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Pathology (Guide) > Haematology - Leukaemia > Flashcards

Haematology - Leukaemia Flashcards

1
Q

What is leukaemia and some features?

A

A neoplastic process affecting blood precursor cells

  • Acute, rapidly progressing + fatal
  • Immature blasts >20% of bone marrow cells (rapid proliferation replaces normal bone marrow)
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2
Q

What are some clinical features of an acute leukaemia?

A

BM function failure (ANT)
- A: Anaemia
- N: Neutropenia (infection)
- T: Thrombocytopenia (bleeding)

Organ infiltration:
- Hepatosplenomegaly
- Lymphadenopathy (mild)
- Bone pain
- CNS
- Skin
- Gum hypertrophy

+ Fevers

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3
Q

What are some causes of acute leukaemia?

A
  • UNKNOWN
  • Ionising radiation
  • Benzene
  • Pre-leukaemic disorders (myelodysplastic syndromes/myeloproliferative disorders)
  • Down’s: Increased risk of AML/ALL
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4
Q

How is acute leukaemia diagnosed?

A
  • Morphology +/- cytochemistry (stains)
  • Immunophenotyping using flow cytometry (lineage, differentiation)
  • Cytogenetics (chromosomal translocations)
  • Molecular genetics (PCR, point mutations)
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5
Q

What is leukostasis, its signs and management?

A

Haematological emergency
- Increased WBC causes viscous blood and end-organ damage

Features:
- Retinopathy
- Pulmonary infiltrates
- Bleeding
- Thrombosis

Mx:
- Leukophoresis
OR
- Chemotherapy/steroids

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6
Q

What is the epidemiology of ALL?

A

Childhood

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7
Q

What are some clinical features of ALL?

A
  • Hepatosplenomegaly
  • Lymphadenopathy
  • CNS involvement
  • Testicular enlargement (rare)
  • Thymic enlargement (mediastinum)
  • Bone pain + lump
  • Fevers
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8
Q

How is ALL investigated?

A
  • High WCC (blasts)
  • Thrombocytopenia
  • Anaemia

Flow cytometry:
- CD34 = precursor/stem cells
- CD3, 4, 8 = T-lymphocytes
- CD19, 20, 22 = B-lymphocytes

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9
Q

What is the treatment for ALL?

A

Chemotherapy
- Remission induction = Chemo + steroids
- Consolidation = High dose multi-drug chemo + CNS Tx (intrathecal chemo)
- Maintenance = 2yrs in girls/adults + 3yrs in boys

?Allo-stem cell transplant IF high risk of relapse

Targeted Tx:
- Nelarabine
- CAR-T cells
- Inotuzumab
- Blinatumumab (B-ALL), imatinib (IF: t9;22)

Supportive:
- Blood products
- Abx
- Allopurinol
- Fluid
- Electrolytes (prevent tumour lysis syndrome)

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10
Q

What is the epidemiology of AML?

A

Adulthood
- Risk increases with age

OR under 2s (infant peak)

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11
Q

What are some clinical features of AML?

A
  • Lymphadenopathy
  • Sx of cytopenias
  • M3 = prone to DIC + bleeding
  • M4/5 = Monoblasts/monocytes (skin/gum infiltration + hypokalaemia)
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12
Q

How is AML investigated?

A
  • High WCC (blasts)
  • Auer rod + granules

Flow cytometry:
- CD34 = precursor/stem cells
- CD33, 13, 117, MPO = myeloid cells

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13
Q

What is the treatment for AML?

A

Chemotherapy
- Remission induction = Daunorubicin + Cytarabine
- Consoloidation = Cytarabine

Older pts = azacytidine +/- venetoclax

?Allo-SCT IF high risk of relapse

Targeted Tx:
- ATRA (for acute promyelocytic leukaemia)
- Midostaurin - FLT3 mutations
- Gemtuzumab - CD33 immunotherapy
- Enasidenib - IDH mutations

Supportive Tx:
- Blood products
- Abx
- Allopurinol
- Fluid
- Electrolytes (prevents tumour lysis syndrome)

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14
Q

What is the prognosis of AML?

A

Worse with age

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15
Q

What mutation causes Acute Promyelocytic leukaemia, how does it present, how is it treated and what’s the prognosis?

A
  • T(15;17)
  • Presents with DIC
  • Tx: ATRA (All-trans retinoic acid) - forces cells to differentiate (stops proliferation)
  • Px: Good after induction
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16
Q

What is a common mutation causing ALL in adults?

A

t(9;22) - BCR-ABL1 (Philadelphia chromosome)

17
Q

What blood results are a concern for malignancy?

A

Increased basophils + eosinophils

18
Q

What is CML and its epidemiology?

A

Myeloproliferative disease with a slower proliferation of malignant cells

Epi:
- Middle ages (40-60yrs)
- Males

19
Q

How does CML present?

A

Asymptomatic if in chronic phase

Accelerated/blast phase Sx:
- General unwell feeling
- Weight loss
- Infections
- Bruising
- MASSIC SPLENOMEGALY
- Lymphadenopathy

20
Q

What are the three phases of CML?

A
  1. Chronic phase
  2. Accelerated phase
  3. Blast phase
21
Q

What are some investigations for CML and how is it usually diagnosed?

A

Dx:
- Routine bloods (Increased differentiated neutrophils)

Ix:
- Bloods (High WCC, neutrophils + basophils)
- Hypercellular BM with spectrum of immature (myelocytes) + mature granulocytic cells in blood
- Left shift (precursors present)
- BM: Hypolobulaetd megakaryocytes
- PCR (BCR-ABL1)
- Chromosome studies (t(9;22) - Philadelphia chromosome)

22
Q

What is seen in the chronic phase of CML and how is it treated?

A
  • <5% blasts in BM/blood
  • WBC increases over years

Tx:
- Imatinibi (BCR-ABL tyrosine kinase inihibitor)
OR dasatinib/nilotinib for resistance
- Tx started immediately

23
Q

What is seen in the accelerated phase of CML and how is it treated?

A
  • > 10% blasts in BM/blood
  • Increasing manifestations (e.g. splenomegaly), lasting <1yr

Less responsive to therapy (?imatinib)

24
Q

What is seen in the blast phase of CML and how is it treated?

A
  • > 20% blasts in BM/blood
  • Resembles acute leukaemia (timeframe = months)

Tx:
- Similar to AML (daunorubicin/cytarabine + supportive)
- ?Allo-SCT for young pts

25
Q

What is CLL and how does it differ from SLL (Small lymphocytic lymphoma)?

A

Lymphoproliferative disease (like lymphoma)

  • CLL primarily seen in BM
  • SLL primarily seen in LNs
26
Q

What is the epidemiology of CLL?

A
  • Males more common than females
  • Elderly (Mean: 65-70yrs) - incidence increases with age
27
Q

What are some clinical features of CLL?

A

Asymptomatic (often diagnosed on routine bloods)
OR
- Symmetrical, painless lymphadenopathy
- BM failure (Anaemia + thrombocytopenia Sx)
- Weight loss, low-grade fevers, night sweats
- Hepatosplenomegaly
- Associated with autoimmunity (Evan’s syndrome) - AIHA/ITP

28
Q

What is Richter’s syndrome?

A

The transformation of CLL to aggressive disease (e.g. ALL/high-grade lymphoma)

29
Q

What investigations are done for CLL and what are the results?

A
  • High WCC (lymphocytosis >5%)
  • Low serum immunoglobulin
  • Blood film = SMEAR CELLS (+lymphocytosis) or SMUDGE CELLS
  • Flow cytometry = monoclonal population (CD5+ CD23+)
  • Abnormal BM (lymphocytic replacement)
30
Q

What mutation statuses indicate a better and worse prognosis for CLL?

A

TP53 = worse
IGHV = better

31
Q

What are some good and bad prognostic factors for CLL?

A

Good:
- Hypermutated Ig gene
- Low ZAP-70 expression
- 13q14 deletion

Bad:
- LDH raised
- CD38 +ve
- 11q23 deletion

32
Q

What staging criteria is used for CLL and what are the different factors in it?

A

Binet staging (A, B, C)

A:
- High WBC
- <3 groups enlarged LNs
- No cytopenia

B:
- >3 groups enlarged lymph nodes
- No cytopenia

C:
- Anaemia or thrombocytopenia
- Cytopenias

33
Q

What stages of CLL require treatment?

A

B + C

A = watch + wait

34
Q

What is the treatment for CLL?

A
  • Watchful waiting if asymptomatic
    OR
  • Anti-20 (rituximab) + chemotherapy
  • Oral BTK inhibitors (ibrutinib)
  • BCL2 inhibitor (venetoclax)

Supportive Tx:
- Transfusions
- Infection prophylaxis

35
Q

What treatment is required fro TP53 + IGHV mutations in CLL?

A

TP53 = BTK inhibitors (e.g. ibrutinib)

IGHV = rituximab + chemotherapy

Pathology (Guide) (84 decks)

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