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March Exams > Haematology > Flashcards

Haematology Flashcards

0
Q

What happens at G1-S checkpoint?

A

Cell checks integrity of DNA to ensure that it is in a fit state to be replicated

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1
Q

Definition of haemopoietic growth factors

A

Soluble mediators that regulate proliferation and differentiation of haemopoietic progenitors
- exert their effect by binding to high affinity receptors on cell surface

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2
Q

What happens at G2 checkpoint?

A

Checks that DNA replication has been satisfactory.

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3
Q

Definition of apoptosis

A

Regulated process of physiological cell death

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4
Q

Why apoptosis and not just necrosis?

A

Ensures the destruction of the cell without releasing any lysosomal or granule content that would cause an inflam reaction

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5
Q

2 pathways to activate apoptotic enzymes

A
  • receptor mediated

- mitochondrial

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6
Q

Steps in apoptosis

A
  • activation of caspase proteases
  • cell shrinkage
  • cleavage of DNA
  • condensation of nuclear chromatin
  • fragmentation of nucleus
  • phagocytosis of apoptotic bodies by macs
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7
Q

Describe the receptor mediated pathway

A
  • signaling through fas or TNF receptor via an intracellular death domain
  • triggers intracellular cascade of caspase a
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8
Q

Describe mitochondrial pathway

A
  • irreparable damage to cell contents triggers cytochrome C release from mitochondria
  • activates caspases
  • regulated by various Bcl proteins (control perm of mitochondrial membrane)
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9
Q

How does p53 activate apoptosis?

A
  • raises the level of BAX

- increases cytochrome C release

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10
Q

Protection from apoptosis

A
  • Bcl 2

- ratio of BAX to Bcl 2 determines cells susceptibility to apoptosis

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11
Q

How apoptosis affected in follicular lymphoma tr

A

Translocation of Bcl 2 to immunoglobulin heavy chain locus

  • over expression if blc 2
  • reduced apoptosis
  • accumulation of malignant cells
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12
Q

Definition of clonal evolution

A

If the mutation in the progeny gives it a survival advantage above the parent, the original cell may be replaced

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13
Q

What is classification of leukemia based on?

A
  • morphology/phenotype/ flow cytometry
  • cytogenetics
  • molecular genetics
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14
Q

Metabolic effects of ALL and Burkitts

A
  • tumour lysis syndrome
  • hyperuricaemia
  • fever
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15
Q

Definition of acute leukemia

A

Presence of at least 20% blasts in peripheral blood or bone marrow

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16
Q

Definition of blasts

A

Immature cells located in the bone marrow that differentiate though series of cell divisions into mature cells present in normal peripheral blood

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17
Q

Normal % blasts

A

<5

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18
Q

Broad categories of AL

A
  • Acute lymphoblastic leukemia
  • acute myeloid leukemia
  • mixed phenotype (rare)
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19
Q

Non-specific features of AL

A
  • unwell
  • tired
  • general aches and pains
  • fever
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20
Q

Signs of bone marrow infiltration

A
  • anaemia
  • bleeding tendency
  • infections
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21
Q

Signs of tissue infiltration

A
  • gum hypertrophy
  • lymphadenopathy
  • splenomegaly
  • CNS disease (ALL)
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22
Q

What is classification of AL based on?

A
  • morphology of blood and bone marrow
  • cytochemistry
  • immune phenotype (FC)
  • genetic abnormalities (karyotyping/FISH/PCR)
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23
Q

What is the most common secondary leukemia?

A

AML

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24
Q

Tests for initial diagnosis

A
  • FBC
  • differential count
  • morphology of slide
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25
Q

Tests for definitive diagnosis

A
  • bone marrow aspirate

- bone marrow trephine biopsy

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26
Q

Morphology of ALL

A

Scanty cytoplasm
Primitive nuclei
No granules
No auer rods

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27
Q

Additional tests to diagnose AL

A
  • lumbar puncture to exclude CNS disease
  • HIV test
  • DIC screen (acute promyelocytic leukemia)
  • electrolytes and renal function
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28
Q

Immunophontype markers for blasts

A
  • CD34

- HLA-DR

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29
Q

Chromosome changes seen in haematologic malignancies

A
  • increase or decrease in no. of chromosomes (ALL)
  • loss of chromosomes
  • gain of chromosomes
  • translocations (AML, ALL, Burkitts)
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30
Q

Supportive therapy

A
  • anaemia (filtered irradiated packed red cells to maintain Hb >8g/dl
  • thrombocytopenia (plt transfusion)
  • prevention of gout and TLS (hydration and allopurinol)
  • prevention of nausea (anti-emetics to prevent)
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31
Q

How to prevent infections

A
  • single room with revers isolation
  • regular hand-washing by staff and visitors
  • avoid anybody with obvious infection
  • no fresh fruit or vegetables
  • antibiotic and anti fungal prophylaxis
  • attention to mouth care
  • care of IV lines
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32
Q

Definition of induction therapy

A

Course of chemo given to induce remission

  • leukemia no longer detectable in blood or BM
  • reduction in tumour burden by 99%
  • does not imply cure
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33
Q

Definition of consolidation therapy

A
  • further courses of chemo given once remission is achieved

- aims to eradicate disease

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34
Q

Definition of high dose intensification therapy

A

Bone marrow transplant

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35
Q

Definition of maintenance therapy

A

Less intensive, often oral chemo, as an out patients

  • usually given monthly
  • given to maintain remission (usually in ALL)
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36
Q

Two main types of stem cell transplants

A
  • autologous (patients own, returned after chemo)

- allogeneic (matched donor, more risky)

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37
Q

Possible complications of therapy

A
  • TLS
  • normal BM also killed
  • affects normally dividing cells in GIT
  • immune suppression
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38
Q

Bad risk factors for ALL

A
  • age >35
  • high white cell count at diagnosis
  • CNS disease at presentation
  • t(9:22), t(4:11)
  • if it takes more than 4 weeks if weekly chemo to a
    Achieve remission
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39
Q

Definition of Burikk lymphoma

A

A highly aggressive mature B cell neoplasm with extremely short doubling time and high tumour burden

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40
Q

Pathogenesis of Burkitts

A
  • translocations involving myc oncogene
  • most t(8:14)
  • t(2:8)
  • EBV is implicated in pathogenesis
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41
Q

3 clinical variants of Burkitts

A
  • endemic
  • sporadic
  • immunodeficiency-associated
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42
Q

Presentation of endemic Burkitts

A
  • jaw/facial mass
  • 4-7 years
  • EBV in most
  • does not manifest as acute leukemia
43
Q

Presentation of sporadic Burkitts

A
  • massive abdo mass with ascites

- retro peritoneal masses with spinal cord compression

44
Q

Burkitt morphology

A

Monomorphic, medium sized cells

  • deeply basophillic cytoplasm with lipid vacuoles
  • round nuclei
  • finely clumped/ dispersed chromatin
  • multiple nucleoli
45
Q

Prognosis and treatment for Burkitts

A
  • highly aggressive but potentially curable
  • poor prognosis if bone marrow and CNS involvement, high serum LDH
  • very high dose combo chemo used
46
Q

Types of chronic lymphoproliferative B cell leukemias

A
  • chronic lymphocytic leukemia
  • hairy cell leukemia
  • pro lymphocytic leukemia
  • some types of NH lymphomas may spill into blood
47
Q

Types of chronic lymphoproliferative T cell leukemias

A
  • T pro lymphocytic leukemia
  • large granular lymphocytic leukemia
  • adult T cell leukemia
48
Q

Diagnosis of CL

A
  • chronic persistent lymphocytosis
  • morphology
  • immunophenotype
  • cytogenetics
  • genetics
49
Q

Epidemiology of CLL

A
  • 60-80
  • common in western world
  • genetic predisposition
  • monoclonal mature B lymphocytes accumulates
  • only diagnosed if clonal B cells >5x10(9)/l
50
Q

Clinical features of CLL

A
  • mostly picked up on routine blood counts
  • non-tender symmetrical lymph node enlargement
  • anaemia
  • thrombocytopenia
  • splenomegaly
  • immunosuppressive
51
Q

Lab features of CLL

A
  • normochromic anaemia

- decrease in serum Ig

52
Q

Morphology of CLL

A
  • lymphocytes increased
  • small round lymphocytes
  • smear cells
  • immunophenotyping NB
53
Q

Prognostic markers of CLL

A
  • genetics (del17p is poor)
  • rapid lymphocyte doubling time is poor
  • staging
  • LDH
  • high expression of ZAP70 is poor
  • CD38 is poor
54
Q

When not to treat CLL

A

If stage 0/1 - normal life span of 12 years

55
Q

What to treat in CLL

A
  • problematic organomegaly
  • bone marrow suppression
  • haemolytic episodes
56
Q

Chemo treatment for CLL

A
  • fludarabine
  • cyclophosphamide
  • rituximab
    Monthly for 4-6 cycles
57
Q

Presentation of hairy cell leukemia

A
  • rare
  • males 40-60
  • severe infection, anaemia, splenomegaly
  • no lymphadenopathy
  • responds well to chemo
58
Q

Features of B cell pro lymphocytic leukemia

A
  • very rare
  • more aggressive
  • poor prognosis
59
Q

Features of T pro lymphocytic leukemia

A
  • very high WCC

- skin, serous effusions

60
Q

Features of large granular lymphocyte leukemia

A
  • mostly have cytopenias

- RA associated

61
Q

Features of adult T cell leukemia

A
  • common in Caribbean and Japan
  • htlv1 assoc
  • skin lesions, heparosplenomegaly, lymphadenopathy, hypercalcaemia
62
Q

Development of multiple myeloma

A
  • peak in 60s
  • early - over expression of cyclin D
  • accumulation of genetic damaging events
  • important in elderly patient with persistent bone pain
63
Q

Clinical features of Mm

A
  • bone pain
  • anaemia
  • recurrent infections
  • renal failure
  • bleeding (paraproteins interfere)
  • amyloidosis
  • hyper viscosity
64
Q

Investigations for MM

A
  • presence of paraprotein (GADE)
  • serum free light chains
  • urine BJP
  • normochromic normocytic anaemia
  • high ESR
  • skeletal survey - osteolytic lesions
  • increased serum calcium
  • increased creatinine (renal failure)
  • cytogenetics and FISH
65
Q

Supportive management in MM

A 
Renal
- rehydrate
- management of hypercalcaemia and hyperuricaemia
Bone disease
- bisphosphonates
Anaemia and infections
- antibiotics
Bleeding
- plasmapheresis
66
Q

Other plasma cell disorders

A
  • monoclonal gammopathy of undetermined significance

- plasma cell leukemia

67
Q

Definition of amyloidosis

A
  • group of disorders

- deposition of protein in fibrillation form extracellularly

68
Q

Systemic AL amyloid

A
  • clonal proliferation of plasma cells
  • serum paraprotein often very low
  • free light chain ratio skewed
69
Q

Classification of chronic myeloproliferative neoplasms

A
  • chronic myelogenous leukemia
  • polycythemia Vera
  • essential thrombocythemia
  • primary myelofibrosis
70
Q

Common clinical feature of chronic myeloproliferative neoplasms

A
  • splenomegaly and hepatomegaly
  • thrombosis
  • bleeding
71
Q

Features of CML

A
  • results from mutation in haemopoietic stem cell
  • result of translocation t(9:22)
  • results in abnormal fusion gene of BCR/ABL ON chromosome 22 (Philadelphia chromosome)
72
Q

Effects of Philadelphia chromosome

A

Has enhanced tyrosine kinase activity leading to:

  • increased prolif
  • decreased apoptosis
  • decreased adherence to BM to haemopoietic cells to stromal cells
73
Q

Peripheral blood findings in CML

A
  • many WCC
  • all stages of maturation present with myeloctye hump
  • neutrophils usually normal
  • basophils increased
  • normochromic normocytic anaemia
  • platelet count usually increased
74
Q

Bone marrow of CML

A
  • hyper cellular
  • predominantly granulocyte precursors
  • myeloid:erythroid increased
75
Q

3 phases of CML

A
  • initial chronic stable phase (most diagnosed here)
  • accelerated phase (unresponsive to treatment)
  • blast phase (resembles AL)
76
Q

Main causes if mortality and morbidity in Ph negative neoplasms

A

Arterial and venous thrombosis

77
Q

Pathogenesis of Ph neg MPN

A
  • JAK2 mutation underlies pathogenesis

- results in unregulated prolif of granulocytes, erythroid series and megakaryocytes

78
Q

Clinical and lab findings in essential thrombocythemia

A
  • sustained increase in plt count due to megakaryocyte prolif
  • bone marrow shows increased numbers of enlarged mature megakaryocytes
  • other cell lines usually not increased
  • abnormal platelet function may lead to bleeding
79
Q

Clinical features of ET

A
  • red, warm, painful fingers and toes
  • thrombosis and haemorrhage
  • splenomegaly
  • very important to exclude reactive causes of thrombocytosis first
80
Q

Reactive causes of thrombocytosis

A
  • infection/inflammation
  • chronic haemorrhage
  • iron deficiency
  • post surgery
  • malignancy
81
Q

Prognosis and treatment of ET

A
  • indolent disorder (long symptoms free survival)
  • median survival with therapy is 20 years
  • principle of treatment is to control plt count
  • cutler ductile agents and anti platelet drugs
82
Q

Clinical and lab findings in polycythemia Vera

A
  • increased red cell production
  • prolif of granulocytes and megakaryocytes
  • JAK2 mutation inmost cases
83
Q

3 phases of PV

A
  • prodromal prepolycythamic phase (mild erthtrocytosis)
  • overt polycythemic phase
  • post polycythemic myelofibrosis phase
84
Q

Bone marrow in PV

A
  • megakaryocytes have hyper lobated nuclei
85
Q

Prognosis and treatment of PV

A
  • most die from thrombosis or haemorrhage
  • repeated venesection is mainstay
  • cytoreductuve agents to control counts
  • long term warfarin for cases with confirmed venous thrombosis
86
Q

Clinical and lab features of primary myelofibrosis

A
  • progressive generalized fibrosis of the bone marrow in association with extra medullary haematopoiesis in spleen and liver
  • leads to anaemia and massive heparosplenomegaly
87
Q

What causes the bone marrow fibrosis in PMF?

A

Cytokine release by abnormal megakaryocyte prolif

88
Q

Two phases of PMF

A
  • prefibrotic phase (hypercell marrow, large MK, minimal fibrosis)
  • fibrotic phase (increased reticulin, red cells with teardrops)
89
Q

Prognosis and treatment of PMF

A
  • varying survival
  • treatment is palliative (decrease splenomegaly and anaemia)
  • cytoreductuve agents (hydroxuyrea)
  • splenectomy/splenic radiation
  • allogeneic BM transplant in young patients
90
Q

Consequence of enhanced tyrosine kinase activity

A
  • increased proliferation
  • decreased apoptosis
  • decreased adherence to BM
91
Q

Acquired factors that can cause platelet inhibition

A
  • asprin

- uraemia

92
Q

Function of Von willebrands factor

A

Facilitates plt vessel wall interaction

93
Q

6 basic coag tests to perform

A
  • VWB
  • PTT
  • INR
  • FBG
  • bleeding time
  • plt count
94
Q

Function of low weight heparin

A
  • binds to anti thrombin
  • inhibits FXa and thrombin
  • little or no monitoring required
95
Q

Common causes of acquired thrombophilia

A
  • pregnancy
  • COC
  • obesity
  • immobility
  • surgery
  • malignancy
96
Q

How a genetic event leads to dysregulation of apoptosis and haematological malignancy

A
  • t(14:18) translocation
  • In follicular lymphoma
  • over expression of bcl2
  • reduces apoptosis
  • accumulation if malignant cells
97
Q

Investigations to confirm multiple myeloma

A
  • SPEP (monoclonal peak)
  • UBJ protein urea
  • bone marrow clonal plasma cell
98
Q

Causes of polycythemia

A

Primary

  • inherited
  • acquired (polycythemia Vera)

Secondary

  • relative (dehydration)
  • true
99
Q

Exogenous factors known to predispose to acute leukemia

A
  • viruses
  • radiation
  • chemicals
100
Q

Factors that determine the phenotype of leukemia

A
  • cell lineage
  • rate of proliferation
  • degree of differentiation
  • degree of apoptosis
101
Q

Molecular mechanisms whereby loss of function of oncogenes may occur

A
  • large deletions
  • small deletions leading to frame shift
  • mutations resulting in stop codons
  • mutations in promoter sequence of gene
  • mutations in splice regions of genes
102
Q

Mechanism of action of chemotherapeuric drugs

A
  • damage to mitotic spindle
  • bind to DNA and interfere with mitosis
  • deprive cells of asparganine
  • lysis of lymphoblasts
  • induce DNA strand break
103
Q

Allopurinol mechanism of action

A

Xanthine oxidase inhibitor

Prevents production of Uric acid in the purine breakdown pathway

104
Q

Properties of stem cells

A
  • have the capacity for self-renewal and differentiation

- capable of producing daughter cells which are either stem cells or more differentiated

March Exams (14 decks)

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