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Haemolytic Anaemia Flashcards

1
Q

What is anaemia

A

Reduction of one or more of the major red blood cell measurement: haemoglobin concentration, haematocrit, RBC count

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2
Q

Do men or wmen have higher ranges of blood count parameters

A

Men

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3
Q

What are the causes of microcytic anaemia

A

Iron deficiency

Thalassemia

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4
Q

What are the cuases of normocytic anaemia

A

Anaemia of chronic disease- sickle cell disease

Blood loss

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5
Q

What are the causes of macrocytic anaemia

A

Alcohol

Nutrient deficiency- vitamin b12 and folate deficiency

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6
Q

What is haemolytic anaemia

A

Anaemia due to shortened survival of circulating red blood cells due to the premature destruction

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7
Q

Where can the destruction of red blood cells occur

A

Inside the cell

Outside the cell

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8
Q

Where does extravascular haemolysis (outside the cell occur)

A

Liver
Spleen
Bone marrow

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9
Q

What are the features of haemolytic anaemia

A
  1. Increased RBC destruction which can give:
    - high LDH
    - low haptoglobin
    - high bilirubin
    - positive DAT
    - high MCV
    - spherocytes (not biconcave shape)
  2. Increased RBC production which can give:
    - reticulocytes
    - erythyorid hyperplasia in the bone marrow
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10
Q

What are the types of coombs test

A

Direct

Indirect

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11
Q

What is the direct anti globulin test (DAT/Coombs test)

A

You put anti human globulin to the patients blood which will bind to the anti RBC in the blood

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12
Q

What is the indirect anti-globulin test (coombs test)

A
  1. Take the patients plasma with the antibody

2. Add test RBC and the anti human globulin

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13
Q

What does a positive coombs test mean

A

There are antibodies that destroy your own red blood cells i.e autoimmune haemolytic anaemia

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14
Q

What is the classification of haemolytic anaemia

A
  1. Hereditary:
    - RBC interior abnormality : enzymes defects, haemoglobinopathies
    - RBC membrane abnormality: hereditary spherocytosis, hereditary elliptocytosis
  2. Acquired:
    Extrinsic factors: hyersplenism, antibody: immune haemolysis, mechanical trauma, infections, toxins and drug induced
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15
Q

Name a enzyme defect that leads to haemolytic anaemia

A

G6PD deficiency

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16
Q

What type of condition is enzyme G6DP deficiency

A

X-linked inherited condition

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17
Q

What is the normal role of G6PD

A

It protects the RBC from harmful effects of reactive oxygen radicals

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18
Q

What happens to the RBC when there is deficient g6dp

A

Becomes destroyed by the radicals

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19
Q

Which factors is g6pd deficiency precipitated by

A

Infection
Drugs
Food: fava beans

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20
Q

What would the blood film for g6pd show for diagnsosis

A

Blister cells
Bite cells
Heinz bodies

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21
Q

What is the treatment for g6pd deficiency

A

Prevention of precipitating factors
Blood transfusion
Folic acid
Splenoctomy if the spleen is destroying the RBC

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22
Q

Give another example of a enzyme deficiency that can affects the RBC

A

Pyruvate kinase deficiency

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23
Q

What type of a disorder is pyruvate kinase deficiency

A

Autosomal recessive disorder

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24
Q

What is the clinical presentation of pyruvate kinase deficiency

A

Gallstones
Jaundice
Anaemia

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25
What is the diagnosis of pyruvate deficiency by
Enzyme assay
26
What is the treatment for pyruvate kinase deficiency anaemia
If symptomatic: folic acid | Splenectomy in some cases
27
What type of abnormality does sickle cell disease show
Qualitative abnormality due to mutation of globin genes
28
What type of abnormality does thalassemia show
Quantitative abnormality
29
How is sickle cell disease diagnoses
HPLC PLOTS | Haemoglobin gel electrophoresis
30
What is the treatment of sickle cell disease
``` Folic acid Prophylactic antibiotics Transfusion ACEI- nephropathy Hydroxycarbamide Bone marrow transplant in children ```
31
What is the presentation of beta thalaessemia major
``` Early in life Failure to thrive Poor weight gain Growth delay Haemolytic anaemia Jaundice Extramedullar haemopoiesis- hepatosplenomegaly ```
32
What is the clinical presenation of beta thalassemia intermedia
Mild or severe anaemia
33
What is the clinical presentation of beta thalaessemia trait
Mild asymptomatic anaemia
34
What is the treatment of thalassemia
``` Regular blood transfusion Iron chelation therpay- due to risk of iron overload as they have a lot of blood Folic acid and prophylactic antibiotics Gene therapy Allogenic stem cell transplant ```
35
What is hereditary spherocytosis
Autosomal dominant condition | Abnormality of the red blood cells caused by defects in the structural membrane proteins
36
What shape does the RBC become in hereditary spherocytosis instead of biconcave shape
Spherical
37
What happens to spherocytes
Phagocytosed by macrophages and destructred in the spleen
38
What can hereditary spherocytosis present with in childhood
Anaemia | Jaundice
39
What is hereditary elleptocytosis
Autosomal dominant condition | Functional abnormality in one+ anchor proteins in RBC membrane (alpha spectrin protein 4.1)
40
What shape does the RBC have in hereditary elleptocytosis
Elliptical
41
What is the treatment for hereditary spherocytosis
Folic acid Blood transfusion Splenectomy for tx dependent
42
What are the causes of acquired haemolytic anaemia , immune causes
Autoimmune: cold and warm AIHA Alloimmune: red cell destruction by antibodies that are foreign antibodies eg transfusion reaction Drug induced
43
What are acquired non immune haemolytic anaemia causes
``` Infection Mechanical PNH TMA Hypersplenism Oxidant substance DIC Severe burns Renal failure ```
44
What is autoimmune haemolytic anaemia
Decompensated acquired haemolysis caused by the hosts immune system reacting to its own red cell anitgens
45
What is DAT in autoimmune haemolytic anaemia
Positive
46
What are the 2 types of autoimmune haemolytic anaemia
Cold | Warm
47
What is warm AIHA
Caused by autoantibodies of igG which binds to the red blood cells at body temp
48
What does the blood film in warm AIHA show
Spherocytes | Macrocytes
49
What are the causes of warm AIHA
Idiopathic | Secondary to: maligannt, infection, immune (sle), drugs
50
What is the treatment of warm AIHA
``` Treat underyling cause Prenisolone (steroid) Rituximab Immunosupressive Splenectomy Transfusion ```
51
What is cold AIHA
Caused by autoantibodies usually igM that bind to the rbc at cooler temperature
52
What will the blood film show in cold AIHA
Red cell agglutination
53
What will the DAT be positive for in cold AIHA
C3d
54
What is the cause for cold AIHA
Primary | Seconday to malignancy, infection, autoimmune disorders
55
What is the treatment for cold AIHA
``` Keeping the paitent warm Transfuse the blood by a blood warmer Folic acid Treat the underlying casue Rituximab ```
56
What is an alloimmune haemolytic anaemia
When the red cells are destroyed by antibodies that are foreign
57
Name an example of alloimmune haemolytic anaemia
Haemolytic disese of the new born
58
Why does haemolytic disease of the newborn happen
1. Mum is RHD negative 2. Mum has first child that is rhd+ there is no problem 3. The mum will develop sensitisation and anti rh antibody 4. When there is a second baby with rhd+ blod the mums antibody will develop antibodies to destory the babies blood
59
Give an example of a non immune acquired haemolytic anaemia
Microangiopathic haemolytic anaemia (MAHA)
60
What is MAHA
When there is intravascular haemolysis 2nd to mechanical injury of the RBC whilst passing through the fibrin strands deposited in the small blood vessels
61
What are the causes of MAHA
``` Haemolytic uraemic syndrome Thrombotic thrombocytopenia Pre eclampsia in pregenancy Disseminated carcinoma Collagen vascular disease Medication Malignant hypertension ```
62
What is thrombotic thrombocytopenia
1. A molecule called ADAMTS13 is in the circulation 2. There is VWF that interact with platelets to hold the platelet plug 3. ADAMTS13 cleaves VWF so the platelets aggreagte 4. Deficiency in ADAMTS14 mean VWF will get together and consume all the platelets so the platelets levels are low and there is high risk of thrombosis
63
What will the blood film in thrombotic thrombocytopenia show
RBC fragments
64
What is the treatment of thrombotic thrombocytopenia
``` Plasma exchange that has ADAMTS13 Steroids e.g prednisolone Folic acid Rituximab Low molecular heparin and aspirin ```

Blood Disorders (9 decks)

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