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MS3 - Haematology > Haemato-oncology > Flashcards

Haemato-oncology Flashcards

1
Q

2 types of Acute leukaemia?

A
  1. acute myeloid leukaemia (AML)

2. acute lymphoblastic leukaemia (ALL)

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2
Q

How leukaemia leads to anaemia/immune suppression?

A

Uncontrolled expansion of hypofunctional cells in blood, bone marrow and other organs leads to suppression of normal haematopoiesis and immunity

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3
Q

Sx of leukaemia

A
  • anaemia,
  • bleeding
  • susceptibility to infection
  • hepatosplenomegaly
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4
Q

Endogenous RFs of leukaemia:

A
  • Chromosome fragility syndromes

- Downs syndrome

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5
Q

Exogenous RFs of leukaemia

A
  • Radiation
  • Chemotherapy
  • Benzene
  • Viral infection
  • Acquired immune deficiency
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6
Q

Epi of Acute Myeloblastic Leukaemia (AML)

A

Commoner in adults, Incidence 1:10,000 annually, Increased frequency with age (median >70 yr)

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7
Q

Bloods of of Acute Myeloblastic Leukaemia (AML)

A
  • low Hb and platelet count
  • WBC usually 20 – 100 x 109/L with blast cells visible on the peripheral blood film.
  • WBC >50 = high risk
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8
Q

Bone marrow results of Acute Myeloblastic Leukaemia (AML)

A
  • blasts > 20% of nucleated cells
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9
Q

Flow cytometry results of Acute Myeloblastic Leukaemia (AML)

A
  • CD13+, CD33+ helpful to confirm AML.
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10
Q

Good prognostics genetics of Acute Myeloblastic Leukaemia (AML)

A

t(15;17),
t(8;21),
inv16 .

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11
Q

Poor prognostics genetics of Acute Myeloblastic Leukaemia (AML)

A

Monosomy 7,

Abnormalities on chromosome 5, 1

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12
Q

Mx of Acute Myeloblastic Leukaemia (AML)

A
  1. combination therapy

2. supportive care

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13
Q

Combination therapy for AML?

A
  • cytosine arabinoside
  • daunorubicin.
  • Retinoic acid/arsenic trioxide
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14
Q

Duration of combination therapy for AML?

A

3-4 cycles of therapy given 4 – 6 weekly.

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15
Q

Complications of AML treatment?

A
  • Infection
  • Bleeding
  • Debility, profound weight loss
  • Social: inpatient for most of 6 months, loss of employment, stress on family
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16
Q

Success rate of AML treatment?

A
  • Overall complete remission rate: 85%

- Overall survival: 45-50% cured with chemotherapy alone

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17
Q

Acute Lymphoblastic Leukaemia (ALL) incident age?

A

peak age 2 – 10 years. Another peak >60 years

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18
Q

how to differentiate ALL from AML?

A

ALL commonly causes severe bone pain, sweats

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19
Q

Flow cytometry results for ALL?

A

may show blasts of B cell (CD10+, CD19+) or T cell lineage (20%)

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20
Q

Philadephila chromosome?

A

Philadelphia chromosome t(9;22) seen in 20-25 % of adults with ALL
- Poor prognostic marker but means specific therapy with tyrosine kinase inhibitors.

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21
Q

Poor prognostics genes of ALL

A
  • t(9;22)
  • t(4;11),
  • t(8;14)
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22
Q

Management of ALL?

A

Chemotherapy with complex combinations of cytotoxic drugs (eg steroids, vincristine, daunorubicin, asparaginase)

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23
Q

Course of chemotherapy for ALL?

A

a. Remission induction 2mo
b. Consolidation: 4 mo intense chemo
c. Maintenance: 2 yrs (less intense therapy; mainly oral cytotoxic drugs)

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24
Q

Success rate of ALL treatment?

A

> 85% cure in childhood ALL

>50% cure rate in adults who have intensive treatment on national trials

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25
Minimum residual disease use?
used to decide treatment in children and adults after response after 2 cycles of therapy
26
Minimum residual disease method? | sensitivity?
- Leukaemic clone can be identifed by DNA fingerprinting and accurately measured using Q PCR. - Sensitive (1 in 10,000 cells or better)
27
How does bone marrow transplant work? Effect on chemo?
Donor T cells kill leuakaemia via a graft versus leukaemia effect - Allows use of higher doses of chemo
28
Immediate complications of bone transplant
* Relapse * Graft versus host disease * Extramedullary toxicity * Infection * Rejection
29
Long term complications of bone transplant
infertility, | second cancers
30
Neutropenic sepsis cause?
results from the treatment of leukaemia and bone marrow transplantation
31
Sx of Neutropenic sepsis
• T >38C | or patient looks unwell
32
Mx of neutropenic sepsis?
culture and start broad spectrum antibiotics within 1 hour
33
Symptoms of myeloma?
``` CRABI” acronym C- Hypercalcaemia R- Renal dysfunction A- Anaemia B- Bone – lytic lesions, fractures, osteoporosis I- Infection ```
34
Chronic Lymphocytic Leukaemia (CLL) def?
- Clonal B cell lymphocytosis of >5 x 109/l persisting for more than 3 months
35
Chronic Lymphocytic Leukaemia (CLL) peak age?
median age at diagnosis 72 years
36
Chronic Lymphocytic Leukaemia (CLL) diagnosi?
Immunophenotyping: Looking at antigens on WBC - If score >3, CLL. - If <3, likely other form of B cell malignancies (eg mantle cell lymphoma)
37
Management of Chronic Lymphocytic Leukaemia (CLL)?
1. chemo | 2. BTK inhibition
38
BTK inhibitors?
- brutons tyrosine kinase inhibitor - ibrutinib - Tyrosine kinase: switches on cellular cycle
39
Genetic prognostics of CLL?
17p depletion on cytogenetics (mutation), poor diagnosis
40
Complications of CLL?
- Infection - Auto-immune haemolytic anaemia - Secondary malignancy
41
Causes of raised WCC
1. infection 2. proliferative 3. inflammation 4. steroids
42
Chronic myeloid leukaemia (CML) sx?
- Asymptomatic or - Hypermetabolic state (Weight loss, Sweats) - Leucostasis (due to high WBC: eg SOB, Priapism) - Symptoms from splenomegaly (eg Early satiety)
43
Genetics of Chronic myeloid leukaemia (CML)
- t(9,22); phaladelphia chromosome
44
BCR-ABL gene?
``` - philadelphia chromosome: Formation of Bcr-Abl - Inhibition of apoptosis - Up-regulates cell cycle progression and differentiation ```
45
Management of Chronic myeloid leukaemia (CML)?
Imatinib blocks formation of Bcr-Abl, improving the symptoms
46
Monitoring of Chronic myeloid leukaemia (CML)?
monitor Bcr-Abl level
47
Prognosis of Chronic myeloid leukaemia (CML)?
good nearly normal if treated
48
Raised haemoglobin causes?
1. EPO production 2. COPD 3. congenital heart disease in adults
49
Causes of raised platelet count?
1. splenectomy 2. inflammation 3. iron deficiency 4. malignancy
50
The Myeloproliferative neoplasms types?
I. Polycythaemia rubra vera II. Essential Thrombocythaemia III. Primary Myelofibrosis
51
Genetics Myeloproliferative neoplasms?
JAK2 mutation
52
JAK2 mutation significance?
- wild type only causes proliferation when EPO binds Mutation: doesnt need epo binding, causes proliferation without it
53
Management of Polycythaemia Vera
1. Venesection to maintain the Hct to <0.45 2. Aspirin 75 mg/d unless contraindicated 3. Cytoreduction should be considered
54
Myelofibrosis Def?
Symptomatic anaemia
55
Sx of Myelofibrosis
- Discomfort from splenomegaly - Hypermetabolic symptoms (weight loss, sweats)
56
Pathophysiology of Myelofibrosis
bone marrow becomes thickened (due to fibrin) so spleen starts producing RBC, and if spleen cant liver takes over
57
Prognosis of Myelofibrosis
depends on the score, but in high risk survival 1/2 years
58
Management of Myelofibrosis
ruxolotinib: JAK2 inhibitor
59
Non hodgkin lymphoma (NHL) presentation
- Enlarged Lymph nodes - B sx - Fatigue ◦ Itching
60
B sx?
B Symptoms ◦ Drenching Night sweats ◦ Unexplained Fevers >38’C ◦ Weight loss >10% in last 6 months
61
Diagnosis of Non hodgkin lymphoma (NHL)
Lymph node biopsy
62
Staging of Non hodgkin lymphoma (NHL)
Ann arbor Staging scan: - CT Neck,Chest,Abdo,Pelvis or PET scan) - Bone marrow biopsy
63
Ann arbor staging of NHL?
Stages 1-4 and tells you how many places you have lymphoma in: 1. one place 2. more than one on the same side of diaphragm 3. both sides of diaphragm 4. exta lymphatic ( eg lungs) A (absence of B sx) or B (presence of B sx)
64
Mx of Non hodgkin lymphoma (NHL)
Stage IA RCHOP x4 + Local Radiotherapy - Stage IIA – IV RCHOP x 6-8cycles Given every 3 weeks Risk of neutropenic sepsis day 7-10
65
RCHOP chemotherapy
- Rituximab (monoclonal antibody) - Cyclophosphamide - Hydroxydaunorubicin chemotherapy - Oncovin - Prednisolone
66
Types of low grade lymphoma
◦ Follicular Lymphoma ◦ Marginal Zone Lymphoma ◦ Mantle Cell Lymphoma ◦ Small lymphocytic Lymphoma
67
Hodgkin lymphoma mx?
Treat with | - ABVD +/- Radiotherapy
68
hodgkin lymphoma RFs?
EBV FH HIV
69
hodgkin lymphoma prognosis
>85% 5 year survival*
70
Myeloma def
Cancer of plasma cells with increased clonal plasma cells
71
Bone marrow finding in myeloma
monoclonal paraprotein
72
Monoclonal gammapathy of underterimined significance (MGUS) def?
- have paraprotein of no significance
73
Monoclonal gammapathy of underterimined significance (MGUS) Ix?
- Serum monoclonal protein <30g/L - Clonal bone marrow plasma cells <10% - Absence of end-organ damage
74
Monoclonal gammapathy of underterimined significance (MGUS) Mx?
Active monitoring
75
smouldering myeloma
- Serum monoclonal protein (IgG or IgA) ≥30g/L - or urinary monoclonal protein ≥500mg per 24h - and/or clonal bone marrow plasma cells 10-60% - Absence of myeloma defining events or amyloidosis
76
General Mx of myeloma
I. Vigorously rehydrate with at least 3 litres of normal saline daily II. Stop nephrotoxic drugs e.g. NSAIDs
77
Main complications of myeloma?
1. high calcium 2. hyper viscosity syndrome 3. spinal cord compression
78
Hyper viscosity syndrome presentation?
◦ blurred vision ◦ headaches ◦ mucosal bleeding ◦ dyspnoea due to heart failure
79
Hyper viscosity syndrome mx?
- plasma exchange | - isovolaemic venesection (if cant plasma exchange)
80
Spinal cord compression mangament?
- Dexamethasone 40 mg daily for 4 days - Urgent MRI whole spine - Neurosurgical/spinal surgical review if bony lesion to decompress or stabilise spine - Local radiotherapy if non-bony lesions ASAP (within 24 hours)
81
Myeloma medical mx?
1. Traditional Chemo eg Cyclophosphamide, 2. Dexamethasone 3. Immune modulatory drugs e.g. thalidomide, 4. Proteosome inhibitors (cause cell death) ◦ e.g. bortezomib

MS3 - Haematology (5 decks)

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