Haematology Flashcards
1
Q
define anaemia
A
low Hb
2
Q
TAILS: causes of microcytic anaemia?
A
- Thalassaemia
- Anaemia of chronic disease
- Iron def
- Lead poisoning
- Sideroblastic
3
Q
3As and 2Hs: causes of normocytic anaemia?
A
- Acute blood loss
- Anaemia of chronic disease
- Aplastic anaemia
- Haemolytic anaemia
- Hypothyroidism
4
Q
how can macrocytic anaemia be classified?
A
- megaloblastic
- normoblastic
5
Q
causes of megaloblastic anaemia?
A
- B12 def
- folate def
6
Q
causes of normoblastic, macrocytic anaemia?
A
- alcohol
- reticulocytosis
- hypothyroidism
- liver disease
- azathioprine therapy
7
Q
symptoms of anaemia?
A
- tiredness
- SOB
- headaches
- dizziness
- palpitations
- worsening of other disease
8
Q
which conditions may worsen with anaemia?
A
- angina
- heart failure
- PVD
9
Q
which 3 symptoms of anaemia are specific to iron deficiency anaemia?
A
- pica
- hair loss
- restless leg syndrome
10
Q
signs O/E of anaemia?
A
- pale skin
- conjunctival pallor
- nail signs
- tachycardia
- raised RR
11
Q
which signs O/E are specific to iron deficiency anaemia?
A
- koilonychia (spoon nails)
- angular chelitis
- atrophic glossitis
- brittle hair and nails
12
Q
which type of anaemia is jaundice specific to?
A
haemolytic anaemia
13
Q
which type of anaemia do bone deformities indicate?
A
thalassaemia
14
Q
findings O/E of anaemia due to CKD?
A
- oedema
- HTN
- excoriations on the skin
15
Q
bloods done to investigate for anaemia?
A
- Hb
- MCV
- B12 and folate
- ferritin
- blood film
16
Q
non-blood test investigations done in anaemia?
A
- oesophageal-gastroduodenoscopy (OGD)
- bone marrow biopsy
17
Q
causes of iron deficiency?
A
- insufficient dietary uptake
- increased requirements (e.g. pregnancy)
- iron being lost from a slow bleed
- inadequate absorption (e.g. coeliac)
18
Q
where in the gut does iron get absorbed? what is the soluble form of iron?
A
- duodenum and jejunum
- ferrous (Fe 2+)
19
Q
how is iron converted from the insoluble Fe3+ to the soluble Fe2+?
A
using stomach acid
20
Q
how can PPIs cause an iron deficiency?
A
- they reduce gastric acid secretion
- stops iron being converted to the soluble form of Fe 2+
- therefore not absorbed
21
Q
most common cause of iron deficiency in adults not menstruating?
A
blood loss in the GI tract
22
Q
most common cause of iron deficiency in children?
A
dietary insufficiency
23
Q
how can transferrin saturation be calculated?
A
serum iron / TIBC
24
Q
what can cause a raised ferritin?
A
infection
25
how are TIBC and transferrin affected by iron deficiency?
both increase
26
how are TIBC and transferrin affected by iron overload?
both decrease
27
what could cause iron studies to give an iron overload impression?
- iron supplements
| - acute liver damage
28
3 management options for iron deficiency anaemia?
1. blood transfusion
2. iron infusion
3. PO ferrous sulfate
29
how should Hb rise with oral iron supplements?
10g/L per week
30
what are the 2 causes of B12 deficiency?
- insufficient dietary uptake
| - pernicious anaemia
31
what is needed to absorb B12? where is this secreted?
- intrinsic factor
| - parietal cells of the stomach
32
where in the gut does B12 get absorbed?
terminal ileum
33
neuro signs of B12 deficiency?
- peripheral neuropathy, incl numbness / paraesthesia
- loss of vibration / proprioception
- visual changes
- mood / cognitive changes
34
pathophysiology of pernicious anaemia?
- antibodies have formed against parietal cells / intrinsic factor
- stops B12 being absorbed in the terminal ileum
35
how is pernicious anaemia diagnosed?
test for the following antibodies:
- intrinsic factor antibody (1st line)
- gastric parietal cell antibody
36
management of insufficient dietary B12?
PO cyanocobalamin supplements
37
management of pernicious anaemia? hint: can't be oral because won't be absorbed
IM hydroxycobalamin
38
why is it important to treat a B12 def before a folate def (in pts with both)?
giving folic acid to pt with B12 def can cause subacute combined degeneration of the spinal cord
39
how can causes of haemolytic anaemia be classified?
inherited vs acquired
40
inherited forms of haemolytic anaemia?
- hereditary spherocytosis
- thalassaemia
- sickle cell anaemia
- G6PD deficiency
41
acquired forms of haemolytic anaemia?
- autoimmune haemolytic anaemia, incl. haemolytic disease of the newborn
- paroxysmal nocturnal haemoglobinuria
- microangiopathic haemolytic anaemia
- prosthetic valve-related haemolysis
42
signs O/E of haemolytic anaemia? (hint: all due to RBC destruction)
- anaemia
- splenomegaly
- jaundice
43
investigations for haemolytic anaemia?
- FBC (normocytic)
- blood film
- direct Coombs test
44
what is seen on the blood film in haemolytic anaemia?
schistocytes (fragments of RBCs)
45
when does the direct Coombs test give a positive result?
autoimmune haemolytic anaemia
46
mode of inheritance of hereditary spherocytosis?
autosomal dominant
47
presentation of hereditary spherocytosis?
- jaundice
- gallstones
- splenomegaly
- aplastic crisis
48
which organism can bring on an aplastic crisis in hereditary spherocytosis patients?
parvovirus
49
how is hereditary spherocytosis diagnosed?
- FHx
- clinical features
- blood film
- FBC
- reticulocyte count (raised)
50
findings on blood film in hereditary spherocytosis?
spherocytes
51
finding on FBC in hereditary spherocytosis? hint: it's a rogue one
MCHC is raised
52
treatment of hereditary spherocytosis?
- folate supplements
| - splenectomy
53
key difference between hereditary spherocytosis and hereditary elliptocytosis?
- in the second one, RBCs are ellipse shaped
| - otherwise identical
54
mode of inheritance for G6PD deficiency?
- X linked recessive
55
G6PD deficiency is more common in patients of which descent?
Mediterranean / African
56
what can trigger a G6PD deficiency crisis?
- infection
- drugs
- fava beans (broad beans)
57
drugs which cause G6PD deficiency crisis?
- primaquine
- ciprofloxacin
- sulfonylurea
- sulfasalazine
58
how is G6PD deficiency diagnosed?
G6PD enzyme assay
59
signs O/E of G6PD deficiency crisis?
- jaundice (usually in neonates)
- gallstones
- anaemia
- splenomegaly
60
what is seen on the blood film of someone in G6PD deficiency crisis?
Heinz bodies
61
how can autoimmune haemolytic anaemia (AIHA) be classified?
- warm type: haemolysis at normal or higher temperatures
| - cold type: haemolysis at colder temps
62
causes of warm AIHA?
idiopathic
63
causes of cold AIHA?
- lymphoma
- leukaemia (e.g. CLL)
- SLE
- infections (EBV, CMV, HIV)
64
management of AIHA?
- blood transfusions
- prednisolone
- rituximab
- splenectomy
65
what is alloimmune haemolytic anaemia? give 2 types
- immune reaction destroying foreign RBCs in circulation
- transfusion reaction
- haemolytic disease of the newborn
66
what are the chains making up normal adult Hb?
2 alpha + 2 beta globin chains
67
pathophysiology of thalassaemia?
- genetic defect causing defective alpha / beta globin chain production
- spleen then recognises RBCs as damaged and breaks them all down
68
potential signs and symptoms of thalassaemia?
- anaemia (microcytic) and assoc signs (fatigue, pallor)
- jaundice
- gallstones
- splenomegaly
- poor growth and development
- pronounced forehead and cheekbones
69
investigations in thalassaemia?
- FBC (microcytic anaemia)
- Hb electrophoresis
- DNA testing
- screened for in pregnancy booking appt
70
how can thalassaemia (and treatment) result in iron overload?
- increased Fe absorption in response to anaemia
| - recurrent blood transfusion therapy
71
what needs to be monitored in thalassaemia patients? why?
- serum ferritin
| - to check for iron overload
72
presentation of iron overload in thalassaemia? hint: similar to haemachromatosis
- fatigue
- liver cirrhosis
- infertility / impotence
- heart failure
- arthritis
- DM
- osteoporosis, joint pain
73
where is the affected gene in alpha thalassaemia?
chromosome 16
74
where is the affected gene in beta thalassaemia?
chromosome 11
75
how is iron overload in thalassaemia managed?
- limit blood transfusions
| - iron chelation
76
management of alpha thalassaemia?
- monitor FBC
- blood transfusions
- splenectomy
- BM transplant (sometimes curative)
77
what are the types of beta thalassaemia?
- thalassaemia minor
- " " intermedia
- " " major
78
presentation of thalassaemia minor?
- mild microcytic anaemia
| - only monitoring needed, no treatment
79
presentation of thalassaemia minor? management?
- mild microcytic anaemia
| - only monitoring needed, no treatment
80
presentation of thalassaemia major?
- severe microcytic anaemia
- splenomegaly
- bone deformities
81
pathophysiology of sickle cell anaemia?
- abnormal beta globin chain gene on chromosome 11
- this causes HbS to form instead of HbA
- HbS causes RBCs to be sickle-shaped
82
what is the relation between sickle cell trait and malaria?
having sickle cell trait reduces the severity of malaria infection
83
how is sickle cell disease tested for in newborns?
part of the day 5 heel-prick test (Guthrie)
84
complications of sickle cell anaemia?
- increased risk of infection
- sickle cell crisis
- avascular necrosis of large joints (e.g. hip)
- pulmonary HTN
- CKD
- priapism in men
- acute chest syndrome
85
general management principles of sickle cell anaemia?
- avoid crisis triggers (e.g. stay well hydrated)
- ABx prophylaxis (penicillin V)
- hydroxycarbamide to stimulate HbF production
- blood transfusion for severe anaemia
- bone marrow transplant can be curative
86
potential triggers of a sickle cell crisis?
- infection
- cold
- dehydration
- significant life events
87
management of a sickle cell crisis?
all supportive:
- low threshold for admission
- treat underlying cause (e.g. ABx, keep warm, hydrated)
- paracetamol / ibuprofen
- penile aspiration if priapism
88
pathophysiology of a vaso-occlusive crisis?
- sickle-shaped RBCs clog up capillaries
| - causes distal ischaemia
89
presentation of vaso-occlusive crisis?
- pain
- fever
- symptoms of underlying infection
- dehydration
- raised haematocrit
- priapism in men
90
pathophysiology of splenic sequestration crisis? how does it present?
- sickle-shaped RBCs blocking blood flow within the spleen
- blood pools within the spleen
- causes severe anaemia, may progress to shock
91
management of splenic sequestration crisis?
- emergency
- blood transfusions
- fluid resus
92
what is an aplastic crisis in sickle cell disease?
temporary cessation of erythropoiesis
93
typical trigger of an aplastic crisis?
parvovirus B19 infection
94
management of aplastic crisis?
- blood transfusions if needed
| - otherwise self-resolving within a week
95
how is acute chest syndrome of sickle cell disease diagnosed?
both must be present:
- fever / resp symptoms
- new infiltrates seen on CXR
96
infective causes of acute chest syndrome?
- bronchiolitis
| - pneumonia
97
non-infective causes of acute chest syndrome?
- pulmonary vaso-occlusion
| - fat emboli
98
management of acute chest syndrome?
- emergency
- ABx / antivirals for infections
- blood transfusions for anaemia
- incentive spirometry (encourages deep breathing)
- NIV / intubation are last line
99
age groups typically affected by ALL?
<5 years and >45 years
100
age group typically affected by CLL?
>55 years
101
age group typically affected by CML?
>65 years
102
age group typically affected by AML?
>75 years
103
presentation of leukaemia? hint: pancytopenia
- non-specific
- fatigue
- fever
- FTT if child
- pallor (anaemia)
- petechiae, abnormal bruising (thrombocytopenia)
- abnormal bleeding
- lymphadenopathy
- hepatosplenomegaly
104
differentials for a non-blanching rash?
- leukaemia
- meningococcal septicaemia
- vasculitis
- HSP (lower limbs, buttocks)
- ITP
- NAI (children / vulnerable adults)
105
initial investigation for leukaemia?
urgent FBC (<48h) for all with suspected leukaemia
106
investigations for leukaemia?
- FBC
- blood film
- lactate dehydrogenase (LDH, raised)
- bone marrow biopsy
- CXR
- lymph node biopsy
- LP
- CT, MRI, PET (for staging)
107
what might the blood film show in leukaemia?
- abnormal cells
| - inclusions
108
where is a bone marrow biopsy usually taken from?
iliac crest
109
which cell type over-proliferates in ALL and CLL?
usually B-lymphocytes
110
which genetic condition is ALL associated with?
downs syndrome
111
blood film findings in ALL?
blast cells
112
characteristic chromosomal change seen in CML in adults?
philadelphia chromosome
113
what type of anaemia could CLL give?
warm autoimmune haemolytic anaemia
114
which other cancer could CLL transform into?
- high-grade lymphoma
| - called richter's transformation
115
blood films findings in CLL?
"smudge" / "smear" cells (WBCs which ruptured in the process of preparing the film)
116
3 phases of CML?
- chronic phase
- accelerate phase
- blast phase
117
3 phases of CML?
- chronic phase (asymptomatic, 5 years)
- accelerate phase
- blast phase
118
what is seen in the blast cells on a blood film in AML?
auer rods
119
which cancer could a myeloproliferative disorder (e.g. PRV) transform into?
AML
120
management of leukaemia?
- chemotherapy
- steroids
- radiotherapy
- bone marrow transplant
- surgery
121
complications of chemotherapy for leukaemia?
- failure
- stunted growth in children
- neurotoxicity
- infertility
- cardiotoxicity
- secondary malignancy
- tumour lysis syndrome
122
pathophysiology of tumour lysis syndrome?
- uric acid released from cells destroyed by chemotherapy
- form crystals in kidneys
- causes AKI
123
management of tumour lysis syndrome?
allopurinol and rasburicase to reduce uric acid levels
124
how can lymphoma be categorised?
- hodgkin's
| - non-hodgkin's (all the other types)
125
peak ages affected by hodgkin's lymphoma? hint: bimodal
aged 20 and then aged 75
126
risk factors for hodgkin's lymphoma?
- HIV
- EBV
- autoimmune (RA, sarcoidosis)
- FHx
127
presentation of hodgkin's lymphoma?
- lymphadenopathy
- B symptoms
- fatigue
- itching
- cough
- SOB
- abdo pain
- recurrent infections
128
what are B symptoms? list them
- systemic symptoms of lymphoma
- fever
- weight loss
- night sweats
129
investigations in hodgkin's lymphoma
- lymph node biopsy is diagnostic
- LDH (raised)
- CT, MRI, PET to stage
130
lymph node biopsy findings in hodgkin's lymphoma?
reed-sternberg cells (large B cells with multiple nuclei)
131
what is ann arbor staging used for? what does it take into account?
- scoring system used to stage all types of lymphoma
- considers whether lymph nodes involved are in same region or not
- considers whether they are on one side of diaphragm (or on both)
132
management of hodgkin's lymphoma?
- chemotherapy
| - radiotherapy
133
prognosis of hodgkin's lymphoma?
- chemo and radiotherapy are curative
- they both carry own risk of secondary malignancy
- B = BAD (B-symptoms have worse prognosis)
134
types of non-hodgkin's lymphoma?
- burkitt lymphoma
- MALT lymphoma
- diffuse large B cell lymphoma
135
key association with MALT lymphoma?
H. pylori infection
136
which other conditions are associated with burkitt lymphoma? hint: all infections
- HIV
- EBV
- malaria
137
risk factors for non-hodgkin's lymphoma?
- HIV
- EBV
- H. pylori (MALT)
- hep B / C
- exposure to pesticides
- FHx
138
management of non-hodgkin's lymphoma?
- watchful waiting
- chemotherapy
- rituximab
- radiotherapy
- stem cell transplant
139
pathophysiology of myeloma? what is multiple myeloma?
- genetic mutation causes rapid proliferation of plasma cells (B cells which produce antibodies) in bone marrow
- myeloma affecting multiple parts of the body
140
skin sign of pernicious anaemia?
gives "lemon tinged" skin
141
most common immunoglobulin to be raised in myeloma?
IgG
142
urinalysis finding in myeloma?
bence jones protein
143
which bones are most commonly affected in myeloma?
- skull
- spine
- long bones
- ribs
144
effects of myeloma on bone turnover? result of this?
- increases osteoclast activity (reabsorb bone Ca into blood)
- pathological fractures
- hypercalcaemia
145
how does myeloma cause renal impairment? hint: multifactorial
- lots of Igs block tubular flow
- high Ca
- dehydration
- bisphosphonate therapy
... all cause impairment
146
how is plasma viscosity affected in myeloma?
- increases
| - due to increased Igs
147
complications relating to increased plasma viscosity in myeloma?
- easy bruising and bleeding
- visual loss
- purple extremities
- heart failure
148
what type of anaemia arises from myeloma?
normocytic, normochromic
149
how does myeloma cause anaemia?
bone marrow being resorbed
150
presentation of myeloma?
- high Ca
- Renal failure
- Anaemia
- Bone pain / lesions
- plus complications of plasma viscosity
151
risk factors for myeloma?
- age >65
- Black
- male
- FHx
- obesity
152
what must be considered in anyone over 60 with bone pain? which investigation is key?
- myeloma
| - urgent FBC required
153
findings on bloods for myeloma?
- pancytopenia
- high Ca
- raised ESR
- raised plasma viscosity
154
BLIPE: initial investigations for multiple myeloma?
- Bence jones protein (urine electrophoresis)
rest are all in blood:
- Light-chain assay
- Immunoglobulins
- Protein Electrophoresis
155
diagnostic investigation for multiple myeloma?
bone marrow biopsy
156
pereferred investigation to stage multiple myeloma?
whole body MRI
157
signs on X-ray in multiple myeloma?
- punched out lesions
- lytic lesions
- "rain-drop skull" appearance
158
management of myeloma?
- bortezomide
- thalidomide
- dexamethasone
- stem cell transplant
- VTE prophylaxis
159
management of bone disease secondary to myeloma?
- bisphosphonates
- radiotherapy
- ortho surgery
- cement augmentation
160
complications associated with myeloma?
- infection
- peripheral neuropathy
- spinal cord compression
161
what are the 3 types of myeloproliferative disorder?
- primary myelofibrosis
- polycythaemia vera
- essential thrombocythaemia
162
gene mutations associated with myeloproliferative disorders?
- JAK2
- MPL
- CALR
163
presentation of myeloproliferative disorders?
- initially asymptomatic
- B symptoms
- anaemia
- abdo pain (splenomegaly)
- portal HTN
- bleeding, petechiae (low platelets)
- red face (raised RBCs)
- infections (low WCC)
164
feautres of polycythaemia vera?
- plethoric conjunctiva and face
- "ruddy" complexion
- splenomegaly
- pruritis, worse after hot bath
- HTN
165
findings on FBC in polycythaemia vera?
isolated rise in Hb
166
findings on FBC in essential thrombocythaemia?
raised platelets
167
findings on FBC in myelofibrosis?
more variable:
- anaemia (low Hb)
- high OR low WCC
- high OR low platelets
168
findings on blood film in myelofibrosis?
- teardrop-shaped RBCs
- varying in size (poikilocytosis)
- blast cells
169
diagnostic investigation and finding for myeloproliferative disorders?
- bone marrow biopsy
| - comes back "dry"
170
management of myelofibrosis?
- if mild, none needed
- allogeneic stem cell transplant could be curative
- chemotherapy
- supportive (for anaemia / splenomegaly / portal HTN)
171
management of polycythaemia vera?
- venesection
- aspirin
- chemotherapy
172
management of essential thrombocythaemia?
- aspirin
| - chemotherapy
173
findings on FBC in myelodysplastic syndrome?
- anaemia
- neutropenia (low neutrophils)
- thrombocytopenia (low platelets)
174
risk factors for myelodysplastic syndrome?
- age >60
- chemotherapy
- radiotherapy
175
which malignancy could myelodysplastic syndrome transform into?
AML
176
presentation of myelodysplastic syndrome?
- could be asymptomatic / found incidentally
- fatigue, pallor SOB
- recurrent infections
- purpura, bleeding
177
how can a diagnosis of myelodysplastic syndrome be confirmed?
on bone marrow aspiration and biopsy
178
management of myelodysplastic syndrome?
- watchful waiting
- blood transfusions if severely anaemic
- chemo
- stem cell transplant
179
causes of thrombocytopenia relating to reduced platelet production?
- sepsis
- B12 / folate deficiency
- liver failure
- leukaemia
- myelodysplastic syndrome
180
causes of thrombocytopenia relating to increased platelet destruction?
- drugs
- alcohol
- ITP
- TTP
- haemolytic uraemic syndrome
181
drugs which can cause thrombocytopenia?
- heparin
- valproate
- methotrexate
- isotretinoin
- antihistamines
- PPIs
182
presentation of thrombocytopenia?
- easy bruising
- prolonged bleeding
- epistaxis
- menorrhagia
- bleeding gums
- blood in urine / stools
183
differentials for prolonged bleeding?
- thrombocytopenia
- haemophilia A or B
- vWD
- DIC
184
pathophysiology of ITP?
antibodies made against platelets
185
management of ITP?
- prednisolone
- IV Igs
- rituximab
- splenectomy
