone. reduces blood count + decreases lymphadenopathy + splenomegaly

BM rarely returns to normal

37
Q

what helps autoimmune haemolysis in CLL?

A

steroids

38
Q

What Tx for CLL has greater impact on BM and can induce remission?

A

fludarabine + cyclophosphamide +/- rituximab

39
Q

Poor prognostic markers for CLL

A

11p or 17q deletions (the site of 2 tumour suppressor genes)

Haematology (10 decks)

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