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Haematology > Concepts in malignant haematology and acute leukaemia > Flashcards

Concepts in malignant haematology and acute leukaemia Flashcards

1
Q

What are the kinetics of normal haemopoiesis?

A 
Self-renewal 
Proliferation 
Differentiation or lineage commitment 
Maturation 
Apoptosis
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2
Q

How can normal mature, non-lymphoid cells be identified?

A 
Morphology
Cell surface antigens (glycophorin A = red cells) 
Enzyme expression (myeloperoxidate = netrophils)
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3
Q

How can normal progenitors/ stem cells be identified?

A

Cell surface antigens; immunophenotyping (CD34)

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4
Q

What is malignant hematopoiesis characterized by?

A

Increased numbers of abnormal and dysfunctional cell

Loss of normal activity

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5
Q

What is leukaemia characterized by?

A

Dysfunctional haemopoieis

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6
Q

What is lymphoma characterized

A

Dysfunctional immune system

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7
Q

What can be encompasses by dysfunctional haemopoesis?

A

Increased proliferation
Lack of differentiation
Lack of maturation
Lack of apoptosis

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8
Q

What is acute leukaemia?

A

Proliferation of abnormal progenitors with block in differentiation/ maturation (AML)

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9
Q

What are chronic myeloproliferative disorders?

A

Proliferation of abnormal progenitors but NO differentiation/ maturation block (CML)

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10
Q

What is a clone?

A

Population of cells derived from a single parent cell

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11
Q

What type of haemopoiesis is polyclonal?

A

Normal

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12
Q

What type of haemopoiesis is monoclonal?

A

Malignant

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13
Q

What do driver mutations confer?

A

Growth advantage on cells

Selected during evolution of cancer

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14
Q

What do passenger mutations confer?

A

No growth advantage

Present in ancestor cancer cell when a driver mutation was acquired

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15
Q

How are haematological malignancies classified?

A

Lineage
Developmental stage (precursor) within lineage
Anatomical site involved

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16
Q

What are the different types of haematological malignancies based on lineage?

A

Myeloid

Lymphoid

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17
Q

How are haematological malignancies classified based on anatomical site involved?

A

Blood = leukaemia
Lymph node = lymphoma
**CLL can involved blood and lymph nodes **
Myeloma = plasma cell malignancy in marrow

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18
Q

What is the difference in presentation of acute leukaemias and high grade lymphomas in comparison with chronic leukaemias and low grade lymphomas?

A

Histologically and clinically; acute and high grade are more aggressive

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19
Q

What are features of histological aggression?

A

Large cells with high nuclear:cytoplasmic ratio
Prominent nucleoli
Rapid proliferation

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20
Q

What are features of clinical aggression?

A

Rapid progression

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21
Q

How will acute leukaemias be differentiated from chronic leukaemias?

A

Acute leukaemias present with a failure of normal bone marrow function

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22
Q

Which malignancies of the haem system involve the most primitive cells?

A

Chronic myeloid leukaemia/ chronic myeloproliferative disorders - stem cell issue

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23
Q

Which part of haemopoiesis will acute myeloid leukaemia affect?

A

Myeloid progenitor cells - will go on to form erythrocytes, megakaryocytes, granulocytes and monoctytes

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24
Q

Which part of haemopoiesis will acute lymphoblastic leukaemia affect?

A

Common lymphoid progenitor cells - will go on to form T cells, B cells and NK cells

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25
Q

Which part of haemopoiesis will chronic lymphocytic leukaemia affect?

A

B cells

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26
Q

Which part of haemopoiesis will myeloma affect?

A

Plasma cells

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27
Q

What is acute leukaemia?

A

Rapidly progressive clonal malignancy of the marrow/ blood with maturation defects
Defined as an excess of blasts (>20%) in either the peripheral or bone marrow
Los of normal haemopoietic reserve

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28
Q

What are the different forms of acute leukaemia?

A 
Acute myeloid (AML) 
Acute lymphoblastic (ALL)
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29
Q

What is ALL?

A

Malignant disease of primitive lymphoid cells (lymphoblasts)

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30
Q

What is the most common childhood cancer?

A

ALL

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31
Q

What is the clinical presentation of ALL?

A

Marrow failure; anaemia, infections, bleeding
Leukaemic; high count with obstruction of circulation
Involvement of areas outside marrow and blood; CNS, testis
Bone pain

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32
Q

Who will AML affect?

A

Elderly >60yrs

Can be denovo or secondary

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33
Q

What is the presentation of AML?

A

Similar to ALL

Subgroups may have characteristic presentation; DIC or gum infiltration

34
Q

What are the investigations indicated for acute leukaemia?

A

Blood count and film
Coag screen; PT, APTT and fibrinogen
Bone marrow aspirate (posterior iliac crests)

35
Q

What will the blood film show in acute leukaemia?

A

Reduction in normal cells
Presence of abnormal cells - increase in blasts with a high nuclear:cytoplasmic ratio
AUER ROD (AML)

36
Q

What feature of the blood film is pathognomonic of AML?

A

Auer Rod

37
Q

What tests should be performed on the bone marrow aspirate?

A

Morphology; what do the cells look like?

Immunophenotyping via flow-cytometry; are there lineage specific proteins on cell surface?

38
Q

What is required for a definitive diagnosis of ALL from AML?

A

Immunophenotyping

Very important because the treatment of AML and ALL differ

39
Q

What does a trephine bone marrow biopsy allow for?

A

Better assessment of cellularity and helpful when aspirate samples are suboptimal

40
Q

What is the curative treatment of ALL?

A

Can last up to 2-3 years
Different phases of treatment of varying intensity
Targeted treatments in certain subsets

41
Q

What is the curative treatment of AML?

A

Intensive
2-4 cycles of chemo (5-10 days of chemo followed by 2-4 weeks of recovery)
Prolonged hospitalisation
Target treatments in subsets

42
Q

What is a hickman-line?

A

Central venous catheter that sits in the SVC and used for administration of chemo

43
Q

What are the problems with marrow suppression?

A

Anaemia
Neutropenia; infections
Thrombocytopenia; purpura, petechiae (plt <20), bleeding gums

44
Q

What are the most dangerous bacteria in marrow suppressed patients?

A

Gram negative bacteria can cause fulminant life-threatening sepsis in neutropenic patients

45
Q

What are the complications of chemo?

A 
N+V
Hair loss
Liver and renal dysfunction 
Tumour lysis syndrome (during first course of tx) 
Infection 
Loss of fertility
46
Q

What specific side effect with anthracyclines cause?

A

Cardiomyopathy

47
Q

What is very important to do if neutropenic occurs in a patient?

A

Empirical tx with broad spectrum antibiotics specifically covering gram negs

48
Q

What agent should be added on in the instance of persisting fever and neutropenia unresponsive to antibiotics?

A

Fungal protection

49
Q

What protozoan needs to be covered in ALL Therapy?

A

PJP

50
Q

What is remission in acute leukaemias?

A

<5% marrow blasts with recovery of normal haemopoiesis

51
Q

What is an example of targeted treatment in ALL?

A

Kinase inhibitors in ALL with philadelphia chromosome

52
Q

Which AML subtype predisposes to DIC?

A

Chromosomal translocation in t(15;17)

Results in DIC

53
Q

What is tumour lysis syndrome?

A

Increased potassium, urate

AKI

54
Q

What are the symptoms of DIC?

A

Bruising
Bleeding
Renal failure

55
Q

What are the blood test signs of DIC?

A 
Decreased platelets 
Increased PT and APTT 
Decreased fibrinogen 
Increased d-dimers 
Blood film; schistocytes
56
Q

What are important associations of ALL?

A

X-rays during pregnancy

Down syndrome

57
Q

What are the si/sy of marrow failure?

A

Anaemia; decreased Hb
Infection; decreased WCC
Bleeding; decreased platelets

58
Q

Where can acute lymphoblastic leukaemia infiltrate?

A

Hepato and splenomegaly
Lymphadenopathy
Orchidomegaly
CNS involvement; meninginism, CN palsy

59
Q

What is the characteristic test results for ALL?

A

Blast cells on film and marrow

WCC high

60
Q

Are the cure rates high for ALL?

A

Yes; children cure rate is 70-90%

61
Q

What confers poor prognostic factors in ALL?

A 
Adult
Male 
Philadelphia chromosome (BCR-ABL gene fusion due to translocation of chromosomes 9 and 22) 
Presentation with CNS signs 
Decreased Hb
WCC >100 
B-cell ALL
62
Q

What is diagnosis of AML dependent on?

A

Bone marrow biopsy
Immunophenotyping
Molecular methods
AML is differentiated from ALL via auer rods

63
Q

What are myelodysplastic syndromes?

A

Disorders that manifest as marrow failure with risk of life-threatning infection and bleeding
Can develop secondary to chemo or radiotherpay
Can transform to acute leukaemia

64
Q

What are the test results for myelodysplastic syndromes?

A

Pancytopenia
Decreased retic count
Marrow cellularity increased due to ineffective hematopoiesis
Ring sideroblasts

65
Q

Which form of leukaemia is philadelphia chromosome most implicated?

A

CML
Reciprocal translocation between long arm of chromosome 9 and long arm of 22 resulting in a fusion of gene BCR/ABL which has tyrosine kinase activity

66
Q

What are the symptoms of CML?

A 
Decreased weight 
Tiredness
Fever 
Sweats 
Gout 
Bleeding
Abdo discomfort; splenomeglay
67
Q

What are the signs of CML?

A

Splenomegaly
Hepatomegaly
Anaemia
Bruising

68
Q

What will the tests show in CML?

A 
V high WCC (>100) with whole spectrum of myeloid cells; neutrophils, monocytes, basophils, eosinophils 
Decreased Hb 
Increased urate 
Increased B12 
Bone marrow hypercellular
69
Q

What is the commonest leukaemia?

A

CLL

70
Q

What is the hallmark of CLL?

A

Progressive accumulation of a malignant clone of functionally incompetent B cells
Mutations, trisomies and deletions influence risk

71
Q

What are the symptoms and signs of CLL?

A

Often no symptoms; can be anaemic, infection prone or have wt loss, sweats and anorexia
Signs; enlarged, rubbery non tender nodes. Splenomegaly, hepatomegaly

72
Q

What will the tests show in CLL?

A

Increased lymphocytes
Autoimmune haemolysis
Marrow infiltration; decreased Hb, decreased neutrophils, decreased platelets
Bone marrow; smudge/ smear cells

73
Q

What is the natural history of CLL?

A

1/3rd never progress
1/3rd progress slowly
1/3rd progress actively

74
Q

on the bone marrow, what is pathognomonic of CLL?

A

Smear/ smudge cells

75
Q

What can cause pancytopenias?

A

Decreased marrow production; aplastic anaemia, infiltration, megaloblastic anaemia, myelofibrosis
Increased peripheral destruction; hypersplenism

76
Q

What is agranulocytosis?

A

Granulocytes have stopped being made

Commonly via drugs; carbimazole, procainamide, sulphonamides, clozapine, dapsone

77
Q

What is the treatment for tumour lysis syndrome?

A

Allopurinol

78
Q

In AML and CML which cells in particular will be increased?

A

Look for an increase in neutrophil count

79
Q

In ALL and CLL what cells in particular will be increased?

A

Look for an increase in lymphocyte count

80
Q

Which leukaemia in particular is associated with the Philadelphia chromosome?

A

CML - translocation between chromosome 9 and 22

Treat with tyrosine kinase inhibitor

Haematology (31 decks)

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