Leukaemia and myeloid diseases Flashcards

1
Q

What is myelodysplastic syndrome?

A

A clonal blood disorder characterised by ineffective haemopoiesis

Pre-leukaemic

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2
Q

How is myelodysplastic syndrome differentiated from acute leukaemia in bone marrow biopsy?

A

> 20% of marrow cells blast cells, its leukaemia

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3
Q

How is MDS treated

A

Need SCT to cure. But since it mostly affects the elderly, normally supportive e.g. blood transfusion, drugs such as azacitidine chemo

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4
Q

What are myeloprofilerative disorders?

A

Clonal blood disorders characterised by effective haemopoiesis

Megakaryocytes (+WBCs)= myelofibrosis
Red cells = polycythaemia vera
Platelets = essential thrombocytopenia

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5
Q

How are ET and PRV treated?

A

Aspirin to reduce risk of thrombotic events

Cytoreduction e.g. venesection, hydroxycarbamide

Can cause AML and myelofibrosis

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6
Q

How does myelofibrosis present?

A

Splenomegaly (haematopoeisis occuring), fatigue, elderly, systemic weight loss/night sweats

High urate and LDH due to cell turnover

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7
Q

How is myelofibrosis treated?

A

SCT is only curable

JAK2 inhibitors: often mutations involved

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8
Q

What is ALL/

A

Malignancy of lymphoid blast cells. Usually occurs in children, with good prognosis (worse if older)

Genetics implicated (translocations)

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9
Q

What are Sx of ALL?

A

Bone marrow failure = SOB, fatigue (anaemia); infections (low WCC); bruising and bleeding (low platelets).

infiltration: hepatosplenomegaly, lymphadenopathy, bone pain

Can be CNS and testicular infiltration

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10
Q

What are predisposing factors in ALL?

A

Downs
Fanconi
ionising radiation in pregnancy
Klinefelter

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11
Q

What Ix for ALL/

A

FBC: will show decreased; WCC high with severe neutropenia

Blood smear: leukaemic cells

Bone marrow biopsy: blast cells > 20%.

Also can do cytogenetics, routine bloods

imaging for any lymphadenopathy

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12
Q

Treatment for ALL?

A

Chemo regimen. BMT if younger

CNS prophylaxis.

Supportive: blood and platelets, allopurinol, fluids

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13
Q

Complications of ALL/

A
Febrile neutropaenia
Tumour lysis syndrome
Chemo s/e
Leukostasis 
Steroid s/e
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14
Q

What is CLL?

A

malignant proliferation of a clone of mature B cells in peripheral blood

most common leukaemia

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15
Q

What are Sx of CLL?

A

Mostly infections - lack of functioning B cells. Also lymphadenopathy, anaemia, splenomegaly

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16
Q

What Ix for CLL?

A

FBC: often found on here as incidental high lymphocytes, w low neutrophils, anaemia, low platelets

Can be AIHA, ITP

Blood smear: spherocytes and polychromasia

Flow cytometry

17
Q

What is Rx for CLL?

A

Watch and wait if asymptomatic.

Treat if symptomatic or progressing (chemo) - SCT only curable but not good in older patients ,

18
Q

What are complications of CLL/

A

Richter tranformation to NHL
MAHA
ITP
Hypogammaglobulinaemia

19
Q

What is AML

A

Clonal expansion of myeloid blasts in bone marrow and peripheral blood

De novo or following MDS or certain cytotoxic drugs

20
Q

What is APML?

A

Subtype of AML with distinct cytological features e.g. normal WCC, bilobed nuclei, Auer rods.. treated differently and more aggressive

21
Q

What is Sx of AML?

A

Same as for CLL (infiltration and bone marrow failure). APML can cause DIC.

22
Q

What ix in AML:?

A

FBC: high WCC low neutrophils, everything low.

(urea and LDH high)

Blood smear - Auer rods differentiate from ALL
bone marrow biopsy needed for diganosis

Cytogenetics and immunophenotyping for classifying AML and tailoring treatment

23
Q

What is Rx for AML?

A

Aggresive chemo

same as for CLL

24
Q

What is CML

A

Malignant clonal disorder of mature myeloid cells in bone marrow

Philadelphia chromosome

25
Q

What are Sx of CMl

A

may be none.

Fatigue, weight loss, night sweats, abdominal pain due to splenomegaly

Chronic, accelerated, then blast phase

26
Q

What Ix in CML?

A

FBC: raised WCC, anaemia, platelets can vary depending on the phase.

Blood smear: myeloid cells. Basophils > 20% = accelerated phsae

Bone marrow biopsy needed for phase of disesae

Cytogenetics/FISH/PCR for t(9,22)

27
Q

What is Rx for CML?

A

Imatinib: TKI

Can add in 2/3rd generations

Chemo if not working

28
Q

What are complications of CML?

A

Related to TKI mainly e.g. muscle cramps, QTc, myelosuppression, rash