Haematology Flashcards
1
Q
What is a reticulocyte count?
A
not part of routine FBC, requested specifically
number of ‘young’ red blood cells, measures rate of red blood cell production
2
Q
Describe the classification of anaemias.
A
MICROCYTIC:
iron deficiency
thalassaemias
chronic disease
NORMOCYTIC:
chronic disease
renal disease
acute bleeding
MACROCYTIC:
folate deficiency
b12 deficiency
haemolytic
bone marrow disorders
3
Q
Why does folate deficiency cause macrocytic red blood cells?
A
folate is needed for DNA synthesis
if there is a deficiency then the RBC cannot divide and continues to mature and get larger
4
Q
How much iron should the average person eat in a day?
A
15mg
5
Q
Where is iron absorbed?
A
duodenum and jejunum
6
Q
What is the name for the process of blood cell production?
A
haematopoesis
7
Q
Describe briefly the differentiation tree in haemotopoesis.
A
haematopoietic stem cells differentiates to either a MYELOID PROGENITOR or a LYMPHOID PROGENITOR
Myeloid progenitor can differentiate into:
- megakaryocyte
- erythrocyte
- mast cell
- myeloblast
Lymphoid progenitor can differentiate into a NATURAL KILLER CELL or SMALL LYMPHOCYTE
The small lymphocyte can become a T cell or B cell
B cells can further differentiate into plasma cells
8
Q
What commonly causes neutrophilia?
A
acute bacterial infections
9
Q
What commonly causes neutropenia?
A
myeloma, lymphoma
10
Q
What is a common cause of lymphocytosis?
A
chronic infection (hepatitis, EBV)
malignancy
stress
11
Q
What commonly causes lymphocytopenia?
A
AIDS/ autoimmune condition
steroids
chemotherapy
12
Q
What does a raised eoisinophil count suggest?
A
parasitic infection
13
Q
What does elevated monocytes suggest?
A
myelodysplastic syndrome
14
Q
What is koilonychia?
A
spoon shaped nails
sign of iron deficiency
15
Q
What is angular stomatis?
A
irritated, cracked sores at the corners of the mouth
commonly caused by iron or B12 deficiency
16
Q
What does jaundice imply in a haematological sense?
A
haemolytic anaemia
17
Q
What is classed as microcytic anaemia?
A
mean corpuscular volume (MCV) < 80
18
Q
What are the causes of microcytic anaemia?
A
TAILS
T- thalassaemia
A- anaemia of chronic disease
I- iron deficiency
L- lead poisoning
S- sideroblastic anaemia
19
Q
Why does iron deficiency cause anaemia?
A
iron is required to produce haemoglobin
so an iron deficiency causes a haemoglobin deficiency
20
Q
What causes iron deficiency anaemia?
A
- reduced intake (diet)
- reduced absorption (malabsoption conditions, PPIs/ tetracyclines)
- increased requirements, e.g. pregnancy
- blood loss
21
Q
How does iron deficiency anaemia present (other than the common universal signs of anaemia)?
A
tired, cold, palpitations
koilonychia
brittle hair
tachycardia
angular stomatis
22
Q
How is iron deficiency anaemia treated?
A
ferrous sulfate
23
Q
What causes a triad of dysphagia, iron deficiency and oesophageal webs?
A
Plummer vinson syndrome
24
Q
What does it mean if an elderly person presents with iron deficiency anaemia?
A
it’s a cause for concern as most common cause in the elderly is bleeding due to a colon cancer
25
What are the causes of anaemia of chronic disease?
chronic infection
chronic inflammation
neoplasia
26
How does anaemia of chronic disease show on a blood test?
FBC: low Hb, normal/ low MCV, HIGH ESR
iron studies: normal/ raised ferritin, low serum iron, low transferrin saturation
27
What is thalassaemia?
a mutation in the alpha or beta units of Hb resulting in reduced production of Hb
28
What is the most common type of thalassaemia?
beta thalassaemia
29
What inheritance pattern does thalassaemia follow?
autosomal recessive
30
How is thalassaemia diagnosed?
Hb electrophoresis: will show abnormal Hb
blood film: microcytic, hypochromic cells
31
How is thalassaemia treated?
- regular blood transfusions
- venesection
- splenectomy
32
Why is splenectomy a treatment for thalassaemia?
decreases the consumption of RBCs and reduce transfusion complications
33
What is the main possible complication of thalassaemia?
organ failure
34
What inheritance pattern does sickle cell anaemia follow?
autosomal recessive
35
What is sideroblastic anaemia?
when the iron levels in the body are normal, but the body cannot insert iron into haemoglobin
36
How does sideroblastic anaemia show up in investigations?
increased iron, transferrin, ferritin
ringed 'sideroblasts' on blood film
37
What is the MCV for normocytic anaemia?
MCV 80-100
38
What are the common causes of normocytic anaemia?
acute blood loss
haemolysis (leading to haemolytic anaemia)
haematological malignancy
pregnancy
CKD
39
How does the presentation of haemolytic anaemia differ from other types of anaemia?
present with jaundice and dark urine
40
How will haemolytic anaemia show on investigations?
raised reticulocytes (in chronic cases)
raised bilirubin
raised urobilinogen
schistocytes on blood film
41
What are the causes of haemolytic anaemia?
autoimmune destruction
sepsis
sickle cell anaemia
thalassaemia
42
What is pernicious anaemia?
lack of intrinsic factor which is required for B12 absorption in the terminal ileum
43
What cells produce intrinsic factor?
parietal cells
44
What are the causes of B12 deficiency anaemia?
pernicious anaemia
malabsorption
decreased dietary intake
chronic nitrous oxide use
45
How does B12 deficiency anaemia present and show up on investigations?
bloods: raised MCV, low Hb, low B12
'megaloblastic' anaemia on blood smear- oval shaped RBCs, hyperhsegmented neutrophils
S+S: general anaemia presentations plus neurological symptoms
46
What can happen in severe B12 deficiency?
demyelination of the spinal cord
47
What is it called when B12 deficiency anaemia cause neurological symptoms?
subacute combined degeneration
48
What are the causes of macrocytic anaemia aside from b12 deficiency?
- diseases of the liver and spleen
- haem malignancy
- chronic alcohol consumption
49
How can you tell the difference between macrocytic anaemia caused by disease of the liver/ spleen and alcohol induced anaemia?
anaemia caused by liver/ spleen disease will show 'codocytes' on the blood film whereas alcohol-induced will not
50
What are the potential causes of thrombocytosis?
infection/ inflammation
tissue injury
splenectomy
51
What is leukaemia (most simple definition)?
cancer of the bone marrow
52
How is leukaemia categorised?
either myeloid or lymphoid
myeloid:
acute myeloid
chronic myeloid
lymphoid:
acute lymphoblastic
chronic lymphoblastic
53
Describe the basic pathophysiology of leukaemia.
- immature blast cells uncontrollably proliferate
- this takes up space in the bone marrow and infiltrates into tissues
- lack of space within the bone marrow means fewer healthy cells can mature and be released into the blood
54
What is the most common leukaemia in young children?
acute lymphoblastic leukaemia
55
What causes ALL pathophysiologically?
proliferation of immature lymphoblasts
56
What is the prognosis for ALL?
high cure rates
5 year survival:
90% in children
30-40% in adults
57
What are the risk factors for ALL?
- Down's syndrome
- neurofibromatosis
- radiation
58
What is neurofibromatosis?
genetic condition that causes tumours to grow on nerves
59
How does ALL present?
- general anaemia symptoms
- bleeding/ bruising
- infections
- hepatosplenomagaly
- lymphadenopathy
- swollen testicles
60
What does headaches suggest in a patient with ALL?
that the leukaemia has infiltrated the central nervous system
may go on to develop central nerve palsies
61
How is ALL diagnosed?
- >20% lymphocytes on blood film
- FBC: anaemia, thrombocytosis, neutropenia
- CXR/ CT to investigate lymphadenopathy
62
How is ALL managed?
- blood and platelet transfusions
- chemotherapy - methotrexate
- steroids
- stem cell/ bone marrow transplant \
- antibiotics
63
What is CLL?
chronic lymphoblastic leukaemia
proliferation o B lymphocytes + failure of cell apoptosis
64
What age group is mostly affected by CLL?
60+
65
What us the most common type of leukaemia in adults?
chronic lymphoblastic
66
What is the prognosis for CLL?
85% 5-year survival
67
How does CLL present?
often asymptomatic !!
- lymphadenopathy
- night sweats/ weight loss
- bone marrow failure
68
How is CLL diagnosed?
*SMUDGE CELLS ON BLOOD FILM*
FBC: anaemia, thrombocytopenia
69
How is CLL managed?
watch and wait in early stages
chemotherapy (rituximab)
stem cell/ bone marrow transplant
70
What is a possible complication of CLL?
RICHTER TRANSFORMATION
sudden transformation of CLL/ SLL into a significantly more aggressive form of large cell lymphoma
commonly presents as sudden and dramatic increase in size of lymph nodes
71
Which type of leukaemia has the worst prognosis?
acute myeloid leukaemia
only 15% 5-year survival
72
What is AML?
acute myeloid leukaemia
proliferation of immature myeloblasts
73
What age group is most commonly affected by AML?
elderly
74
How does AML present?
general anaemia symptoms
bleeding/ bruising
infections
hepatosplenomegaly
GUM HYPERTROPHY
75
How is AML diagnosed?
AUER RODS ON BONE MARROW BIOPSY
FBC: anaemia, thrombocytopenia
76
How is AML managed?
blood and platelet transfusions
chemotherapy
stem cell/ bone marrow transplant
antibiotics
77
What is CML?
chronic myeloid leukaemia
proliferation of myeloid blood cells (overproduction of myeloid progenitor)
78
What age group is most affected by CML?
adults over 40
79
What is the genetics of CML?
vast majority of cases are associated withe the PHILADELPHIA CHROMOSOME
80
How does CML present?
GOUT
general anaemia symptoms
bleeding/ bruising
infections
hepatosplenomegaly
weight loss and night sweats
81
How is CML diagnosed?
- genetic testing for BCR ABL/ Philadelphia chromosome
FBC: anaemia, thrombocytopenia, leukocytosis
82
Describe the association between BCR ABL gene and the 'Philadelphia chromosome'.
- A piece of chromosome 9 and a piece of chromosome 22 break off and trade places
- The BCR-ABL gene is formed on chromosome 22 where the piece of chromosome 9 attaches
- The changed chromosome 22 is called the Philadelphia chromosome.
83
How is CML managed?
chemo
stem cell/ bone marrow transplant
tyrosine kinase inhibitors (imatinib)
84
What are the best factors to differentiate between the 4 types of leukaemia?
ALL- children < 6 years old, may see swollen testicles and CNS palsies
CLL- smudge cells on blood film
AML- auger rods on bone marrow biopsy, may see gum hypertrophy
CML- Philadelphia chromosome, may see gout
85
Describe the features of Hodgkin's and Non Hodgkin's lymphoma.
both H and NH includes proliferation of lymphocytes in the lymph nodes and are associated with EBV + immunosuppression
however, Hodgkins has a bimodal incidence distribution (e.g. peaks in early 20s and again in 70s) but non-hodgkins predominantly affects adults over the age of 40
86
Describe the presentation of Hodgkin's and non Hodgkins lymphoma.
Get B symptoms with both (fever, night sweats, weight loss)
lymphadenopathy with both however it's painless in non-hodgkins and painful upon drinking alcohol in Hodgkins
non-hodgkins may also present with hepatosplenomegaly
87
How is Hodgkin's lymphoma diagnosed?
- raised ESR
- CT/CXR for staging
- lymph node biopsy REED STERNBERG CELLS
88
How is non-hodgkin's lymphoma diagnosed?
- imaging CXR/ CT for staging
- NO REED STERNBERG CELLS ON THE LYMPH NODE BIOPSY
89
What is the easiest way to differentiate between Hodgkins and non-hodgkins lymphoma?
Reed Sternberg Cells only present on lymph node biopsy in HODGKINS, never in non-hodgkins
90
How is lymphoma managed?
H and NH both possibly treated with radiotherapy and chemotherapy
for Hodgkins- chemo is ABVD therapy
non-hodgkins- chemo is RCHOP therapy
Hodgkin's patients may also take steroids and have a stem cell/ bone marrow transplant
91
What is ABVD chemotherapy?
doxorubicin
bleomycin
vinblastine
dacarbazine
92
What is RCHOP chemotherapy?
rituximab
cyclophospamide
hydroxy-daunorubicin
vincristine
prednisolone
93
How is lymphoma staged?
ANN ARBOR STAGING
stage 1: the disease is in ONE AREA ONLY
stage 2: the disease is in TWO OR MORE areas on the SAME SIDE of the diaphragm
stage 3: the disease is in TWO OR MORE areas on BOTH SIDES of the diaphragm
stage 4: the disease has spread BEYOND the lymph nodes
94
What is the prognosis for multiple myeloma?
55% 5-year survival
95
What is multiple myeloma?
plasma cell cancer in the bone marrow
96
What condition is linked to myeloma?
close link to a condition called monoclonal gammopathy of undetermined significance (MGUS) which is when there is too much of an immunoglobulin released by abnormal plasma cells
1% of cases turn into myeloma
97
How does myeloma present?
CRAB
C- hyperCalcaemia
R- renal impairment
A- anaemia (fatigue, dizziness, etc)
B- bone lesions (bone pain)
98
How is multiple myeloma diagnosed?
- FBC: anaemia
- ESR: raised
- Blood film: ROULEUX FORMATION
- Serum and urine electrophoresis: Bence Jones protein in urine
- bone marrow biopsy
- x-ray/ CT: bone lesions
99
How is multiple myeloma managed?
- chemo (VCD, VTD, MTP)
- stem cell transplant
- analgesics
- bisphosphonates (zoledronic acid)
- blood transfusions
100
What is polycythaemia?
high concentration on erythrocytes in the blood
101
How is polycythaemia categorised?
absolute:
- primary (AKA polycythaemia vera)
- secondary
relative
102
What is relative polycythaemia?
when there is a normal number of erythrocytes but a reduction in plasma
103
What causes reactive polycythaemia?
obesity
dehydration
excessive alcohol consumption
104
What is polycythaemia vera?
primary absolute polycythaemia
myeloproliferative neoplasm
increased number of erythrocytes due to an abnormality in the bone marrow
105
What is secondary polycythaemia?
when a disease outside of the bone marrow causes over-stimulation of the bone marrow causing increased number of erythrocytes
106
What causes secondary polycythaemia?
COPD
sleep apnoea
PKD
renal artery stenosis
kidney cancer
107
What is the most common cause of polycythaemia vera?
JAK2 mutation
108
What occurs in polycythaemia vera other than increased number of erythrocytes?
may also produce excessive amounts of platelets and white blood cells
109
How does polycythaemia present?
headaches
dizziness
fatigue
blurred vision
red skin (hands, face, feet)
hypertension
itching (after contact with warm water)
hepatosplenomegaly
110
How is polycythaemia vera diagnosed?
FBC: raised: Hb, haematocrit, white cell count, platelet count
genetic testing for jak2 mutation
serum erythropoietin decreased
111
How is polycythaemia managed?
venesection
daily low dose aspirin
hydroxycarbamide (for those at high risk of thrombus)
112
What is venesection?
quickest way to remove RBCs by removing approx. 1 pint of blood at a time
113
What is the most common cause of B12 deficiency?
pernicious anaemia
114
When would you see Howell-jolly bodies on a blood film?
patient who has had a recent splenectomy or has hyposplenism
115
What are the risks of vitamin B3 deficiency?
the 3 d's
dementia
dermatitis
diarrhoea
116
Where are RBCs broken down?
spleen
117
What is the concern when somebody presents with infection symptoms within 14 days of having chemotherapy? What is the treatment?
neutropenic sepsis
take blood cultures and administer IV piperacillin with tazobactam while waiting results
