haem oncology Flashcards
1
Q
types of myeloid cells
A
red cells
granulocytes/monocytes
platelets
2
Q
types of lymphoid cells
A
B cells
T cells
natural killer cells
antigen presenting cells
3
Q
which disease is defined by no bone marrow maturation
A
acute myeloid leukaemia
4
Q
which disease is defined by dysfunctional bone marrow maturation
A
myelodysplasia
5
Q
which disease is defined by overproduction of red cells
A
polycythaemia rubra vera
6
Q
which disease is defined by overproduction of granulocytes
A
chronic myeloid leukaemia
7
Q
which disease is defined by overproduction of platelets
A
essential thrombocytosis
8
Q
which disease is defined by overproduction of megakaryocytes
A
primary myelofibrosis
9
Q
link between myelodysplasia and acute myeloid leukaemia
A
- myelodysplasia = dysfunctional bone marrow maturation
- AML = NO bone marrow maturation
- 25% of myelodysplasia progresses to AML
10
Q
prognosis of ALL vs CLL
A
ALL = rapidly progressive and survival only months without tx, however curable with chemo CLL = slow progression and may never require tx, however not curable with chemo (managed not cured)
11
Q
classification of lymphoid malignancies
A
- high grade e.g. ALL
- low grade e.g. CLL
12
Q
4 types of leukaemia and common ages affected
A
- ALL = <5yo, >45yo
- CLL = >55yo
- CML = >65yo
- AML = >75yo
13
Q
general sx of leukaemia
A
- fatigue
- fever
- failure to thrive in children
- pallor (anaemia)
- petechiae and abnormal bruising (thrombocytopaenia)
- abnormal bleeding
- lymphadenopathy
- hepatosplenomegaly
14
Q
differentials for petechiae
A
- leukaemia
- meningococcal septicaemia
- vasculitis
- HSP
- ITP
- non-accidental injury
15
Q
ix in suspected leukaemia
A
bloods: - FBC - LDH - blood film imaging: - CXR - CT, MRI, PET special tests: - bone marrow biopsy - lymph node biopsy - LP
16
Q
3 types of bone marrow investigation
A
- bone marrow aspiration
- bone marrow trephine (solid core sample, better assessment of cells)
- bone marrow biopsy (iliac crest, under LA)
17
Q
pathophysiology of ALL
A
acute proliferation of B lymphocytes
+ pancytopaenia (all other cell types are suppressed)
18
Q
condition associated with ALL
A
downs syndrome
19
Q
blood film finding in ALL
A
blast cells
20
Q
genetics of ALL
A
associated with philadelphia chromosome (t(9:22) translocation)
21
Q
pathophysiology of CLL
A
chronic proliferation of B lymphocytes
22
Q
presentation of CLL
A
often asymptomatic
infections, anaemia, bleeding, weight loss
23
Q
anaemia in CLL
A
warm autoimmune haemolytic anaemia
24
Q
prognosis of CLL
A
can transform to high-grade lymphoma (Richter’s transformation)
25
blood film findings in CLL
smear or smudge cells
26
disease course in CML
1. chronic phase -> can last ~5 years, often dx incidentally with raised WCC
2. accelerated phase -> more symptomatic with anaemia, thrombocytopaenia and immunocompromise
3. blast phase -> highest proportion of blast cells leading to severe sx and pancytopaenia (fatal)
27
genetics of CML
associated with philadelphia chromosome (t(9:22) translocation)
28
aetiology of AML
can be the result of a transformation from a myeloproliferative disorder such as PRV or myelofibrosis
29
blood film findings in AML
high proportion of blast cells
| can have rods inside cytoplasm known as Auer rods
30
general mx in leukaemia
- chemotherapy and steroids
- radiotherapy
- bone marrow transplant
- surgery
31
complications of chemotherapy
- failure
- stunted growth and development in children
- infections
- neurotoxicity
- infertility
- secondary malignancy
- cardiotoxicity
- tumour lysis syndrome
32
what is tumour lysis syndrome
- caused by release of uric acid from cells being destroyed by chemotherapy
- uric acid forms crystals in interstitial tissue and tubules of kidneys causing AKI
- can also release other chemicals such as potassium and phosphate
33
mx of tumour lysis syndrome
reduce uric acid levels with allopurinol or rasburicase
34
complication of tumour lysis syndrome
- high uric acid levels
- high phosphate levels (-> low calcium levels)
- high potassium levels
35
definition of lymphoma
cancers affecting the lymphocytes in the lymphatic system
| proliferation within lymph nodes leading to lymphadenopathy
36
epidemiology of Hodgkin's lymphoma
~20% of all lymphomas
| peaks at age 20 and age 75
37
risk factors for Hodgkin's lymphoma
- HIV
- EBV
- autoimmune conditions: RA, sarcoidosis
- FHx
38
main presentation of Hodgkin's lymphoma
lymphadenopathy (neck, axilla, groin) which is non-tender and rubbery
may get pain in lymph nodes when drinking alcohol
fever, weight loss, night sweats
39
general symptoms of lymphoma
```
fatigue
itching
cough
SOB
abdo pain
recurrent infections
```
40
ix in Hodgkin's lymphoma
```
bloods:
- LDH
imaging:
- CT, MRI, PET
special tests:
- lymph node biopsy
```
41
findings on lymph node biopsy in Hodgkin's lymphoma
Reed-Sternberg cells
-> abnormally large B cells with multiple nuclei with nucleoli inside them
appear like the face of an owl with large eyes
42
what staging system is used for lymphoma
Ann Arbor
43
Ann Arbor staging
```
1 = confined to one region of lymph nodes
2 = >1 region but same side of diaphragm
3 = lymph nodes affected above and below diaphragm
4 = widespread involvement with non-lymphatic organs (lungs, liver)
```
44
mx of Hodgkin's lymphoma
chemotherapy + radiotherapy with aim to cure
45
side effects of radiotherapy
risk of cancer
damage to tissues
hypothyroidism
46
3 types of non-Hodgkin lymphoma and associations
1. burkitt lymphoma (EBV, HIV, malaria)
2. MALT lymphoma (H. pylori)
3. diffuse large b cell lymphoma
47
typical presentation of diffuse large b cell lymphoma
rapidly growing painless mass in >65yo
48
risk factors for non-Hodgkin lymphoma
- HIV
- EBV
- H. pylori
- hepatitis B or C
- exposure to pesticides (trichloroethylene)
- FHx
49
mx options in non-Hodgkin lymphoma
- watchful waiting
- chemotherapy
- mab's e.g. rituximab
- radiotherapy
- stem cell transplant
50
definition of myeloma
cancer of plasma cells (antibody producing b cells)
51
what is MGUS
monoclonal gammopathy of undetermined severity
excess of single type of antibody/ab components without other features of myeloma or cancer
often found incidentally, ?risk of progression to myeloma
52
what is smouldering myeloma
progression of MGUS with higher levels of antibodies/ab components
pre-malignant, more likely to progress to myeloma
53
most common antibody affected in multiple myeloma
IgG (~50% cases)
54
urine findings in myeloma
Bence Jones protein
light chain component from monoclonal paraprotein (single type of antibody produced by all identical cancerous plasma cells)
55
impact of myeloma on bone marrow
cancerous plasma cells invade bone marrow (bone marrow infiltration) leading to anaemia, neutropaenia and thrombocytopaenia
56
what is myeloma bone disease
cytokines released from plasma cells leads to increased osteoclast activity and suppressed osteoblast activity
=> excess resorption of bone
57
common sites of myeloma bone disease
skull
spine
long bones
ribs
58
appearance of myeloma bone disease
patches of thin bone = osteolytic lesions
| 'pepper pot' or 'rain drop' skull
59
complications of myeloma bone disease
- pathological fractures
| - excessive resorption of bone leads to hypercalcaemia
60
what are plasmacytomas
individual tumours made from cancerous plasma cells as a result of myeloma
61
what is myeloma renal disease
renal impairment in myeloma due to:
- high antibody levels blocking flow through tubules
- hypercalcaemia
- dehydration
- meds used to treat condition e.g. bisphosphonates
62
main 4 features of myeloma and mnemonic
CRAB
- calcium (elevated)
- renal failure
- anaemia (normocytic and normochromic)
- bone lesions/pain
63
risk factors for myeloma
- older age
- male
- Black African ethnicity
- FHx
- obesity
64
when to suspect myeloma
>60yo with persistent bone pain (back pain++) or unexplained fracture
65
initial bloods in suspected myeloma
- FBC
- calcium
- ESR
- PV
66
ix in myeloma and mnemonic
BLIP
- urine Bence jones protein
- serum free Light chains
- serum Immunoglobulins
- serum Protein electrophoresis
67
imaging in myeloma
assess for bone lesions - preference:
1. whole body MRI
2. whole body CT
3. skeletal survey (XR)
68
mx of myeloma
- combination of chemotherapy with antineoplastic agent, thalidomide, dexamethasone
- stem cell transplant
- VTE prophylaxis while on chemo
69
mx of myeloma bone disease
- bisphosphonates
- radiotherapy
- orthopaedic surgery e.g. prophylactic IM rod or tx#
- cement augmentation (injecting cement into vertebral# or lesions)
70
complications of myeloma/tx
- infection
- pain
- renal failure
- anaemia
- hypercalcaemia
- peripheral neuropathy
- spinal cord compression
- hyperviscosity
71
definition of myeloproliferative disorders
uncontrolled proliferation of a single type of stem cell
| considered bone marrow cancers
72
3 myeloproliferative disorders
1. primary myelofibrosis
2. polycythaemia rubra vera
3. essential thrombocythaemia
73
what is primary myelofibrosis
uncontrolled proliferation of haematopoietic stem cells
74
what is polycythaemia rubra vera
uncontrolled proliferation of erythroid cell line
75
what is essential thrombocythaemia
uncontrolled proliferation of megakaryocytic cell line
76
prognosis of myeloproliferative disorders
have potential to progress to AML
77
main gene association with myeloproliferative disorders
JAK2
78
what is myelofibrosis
where uncontrolled proliferation (due to myeloproliferative disorders) leads to fibrosis of the bone marrow
79
pathophysiology of myelofibrosis
proliferating cells release cytokines (important cytokine = fibroblast growth factor) which lead to fibrosis of the bone marrow. fibrosis affects production of blood cells leading to anaemia and leucopaenia
80
what is extramedullary haematopoiesis and when does it occur
production of blood cells (haematopoiesis) occurring outside of the bone marrow (e.g. in liver and spleen) due to myelofibrosis (fibrosis of the bone marrow)
causes hepatosplenomegaly
81
presentation of myeloproliferative disorders
- may be asymptomatic
- systemic sx: FLAWS
- anaemia
- splenomegaly
- portal hypertension
- low platelets (bleeding, petechiae)
- thrombosis
- red face (raised RBCs)
- infections (low WCC)
82
4 key signs on examination of polycythaemia
1. conjunctival and facial plethora (excessive redness to conjunctiva)
2. 'ruddy' complexion
3. splenomegaly
4. erythromelalgia (burning pain in hands)
83
blood test findings in myelofibrosis
- anaemia
- leucocytosis or leucopaenia
- thrombocytopaenia or thrombocytosis
84
3 blood film findings in myelofibrosis
1. teardrop-shaped RBCs
2. varying sized RBCs (poikilocytosis)
3. immature RBC and WC (blasts)
85
when does myelofibrosis occur
in primary myelofibrosis or secondary to PV or essential thrombocythaemia
86
how to diagnose myelofibrosis
- bone marrow biopsy
- bone marrow aspiration -> 'dry' as bone marrow has turned to scar tissue
- genetic testing for JAK2 gene mutation
87
mx of primary myelofibrosis
- mild disease = monitoring
- allogenic stem cell transplant
- chemotherapy
- supportive mx of anaemia, splenomegaly and portal hypertension
88
mx of polycythaemia
- regular venesection to keep Hb in range
- aspirin to reduce risk of thrombosis
- chemotherapy
89
mx of essential thrombocythaemai
- aspirin to reduce risk of thrombosis
| - chemotherapy
90
what is myelodysplastic syndrome
myeloid bone marrow cells don't mature properly and don't produce healthy blood cells
91
epidemiology of myelodysplastic syndrome
- more common in >60yo
| - patients previously treated with chemo or radiotherapy
92
prognosis of myelodysplastic syndrome
risk of transforming to AML
93
presentation of myelodysplastic syndrome
- asymptomatic and incidentally diagnosed on FBC
- sx of anaemia
- frequent/severe infection (neutropaenia)
- purpura, bleeding (thrombocytopaenia)
94
diagnosis of myelodysplastic syndrome
FBC -> anaemia, thrombocytopaenia, neutropaenia
blood film -> blasts
bone marrow aspiration and biopsy
95
mx options for myelodysplastic syndrome
- watchful waiting
- supportive tx with blood transfusion
- chemotherapy
- stem cell transplant
