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Year 3- Specialties > Haematology- Red Cells > Flashcards

Haematology- Red Cells Flashcards

1
Q

What is anaemia?

A

Reduction in RBCs or their haemoglobin content.

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2
Q

What are some of the different underlying causes of various anaemias?

A

Blood loss
Increased destruction
Lack of production
Defective production

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3
Q

List some of the substance required for RBC production.

A

Metals

Vitamins

Amino acids

Hormones

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4
Q

Which metals are needed for the production of RBCs?

A

Iron
Copper
Cobalt
Manganese

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5
Q

Which vitamins are required for the production of RBCs?

A

B12, folic acid, thiamine, Vit.B6, C and E

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6
Q

Which hormones are required for the production of RBCs?

A

Erythropoietin
Androgens
Thyroxine
GM-CSF

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7
Q

What is erythropoietin?

A

A hormone synthesised in the kidneys in response to hypoxia

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8
Q

How long is normal RBC lifespan?

A

120 days

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9
Q

Where is haemoglobin broken down?

A

Macrophages in spleen, liver, lymph nodes and lungs

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10
Q

What are the components of haemoglobin?

A

Haem= iron

Globin= amino acids

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11
Q

What can haem also be converted into?

A

Bilirubin

->bilirubin is bound to albumin in the plasma

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12
Q

What is haemolysis?

A

Destruction of RBCs

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13
Q

What is hereditary spherocytosis?

A

Genetic blood disorder where RBCs are spherical

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14
Q

Which type of inheritance pattern is hereditary spherocytosis?

A

Most common forms autosomal dominant

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15
Q

What is the clinical presentation of hereditary spherocytosis?

A

Variable.

Can be anaemia, neonatal jaundice, splenomegaly, pigment gallstones.

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16
Q

Treatment of hereditary spherocytosis?

A

Folic acid
Transfusion

Splenectomy if anaemia is very severe

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17
Q

What is the role of the enzyme Glucose 6 Phosphate Dehydrogenase (G6PD)?

A

Protects RBC proteins (haemoglobin) from oxidative damage

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18
Q

What is G6PD deficiency?

A

X lined genetic condition meaning affects males and females are carriers.

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19
Q

Individuals with G6PD deficiency have some protection against which disease?

A

Malaria

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20
Q

What cells are seen upon histology of those with G6DP deficiency?

A

Blister cells
Bite cells

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21
Q

Clinical presentation of G6PD deficiency?

A

Variable.

-variable degrees of anaemia
-neonatal jaundice
-splenomegaly
-pigment gallstones

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22
Q

Haemolysis can occur in those with G6PD deficiency.

What are some of the triggers which cause the haemolysis?

A

Infection
Acute illness e.g. DKA
Broad/fava beans (kinda cool actually)
Drugs

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23
Q

List some of the drugs which can trigger haemolysis in patients with G6PD deficiency.

A

Antimalarials
Antibacterial- nitrofurantoin
Analgesics- aspirin
Antihelminthics- B-napthrol

Miscellaneous- vitamin K analogues

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24
Q

Haemoglobin function?

A

Gas exchange- oxygen to tissues, carbon dioxide to lungs

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25
Describe the structure of haemoglobin.
Haem molecule 2 alpha chains 4 alpha genes 2 beta chains 2 beta genes
26
Give some examples of haemoglobinopathies.
Thalassemia Sickle cell anaemia
27
Describe the structure of sickle haemoglobin.
Haem molecule 2 alpha chains 2 beta sickle chains
28
What type of mutation causes sickle cell disease?
Single point mutation
29
Sickle cell disorder is one of the commonest inherited disorders worldwide. What is the pattern of inheritance for sickle cell disorder?
Autosomal recessive ->1 in 4 chance of having an affected child 1 in 2 chance having a carrier child
30
What are the four stages on sickle cell pathophysiology?
1. Haemoglobin S polymerization 2. Vaso-occlusion 3. Endothelial dysfunction 4. Sterile inflammation
31
What are some of the triggers for a sickle crisis?
Infection Hypoxia Dehydration Cold Stress
32
What is meant by sickle cell crisis?
A spectrum of acute exacerbations caused by sickle cell disease. These range from mild to life-threatening.
33
Treatment for sickle cell crisis?
Supportive management Low threshold for admitting Analgesia Hydration Treatment of infectious causes Oxygen
34
What is vaso-occlusive crisis aka painful crisis?
Most common type of sickle cell crisis. Sickle shaped RBCs clog capillaries, causing distal ischaemia.
35
Splenic sequestrion crisis?
RBCs block blood flow to spleen leading to enlarged spleen. ->Blood pooling in the spleen can lead to severe anaemia and hypovolaemic shock. Splenectomy is considered if recurrent.
36
Aplastic crisis?
Temporary absence of the creation of new red blood cells.
37
Which infection commonly triggers aplastic crisis?
Paravirus B19
38
Management of aplastic crisis?
Supportive, with blood transfusions if necessary
39
How are acute events of sickle cell disease managed?
Hydration Oxygenation Prompt treatment of infection Analgesia- Opiates or NSAIDs Blood transfusions
40
What are the long term prophylaxis managements of sickle cell disease?
Vaccination Penicillin and malarial prophylaxis Folic acid
41
What is some of the more general management for sickle cell disease?
Disease modifying drugs: -Hydroxycarbamide -Voxelotor Bone marrow transplantation Gene therapy Gene editing
42
What happens in thalassaemias?
Reduced or absent globin chain production ->chain imbalance can lead to chronic haemolysis and anaemia
43
What happens in beta thalassemia major?
Severe anaemia Presents at 3-6months of age Expansion of ineffective bone marrow
44
What are some of the symptoms of beta thalassaemia major?
Bony deformities Splenomegaly Growth retardation ->life expectancy if untreated or with irregular transfusions is <10yrs
45
Treatment of beta thalassaemia major?
Chronic transfusion support every 4-6weeks ->this means there can be normal growth and development
46
Despite beta thalassaemia being treated with chronic transfusion support, allowing for normal growth and development, what else can happen?
Iron overloading ->if untreated, death can occur in 2nd or 3rd decade due to heart/liver/endocrine failure
47
What can be done for iron overloading in the treatment of beta thalassaemia major?
Iron chelation therapy
48
What is the curative treatment for beta thalassaemia major
Bone marrow transplant
49
What are some of the general symtpoms of anaemia?
Tiredness Pallor Breathlessness Swelling of ankles Dizziness Chest pain ->symptoms depend on age, speed of onset and Hb level
50
Anaemia is not a diagnosis of itself. It's related to underlying cause. Which system in anaemia usually related to??
GI ->e.g. iron deficiency cough cough
51
Three subtypes of anaemia?
Hypochromic microcytic Normochromic normocytic Macrocytic ->hypochromic= lack of colour -->microcytic= small cells etc. etc.
52
Next investigation if hypochromic microcytic anaemia?
Serum ferritin
53
Commonest cause of hypochromic microcytic anaemia?
Iron deficiency ->ferritin is iron stores so that is why next investigation
54
Good investigation for normochromic normocytic anaemia?
Reticulocyte count
55
Good investigations for macroscopic anaemia?
B12/folate levels Bone marrow
56
Commonest cause of macroscopic anaemia?
B12/folate deficiency
57
In hypochromic microcytic anaemia, if serum ferritin is low, what is the cause?
Iron deficiency
58
In hypochromic microcytic anaemia, if serum ferritin is normal or increased, what is the cause?
Possibly thalassaemia Or secondary anaemia due to chronic disease
59
Where is iron absorbed?
Duodenum
60
Where and what is iron stored?
Stored in liver as ferritin
61
When you have enough iron, what happens?
Hepcidin synthesised in the liver and blocks ferroportin. This reduces intestinal iron absorption. ->unlikely to be questioned on but for interest
62
Iron-deficiency anaemia is very common, most common cause of anaemia. It is not a diagnosis. You need to determine the cause of the iron deficiency. What are some of the causes?
Dyspepsia GI bleeding Diet Other bleeding e.g. menorrhagia Increased requirement e.g. pregnancy
63
Clinical features of anaemia?
Angular stomatitis Atrophic tongue Koilonychia- spoon shaped nails
64
Management of iron deficiency?
Oral iron tablets IV iron if cannot have oral Blood transfusion but very rare Correct the cause too!!
65
If reticulocyte count in increased in normochromic normocytic anaemia, what are the possible causes?
Acute blood loss Haemolysis
66
If reticulocyte count is normal or low in normochromic normocytic anaemia, what is the cause?
Secondary anaemia, perhaps to chemotherapy
67
Which type of anaemia are the majority of anaemia of chronic disease?
70% normochromic normocytic anaemia
68
In which disease can there be elevated ferritin levels?
Liver disease
69
What happens in haemolytic anaemia?
Accelerated red cell destruction ->how anaemic you become depends on how quickly you can produce RBCs vs how quickly they are destroyed
70
What are some of the conditions which can cause cogenetic haemolytic anaemia?
Hereditary spherocytosis (HS) Enzyme deficiency (G6PD deficiency) Haemoglobinopathy (HbSS)
71
What are some of the acquired causes of haemolytic anaemia?
Extravascular: Auto-immune haemolytic anaemia Intravascular: Mechanical eg.artificial valve Severe infection/DIC PET/HUS/TTP ->basically immune or non-immune.
72
Which test confirms if haemolytic anaemia is of immune or non-immune cause?
DAT ->DAT +ve means immune cause
73
Management of haemolytic anaemia?
Support marrow function by giving folic acid Correct cause- immunosuppression if autoimmune, remove site of RBC destruction e.g. splenectomy
74
What is megaloblastic anaemia caused by?
B12 deficiency Folate deficiency ->macrocytic anaemia is either classified as megaloblastic or non-megaloblastic
75
Commonest causes of vitamin B12 deficiency?
Pernicious anaemia Gastric/ileal disease
76
Commonest cause of folate deficiency?
Diet
77
For B12 to be absorbed by the gut, what does it need to bind to?
Intrinsic factor
78
What clinical sign can be seen in megaloblastic anaemia?
Lemon yellow tinge -.due to higher levels of bilirubin as their RBCs are friable
79
What type of disease is pernicious anaemia?
Autoimmune disease ->can have antibodies against intrinsic factor so B12 cannot be absorbed
80
Why do symptoms of pernicious anaemia take 1-2yrs to show?
Stores of B12 last a long time
81
Treatment of megaloblastic anaemia?
Replace vitamin B12- IM injection or Replace folate, depending of the deficiency. However, if giving folate, you also have to give vitamin B12
82
Which other things can cause macrocytic anaemia?
Alcohol Drugs- methotrexate, antiretroviral, hydroxycarbamide Disordered liver function Hypothyroidism Myelodysplasia
83

Year 3- Specialties (26 decks)

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