content Flashcards
1
Q
5 causes of microcytic anaemia
A
- thalassaemia
- anaemia of chronic disease
- iron deficiency
- lead poisoning
- sideroblastic anaemia
2
Q
5 causes of normocytic anaemia
A
- acute blood loss
- anaemia of chronic disease
- aplastic anaemia
- haemolytic anaemia
- hypothyroidism
3
Q
2 types of macrocytic anaemia
A
megaloblastic = result of impaired DNA synthesis preventing normal division normoblastic = no problem with DNA synthesis
4
Q
2 causes of megaloblastic macrocytic anaemia
A
- B12 deficiency
2. folate deficiency
5
Q
5 causes of normoblastic (non-megaloblastic) macrocytic anaemia
A
- alcohol
- reticulocytosis
- hypothryoidism
- liver disease
- drugs e.g. azathioprine
6
Q
symptoms of anaemia
A
- tiredness
- SOB
- headaches
- dizziness
- palpitations
- worsening of other conditions e.g. angina, HF, PVD
7
Q
symptoms specific to iron deficiency anaemia
A
- pica => dietary cravings for abnormal things such as dirt
- hair loss
8
Q
signs of general anaemia
A
- pale skin
- conjunctival pallor
- tachycardia
- tachypnoea
9
Q
signs specific to iron deficiency anaemia
A
- koilonychia (spoon shaped nails)
- angular chelitis/stomatitis
- atrophic glossitis
- brittle hair and nails
10
Q
sign specific to haemolytic anaemia
A
jaundice
11
Q
sign specific to thalassaemia
A
bone deformities
12
Q
3 ix in anaemia
A
- bloods:
- FBC: Hb, MCV
- haematinics: B12, folate, ferritin
- blood film - OGD and colonoscopy under urgent GI cancer referral in unexplained IDA
- bone marrow biopsy
13
Q
4 ways in which you can become iron deficient
A
- insufficient dietary intake
- increase requirements e.g. pregnancy
- loss of iron e.g. bleeding
- inadequate absorption e.g. coeliac, crohn’s
14
Q
where is iron absorbed and what is important for the process
A
duodenum and jejunum
stomach acid ensures iron in soluble form (Fe2+ rather than Fe3+) therefore meds reducing stomach acid can interfere with iron absorption
15
Q
formula for transferrin saturation
A
serum iron / total iron binding capacity (directly related to transferrin levels)
16
Q
what is transferrin
A
carrier protein that iron travels around the blood bound to
directly related to total iron binding capacity
17
Q
level of TIBC in iron deficiency anaemia
A
increased in IDA
-> low iron levels therefore more room left on ferritin for iron to bind
18
Q
mx options in iron deficiency anaemia
A
- blood transfusion
- iron infusion (monofer)
- oral iron (ferrous sulphate 200mg TDS)
19
Q
aim for rise in haemoglobin when correcting IDA
A
10g/litre per week
20
Q
2 causes of b12 deficiency
A
insufficient intake
pernicious anaemia
21
Q
pathophysiology of pernicious anaemia
A
autoimmune condition where antibodies against parietal cells or intrinsic factor prevent absorption of vitamin b12 in the ileum
22
Q
presentation of b12 deficiency
A
- peripheral neuropathy
- loss of vibration sense or proprioception
- visual changes
- mood or cognitive changes
23
Q
mx of pernicious anaemia
A
IM hydroxycobalamin (can’t use oral replacement due to problem being absorption)
24
Q
regime for IM hydroxycobalamin tx in pernicious anaemia
A
1mg 3x weekly for 2 weeks then every 3 months
or if neuro sx: 1mg every other day until improvement in sx
25
tx of vitamin b12 deficiency due to diet
oral cyanocobalamin
26
important consideration in joint b12 and folate deficiency
treat b12 deficiency first or risk subacute combined degeneration of the cord
27
5 causes of inherited haemolytic anaemia
1. hereditary spherocytosis
2. hereditary elliptocytosis
3. thalassaemia
4. sickle cell
5. g6pd deficiency
28
5 causes of acquired haemolytic anaemia
1. autoimmune haemolytic anaemia
2. alloimmune haemolytic anaemia (transfusion reaction or haemolytic disease of the newborn)
3. paroxysmal nocturnal haemoglobinuria
4. microangiopathic haemolytic anaemia
5. prosthetic valve related haemolysis
29
3 key features of haemolytic anaemia
1. anaemia
2. splenomegaly
3. jaundice
30
3 ix in haemolytic anaemia
1. FBC -> normocytic anaemia
2. blood film -> schistocytes
3. direct coombs test -> +ve in autoimmune haemolytic anaemia
31
genetics of hereditary spherocytosis
autosomal dominant
32
presentation of hereditary spherocytosis
- jaundice
- gallstones
- splenomegaly
- aplastic crisis (in presence of parvovirus)
33
what is haemolytic anaemia
low haemoglobin due to abnormal breakdown of RBC (can be caused by various pathologies)
34
what is hereditary spherocytosis
most common inherited haemolytic anaemia in northern europe
| sphere shaped RBCs which are fragile and break down when passing through spleen
35
key findings on ix in hereditary spherocytosis
- FHx (AD)
- spherocytes on blood film
- MCHC (mean corpuscular concentration) is raised on FBC
- reticulocytes raised (high turnover of RBC)
36
tx of hereditary spherocytosis/elliptocytosis
folate supplementation + splenectomy
| cholecystectomy may be required if problematic gallstones
37
what is hereditary elliptocytosis
autosomal dominant inherited condition where the RBCs are ellipse shaped and fragile meaning they break down easily when passing through the spleen
38
what is G6PD deficiency
defect in the RBC enzyme G6PD - causes haemolytic crises which are triggered by infections, medications or fava beans (broad beans)
39
presentation of G6PD deficiency
- jaundice (usually in neonatal period)
- gallstones
- anaemia
- splenomegaly
40
ix in G6PD deficiency
- blood film shows Heinz bodies
| - G6PD enzyme assay is diagnostic
41
medications which can trigger G6PD haemolysis
primaquine (antimalarial)
ciprofloxacin
sulfonylureas
sulfasalazine
42
what is autoimmune haemolytic anaemia
antibodies created against own RBC leading to destruction of RBC
43
classification of autoimmune haemolytic anaemia
depends on temperature of when the autoantibodies work and cause haemolysis
- cold
- warm (more common)
44
pathophysiology of cold autoimmune haemolytic anaemia
at temperatures <10c, autoantibodies attach themselves to RBCs and cause agglutination (clump together) resulting in destruction of RBCs
45
aetiology of cold autoimmune haemolytic anaemia
secondary to lymphoma, leukaemia, SLE, infection (mycoplasma, EBV, CMV, HIV)
46
aetiology of warm autoimmune haemolytic anaemia
idiopathic
47
mx of autoimmune haemolytic anaemia
- blood transfusions
- prednisolone
- rituximab
- splenectomy
48
what is alloimmune haemolytic anaemia
foreign RBCs or foreign antibody circulating causing an immune reaction leading to destruction of RBCs (haemolysis)
49
2 scenarios that can lead to alloimmune haemolytic anaemia
1. transfusion reactions
| 2. haemolytic disease of the newborn
50
what is microangiopathic haemolytic anaemia
small blood vessels have structural abnormalities causing haemolysis of blood cells travelling through them
51
aetiology of microangiopathic haemolytic anaemia
normally secondary to another condition:
- haemolytic uraemic syndrome
- DIC
- thrombotic thrombocytopaenic purpura
- SLE
- cancer
52
mx of prosthetic valve haemolysis
- monitoring
- oral iron
- blood transfusion if severe
- revision surgery may be required in severe cases
53
genetics of thalassaemia
autosomal recessive
54
presentation of thalassaemia
- microcytic anaemia
- fatigue
- pallor
- jaundice
- gallstones
- splenomegaly
- poor growth and development
- pronounced forehead and cheekbones (expanded bone marrow)
55
dx of thalassaemia
- FBC -> microcytic anaemia
- Hb electrophoresis to diagnose globin abnormalities
- DNA testing
56
when is thalassaemia screening offered
booking appointment in pregnancy (UK)
57
how does iron overload occur in thalassaemia
faulty RBC creation, recurrent transfusions and increased absorption of iron in response to anaemia
58
common complication in thalassaemia
iron overload
59
monitoring for iron overload in thalassaemia
monitoring of serum ferritin levels
60
mx of iron overload in thalassaemia
limiting transfusions
| iron chelation
61
complications of iron overload in thalassaemia
- fatigue
- liver cirrhosis
- infertility and impotence
- heart failure
- arthritis
- diabetes
- osteoporosis and joint pain
62
chromosome responsible in alpha thalassaemia
16
63
chromosome responsible in beta thalassaemia
11
64
mx of alpha thalassaemia
- monitoring FBC
- watch for complications
- blood transfusions
- splenectomy
- bone marrow transplant -> can be curative
65
types of beta thalassaemia and difference
- thalassaemia major => both deletion genes
- thalassaemia intermedia => both defective or one defective + one deletion
- thalassaemia minor => only one abnormal (deletion or defective)
66
disease course in thalassaemia minor
causes mild microcytic anaemia
| pts usually only require monitoring and no active tx
67
disease course in thalassaemia intermedia
causes more significant microcytic anaemia
| pts require monitoring and occasional blood transfusions +/- iron chelation to avoid overload
68
disease course in thalassaemia major
causes severe microcytic anaemia, splenomegaly and bone deformities
69
mx of beta thalassaemia major
- regular transfusions
- iron chelation to avoid overload
- splenectomy
- bone marrow transplant (potentially curative)
70
what is sickle cell anaemia
genetic condition causing sickle (crescent) shaped RBCs when deoxygenated meaning they are more fragile and susceptible to destruction -> haemolytic anaemia
71
genetics of sickle cell anaemia
autosomal recessive condition where there is an abnormal gene for beta-globin on chromosome 11
one copy = trait, usually asymptomatic
two copies = disease
72
how is sickle cell related to malaria
sickle cell is more common in patients from traditionally malaria affected areas
-> sickle cell trait (one copy) reduces severity of malaria therefore more prevalent through evolution
73
when is sickle cell tested for
pregnancy in those at risk of being carriers
| newborn screening heel prick test at 5 days old
74
complications of sickle cell
- anaemia
- increased infection risk
- stroke
- avascular necrosis
- pulmonary hypertension
- painful and persistent erection (priapism)
- CKD
- sickle cell crises
- acute chest syndrome
75
lifestyle mx of sickle cell
- avoid dehydration and other triggers
| - vaccination
76
medication mx of sickle cell
- abx prophylaxis (pen v)
| - hydroxycarbamide to stimulate production of foetal haemoglobin (doesn't sickle)
77
procedural mx options in sickle cell
- blood transfusion in severe anaemia
| - bone marrow transplant (may be curative)
78
what is meant by sickle cell crises
spectrum of acute crises related to the condition which can be mild to life-threatening. may be spontaneous or triggered by stresses.
79
stresses which may trigger sickle cell crises
infection
dehydration
cold
significant life events
80
mx of sickle cell crises
supportive mx:
- low threshold for admission
- tx infection
- keep warm
- hydration
- simple analgesia
- penile aspiration in priapism
81
4 examples of sickle cell crises
1. vaso-occlusive crisis (painful crisis)
2. splenic sequestration crisis
3. aplastic crisis
4. acute chest syndrome
82
what is vaso-occlusive crisis
sickle cell crisis where sickle shaped RBCs clog capillaries causing distal ischaemia
83
sx of vaso-occlusive crisis
pain
fever
sx of triggering infection
may cause priapism
84
triggers for vaso-occlusive crisis
dehydration
infection
raised haematocrit
85
what is splenic sequestration crisis
sickle cell crisis where RBCs block blood flow within spleen causing acutely enlarged and painful spleen
can lead to severe anaemia and hypovolaemic shock
86
mx of splenic sequestration crisis
```
emergency:
- blood transfusion to tx anaemia
- fluid resuscitation to tx shock
definitive:
- splenectomy
```
87
what is aplastic crisis
temporary loss of creation of new RBCs causing severe anaemia
88
trigger for aplastic crisis
parvovirus B19
89
mx of aplastic crisis
blood transfusions if necessary
| usually resolves spontaneously within a week
90
what is acute chest syndrome
sickle sell crisis:
- fever or respiratory sx with
- new infiltrates seen on CXR
91
causes of acute chest syndrome
infection (pneumonia, bronchiolitis)
| non-infective (pulmonary vaso-occlusion, fat emboli)
92
tx of acute chest syndrome
emergency:
- abx or antivirals
- blood transfusions
- incentive spirometry (encourages effective and deep breathing)
- artificial ventilation (NIV, intubation)
93
5 causes of thrombocytopaenia related to production issues
1. sepsis
2. b12 or folate deficiency
3. liver failure (reduced thrombopoietin production)
4. leukaemia
5. myelodysplastic syndrome
94
6 causes of thrombocytopaenia related to destruction issues
1. medications
2. alcohol
3. immune thrombocytopaenic purpura
4. thrombotic thrombocytopaenia purpura
5. heparin-induced thrombocytopaenia
6. haemolytic uraemic syndrome
95
medications that can cause thrombocytopaenia
- heparin
- sodium valproate
- methotrexate
- antihistamines
- PPIs
96
presentation of thrombocytopaenia
- incidental finding
- if count <50: nosebleeds, bleeding gums, menorrhagia, easy bruising, haematuria
- if count <10: spontaneous IC or GI haemorrhage
97
differentials for abnormal or prolonged bleeding
- thrombocytopaenia
- haemophilia A or B
- von willebrand disease
- DIC secondary to sepsis
98
what is ITP
immune thrombocytopaenia purpura
| antibodies created against platelets causing an immune response that destroys the platelets
99
4 medical mx options for ITP
- prednisolone
- IV Ig
- rituximab
- splenectomy
100
general mx/advice in ITP
- safety net about concerning bleeding e.g. melaena, persistent headaches
- control BP
- suppress periods
101
what is TTP
thrombotic thrombocytopaenia purpura
tiny blood clots develop throughout small vessels of body and use up platelets resulting in thrombocytopaenia, bleeding under the skin etc.
= microangiopathy
102
pathophysiology of TTP
blood clots develop in small vessels throughout the body in response to a lack of protein ADAMTS13 (normally inactivates vWF to prevent clot formation)
clots lead to haemolytic anaemia and use up platelets causing thrombocytopaenia
103
aetiology of TTP
- inherited gene mutation causing deficiency in ADAMST13 protein
- autoimmune conditions creating antibodies against ADAMST13 protein
104
tx options in TTP
- plasma exchange
- steroids
- rituximab
105
what is von Willebrand disease
most common inherited cause of abnormal bleeding due to problem with von Willebrand factor
106
genetics of von willebrand disease
autosomal dominant
107
presentation of von willebrand disease
hx of easy, prolonged or heavy bleeding
- bleeding gums
- nose bleeds
- menorrhagia
- heavy bleeding during surgery
- FHx heavy bleeding or von willebrand disease
108
mx of von willebrand disease
only required in response to major bleeding or trauma or in preparation for operations:
- desmopressin stimulates release of vWF
- vWF infusion
- factor VIII
109
mx of menorrhagia in von willebrand disease
- tranexamic acid
- mefanamic acid
- norethisterone
- COCP
- Mirena coil
110
pentad of symptoms/signs in thrombotic thrombocytopaenic purpura
1. fever
2. altered mental state
3. thrombocytopaenia
4. haemolytic anaemia
5. renal impairment
