Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Haematology > Leukaemia > Flashcards

Leukaemia Flashcards

1
Q

what is acute leukaemia?

A

Uncontrolled proliferation of partially developed white blood cells, also called blast cells, which build up in the blood over a short period of time.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

what are the 2 consequences of the causative mutations in leukaemia?

A

causes precursor blood cells to lose ability to differentiate into mature blood cells
blast cells divide uncontrollably

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

what is the consequence of cells not maturing in leukaemia?

A

stuck as blast cells, don’t function properly

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

what are the consequence of uncontrollable cell division of blast cells?

A

Take a lot of space and nutrition in bone marrow
Other cells get crowded out
Causes cytopenia’s = Anaemia, Thrombocytopenia, Leukopenia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

what happens when blast cell numbers increase?

A

they spill into blood:
Lymphoblasts = settle in liver and spleen
Pre-t cell settle in Thymus and Lymphnodes – enlargement
Acute promyelocytic anaemia -
Promyelocytes activate the clotting process, combined with decreased platelets = DIC

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

what are the shared clinical features of acute leukaemia?

A
  • Fatgie – anemia
  • Bleeding – thrombocytopenia
  • Increased infections – leukopenia
  • Pain and tenderness in the bones – increased cell production
  • Abdominal fullness – hepatosplenomegaly
  • Pain in lymphnodes (lymphadenopathy)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

what are clinical features more indicative of ALL?

A

hepatosplenomegaly and lymphadenopathy more common
o Thymus enlargement in T-ALL – mass in mediastinum
o Also testicular
o Cranial nerve palsies

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

what are clinical features more indicative of AML?

A

monocytic variety causes swelling of guns

Violaceous skin lesions

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

what are the common investigations done for Acute leukaemia?

A 
FBC,
Blood film,
Coagulation screen
Bone Marrow Aspirate +/- Biopsy 
CXR and CT scan 
Lumbar puncture
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

what is the general management of acute leukaemia?

A

Chemo
Biological therapy
stem cell transplants
bone marrow transplants

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

what will FBC show in acute leukaemia?

A

decreased haemaglobin
increased wcc (tho blasts so not functioning)
decreased platelets

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

what will the blood film in AML show?

A

large cells, fine chromatin, auer rods (crystalised aggregates of the myeloperoxidase enzyme), myeloid blasts

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

what will the blood film in ALL show?

A

smaller cells, coarse chromatin, little cytoplasm (glycogen granules) – lymphoblasts

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

what will the morphology of a bone marrow aspirate show?

A

 ↓erythropoiesis; ↓megakaryocytes, i.e. platelet precursors; > 20% blast cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

what is the immunophenotyping of B lineage acute leukaemia?

A

HLA-DR+, TdT-, CD19+, CD10+, surface IgM+

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

what is the immunophenotyping of T lineage acute leukaemia?

A

TdT+, cytoplasmic CD3+, CD1a/2/3+, CD5+

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

what are the additional immunophenotyping findings of acute leukaemia?

A

Anti-MPO, CD13, CD33, CD45, CDxw65, CD117

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

what is the epidemiology of ALL?

A
  • Most common childhood cancer (Newborn – 14 yeras)

* Most common leukemia overall

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

what is the difference between acute and chronic leukaemia

A 
Acute = blast (non mature)
Chronic = cytic (slightly more mature)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

what can ALL be classified into?

A

o T cell ALL (proliferation of T cell precursors)
o Pre-B cell ALL (proliferation of B cell precursors)
o B-cell ALL

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

what are the histological features of lymphoblasts?

A

no granules, increased N/C ration, scanty cytoplasm, less prominent nucleolus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

what is the spread and consequence of ALL?

A

bone marrow - replaces other cells (pancytopenia)
enter peripheral blood - leukostasis
Metastasize through body - hepatosplenomegaly, lymphadeonpathy, testes enlarge, CNS, bone

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

what markers are commonly present in ALL?

A

TdT+

Periodic acid shiff (+ve)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

what are the common causes of ALL?

A

chromosomal translocations
abnormal chromosome number
genetic conditions
Radiation, smoking, viral infections, folate metabolim polymorphisms, chemotherapy, benzne, multiple myeloma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

what are the common chromosomal translocations associated with ALL and what does it cause?

A

12 and 21
22 and 9 (Philadelphia)
Production of abnormal intracellular proteins, affect cell function + division

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

what geentic conditions are associated with ALL?

A

trisomy 21, Klinefelters, Fanconi anameia, Blooms syndrom

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

what are the features, translocations and markers associated with Pre B-ALL?

A

o Child
o Trisomy
o CD10, CD19, CD20
o t(9:22), t(12:21

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

what are the features, translocations and markers associated with B cell ALL?

A

o t(8:!4), t(8:22), t(2:8) = burkitts lymphoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

what are the features, translocations and markers associated with T cell ALL?

A

o Adult mass in the anterior mediastinum
o CD3+, CD7+
o 14q11, 7q34

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

what are the clinical features associated with ALL?

A

acute, anaemia, infection, fever, malasie, sweats, haemorrhage, lecostasis, bone or joint pain, mediastinal involvement, CNS involvement, widespread lymphadeonpathy, hepatosplenamegaly, rhabdomyosarcoma, Ewings sarcoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

what are the signs of haemorrhage seen in ALL?

A

purpura, menorrhagia, epitaxiss, bleeding gum, retina, rectal

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

what are the signs of leukostasis in ALL?

A

hypoxia, retinal haemorraghe, confusion or diffuse pulmonary shadowing

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

what does mediastinal involvement in ALL cause?

A

SVC obstruction

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

what does CNS involvement in ALL cause?

A

cranial nerve palsy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

what investigations should be done in ALL?

A 
morphologic analysis
cytogenic studies
cell surface markers
molecular markers
cytochemical analysis
peripheral blood smear
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

what will the peripheral blood smear show in ALL?

A

Normocytic or macrocytic anaemia
Platelets <100,000
WBC <10,000 -> 100,000

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

which subsets of ALL can have targeted treatments?

A

o BCR-ABL+ALL
o Burkitts
o T-cell ALL
o CNH Leukaemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

what is the remission induction treatment of ALL?

A

vincritin, predinisolone, daunorubicin, aspraraginase

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

what is consolidation therapy of ALL treatment?

A

alternating cycles of induction agents and cytotoxic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

what CNS prophylaxis is used in ALL?

A

cranial irradiation plus IT chemotherapy (methotrexate +/- cytarabine, prednisolone)

41
Q

what is the maintenance therapy used in ALL?

A

daily 6-MP PO and weekly methotrexate PO

42
Q

what are the poor prognostic factors of ALL?

A
  • Age > 60
  • WBCs > 100,000
  • Mature B or early T cell types
  • Phildelphia chromosome (ALL bad, CML good)
43
Q

what is the epidemiology of AML?

A

more common in the elderly (>60s)

44
Q

what is AML?

A

the clonal expansion of myeloid blasts in the bone marrow, peripheral blood, or extramedullary tissues

45
Q

what is the spread of AML?

A
  • replace most of bone marrow cells crowding out of normal hamatopoeisis
  • enter the peripheral blood – leukostasis
  • metasiatisze throughout the body – liver, spleen, CNS, lymphnodes, bone, skin, gum infiltration (m5)
46
Q

what are myeloid cells?

A

granulocytes

47
Q

what are the causes of AML?

A

chromosomal translocations
myelodysplastic
antineoplastic agents, alkylating agents, ionising radiation, benzene
aplastic anaemia, myeloproliferative disorders, Fanconi anaemia, PNH

48
Q

what is myelodysplastic syndrome?

A

Defective maturation of myeloid cells
Build of blasts
initially <20% blast, but enough to cause decrease in function of red blood cells, granulocytes and platelets
as disease progresses the blast percentage may >20% resulting in AML with myelodysplasia

49
Q

what is the classification of AML based on?

A

the morphology of the myeloblast

50
Q

what are the different classifications of AML?

A 
AML without maturation
AML with minimal maturation
AML with maturation (M2)
Acute promyelocytic leukaemia (M3)
Acute myelomonocytic leukaemia
Acute monocytic leukaemia (M5)
Acute erythroid leukaemia 
Acute megakaryoblastic leukaemia
51
Q

what are the features of AML M2?

A

caused by t8:21

most common

52
Q

what are the features of acute promyelocytic leukaemia (M3)

A

Characterised by translocation of chromosome 15:17 which disrupts retinoic acid receptor gene which is required for normal cell division
Associated with DIC
Treats with Vit A – good prognosis

53
Q

what feature is associated with AML M5

A

gum infiltration

54
Q

what are the clinical features of AML?

A

acute, anaemia, infection, fever, malaise, sweats, haemorrhage (M3), gum hypertrophy and skin infiltration (M4/M5), leucostasis

55
Q

what are the diagnostic investigations for

A 
morphologic analysis
cytogenic studies
cell surface markers
molecular markers
cytochemical analysis
peripheral blood
56
Q

what will morphological analysis show in posistive AL?

A

> 20% blasts

57
Q

what chemical markers are present in AML?

A

TdT-, MPO+

58
Q

what will peripheral blood in AML show?

A

Normocytic or macrocytic anemia
Platekets <100,000
WBC <10,000 – 100,000
Auer rods – myeloperoxidase +

59
Q

what is the treatment of AML?

A

• 2-4 cycles of chemotherapy (5-10 days of chemotherapy followed by 2-4 weeks recovery)

60
Q

what are the poor prognostic factors of AML?

A
  • Age >60
  • WBCs >100,000
  • Poor performance status
  • Mutation of FLT3
  • 2ry AML
61
Q

what are common complications of treatment in AL?

A

Bone Marrow Suppression - anaemia, neutropenia, thrombocytopenia
Ohers - n+v, hair loss, liver, renal dysfunction, tumour lysis syndrome, infection, hyperviscosity sundrome
late effects - loss of fertility, cardiomyopathy

62
Q

AL - presence of auer rods in blood?

A

ALL - none

AML - always

63
Q

AL- presence of lymphoblast’s in blood

A

ALL - always present

AML - may or may not be present

64
Q

AL - bone and joint pain

A

ALL - more common

AML - less common

65
Q

AL - hepatosplenomegaly

A

ALL - more common

AML - less common

66
Q

AL - organ infiltration

A

ALL - more common

AML - quite unusual

67
Q

AL - Immunophenotyping

A

ALL - B Cell (CD19, cytoplasmic CD22, cytoplasmic CD79a, CD10), T cell (CD4, CD2, CD7)
AML - Anti-MPO, CD13, CD33, CD45, CDxw65, CD117

68
Q

what is the pathophysiology of chronic leukaemia?

A

Cells only mature partially
Too many premature cells = accumulate
Leukocytes accumulate in bone marrow and eventually spill out – blood and tissues Accumulated cells causes healthy cells to get crowded out

69
Q

What is the affect of cell dysfunction in each type of CL?

A

CML - divide too quickly

CLL - don’t die as they should

70
Q

most is the epidemiology of CLL?

A
  • Most common type in Western countries

* adults – increased risk with increasing age (>60s)

71
Q

what type of cells does CLL impact?

A

T and B cells

72
Q

what is the underlying pathophysiology of CLL?

A

• B cell interfere with pathways of B cell receptors, which should only be activated during infection – to activate specific tyrosine kinase
o Brutons tyrosine kinase which stops lymphoids developming developing
o Interaction with each other – causes them to die slowly

73
Q

what markers are expressed in CLL?

A

CD5, CD19, CD23

74
Q

what is the spread of leukocytes in CLL?

A

• Premature leukocytes build up in bone marrow, spill in blood and move to lymphatic system – primarily lymph nodes – lymphadenopathy then lymphomas
o Richter transformation – small lymphomas collect into masses

75
Q

What are the consequences of cell spread in CLL?

A

Autoimmune haemolytic anaemia
Hypogammaglobulinemia
Cytopenia

76
Q

what are the clinical features of CLL?

A

asymptomatic
lymphadenopathy
lymphocytosis “smudge cells”
marrow failure – anaemia, neutropenia and thrombocytopenia
recurrent infection
weight loss, night sweats, malaise
massive hepatosplenamegaly
autoimmune haemolytiv anameia – DAT, warm type
10% develop diffuse large cell lymphoma = aggressice (fever, increased LDH and LD size)

77
Q

what investigations can be done in CLL?

A 
blood count
blood film
immunophenotyping
immunoglobulins 
cytogenics
78
Q

what will a blood count in CLL show?

A

lymphocytosis, platelets normal or low

79
Q

what will a blood film in CLL show?

A

increased lymphhocytes, smear cells

80
Q

what will immunophenotyping in CLL show?

A

o CD5, CD19 and CD23 +

o CD2 and FMC7 -ve

81
Q

what will immunoglobulins in CLL show?

A

hypogammaglobulinemia

82
Q

what is the cytogenic of CLL?

A

13q –, 11q –, 12q +, 17p –, 6q –, +12

83
Q

what staging system is used for CLL?

A

The RAI system:
o Stage 0 lymphocytosis (low)
o Stage 1 lymphocytosis and adenopathy (mid)
o Stage 2 lymphocystosis and hepatosplenomegaly (mid)
o Stage 3 anemia (high)
o Stage 4 thrombocytopenia (high)

84
Q

what are the indications for treatment in CLL?

A

o weight loss of more than 10% over 6 months
o extreme fatigue
o fever related to leukemia for longer than 2 weeks
o night sweats for longer than 1 month
o progressive marrow failure (anemia or thrombocytopenia)
o autoimmune anemia or thrombocytopenia not responding to glucocorticoids
o progressive or symptomatic splenomegaly
o massive or symptomatic lymphadenopathy
o progressive lymphocytosis, as defined by an increase of greater than 50% in 2 months or a doubling time of less than 6 months

85
Q

What is the 1st line treatment for CLL?

A

Chlorambucil +/- prednisolone

86
Q

what is the epidemiology of CML?

A

elderly

87
Q

what are the causes of CML?

A

Translocation that effects granulocytes = Philadelphia chromosome (t(9;22) = 22
Radiation
Chronic myeloproliferative – CML, p.vera, ET, MMM

88
Q

what cell does CML affect?

A

myeloid cells:
granulocytes and their precursors
other lineages (platelets)

89
Q

how does the Philadelphia chromosome cause CML?

A

• Philadelphia chromosome produces BCR gene sites next to ABL = BCR-ABL gene
• Produces BCR-ABL protein
o Activates tyrosine kinases (causes abnormal phosphorylation = signalling) – on/off switch always on
o Switch on cell division – myeloid cells divide more quickly
 causes buildup in bone marrow, spill into blood

90
Q

what is natural progression of CML?

A

o chronic phase – increased WCC <10% of blast cells in blood
o aggressive/accelerated phase – more likely to find blast cells in the blood – 10-10%, more systemic symptoms
o blast phase/crisis – blood contains >20% blast cells
 increased mutatuons as cells divide more quickly

91
Q

what are the clinical features of CML?

A
  • presents in chronic phase
  • many asymptomatic
  • fatigue, lethargy, weight loss, sweats
  • hepatospenomegaly – feeling of abdominal fullness (due to build up of cells)
  • gout, brusing/bleeding, splenic infarction, priapism
  • retinal haemorraghe, dyspnoea and cough
92
Q

what are the clinical features of a blast crisis?

A

rapid increase in blast cells, fever, bone pain, fatigue, increased severity of anemia and thrombocytopnia, splenomegaly, large clusters of blasts in bone amrrow

93
Q

what investigations can be used in CML?

A 
FBC
blood film
NAP score decreased, ESR reduced
BM biopsy
cytogenic analysis
FiSH
94
Q

what is the management of CML?

A

Imatinib
Interferon Alpha
Stem Cell Transplant
SOKAL - prognosis

95
Q

how does Imatinib work in CML management?

A

o tyrosine kinase inhibitor that prevents the action of the BCR-ABL fusion protein, i.e.this is the abnormal protein produced by the Ph mutation.

96
Q

what is the diagnostic feature of blast cells in CML?

A

<10%

97
Q

what will the blood film and FBC show in CML?

A

increased WBC
o normal/↓Hb
o leucocytosis with neutrophilia and myeloid precursors (myelocytes – of all stages), eosinophilia, basophilia
o thrombocytosis
o Rapid turnover – raised uric acid and LDH

98
Q

what will cytogenic analysis in CML show?

A

t9:22

99
Q

what will FiSH anaylsis in CML show?

A

BCR-ABL

Haematology (13 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions