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Blood Disorders > Haemolytic Anaemia > Flashcards

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
Q

What is the diagnosis of pyruvate deficiency by

A

Enzyme assay

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26
Q

What is the treatment for pyruvate kinase deficiency anaemia

A

If symptomatic: folic acid

Splenectomy in some cases

27
Q

What type of abnormality does sickle cell disease show

A

Qualitative abnormality due to mutation of globin genes

28
Q

What type of abnormality does thalassemia show

A

Quantitative abnormality

29
Q

How is sickle cell disease diagnoses

A

HPLC PLOTS

Haemoglobin gel electrophoresis

30
Q

What is the treatment of sickle cell disease

A 
Folic acid
Prophylactic antibiotics 
Transfusion
ACEI- nephropathy 
Hydroxycarbamide
Bone marrow transplant in children
31
Q

What is the presentation of beta thalaessemia major

A 
Early in life
Failure to thrive 
Poor weight gain
Growth delay 
Haemolytic anaemia
Jaundice
Extramedullar haemopoiesis- hepatosplenomegaly
32
Q

What is the clinical presenation of beta thalassemia intermedia

A

Mild or severe anaemia

33
Q

What is the clinical presentation of beta thalaessemia trait

A

Mild asymptomatic anaemia

34
Q

What is the treatment of thalassemia

A 
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
Q

What is hereditary spherocytosis

A

Autosomal dominant condition

Abnormality of the red blood cells caused by defects in the structural membrane proteins

36
Q

What shape does the RBC become in hereditary spherocytosis instead of biconcave shape

A

Spherical

37
Q

What happens to spherocytes

A

Phagocytosed by macrophages and destructred in the spleen

38
Q

What can hereditary spherocytosis present with in childhood

A

Anaemia

Jaundice

39
Q

What is hereditary elleptocytosis

A

Autosomal dominant condition

Functional abnormality in one+ anchor proteins in RBC membrane (alpha spectrin protein 4.1)

40
Q

What shape does the RBC have in hereditary elleptocytosis

A

Elliptical

41
Q

What is the treatment for hereditary spherocytosis

A

Folic acid
Blood transfusion
Splenectomy for tx dependent

42
Q

What are the causes of acquired haemolytic anaemia , immune causes

A

Autoimmune: cold and warm AIHA
Alloimmune: red cell destruction by antibodies that are foreign antibodies eg transfusion reaction
Drug induced

43
Q

What are acquired non immune haemolytic anaemia causes

A 
Infection
Mechanical
PNH
TMA
Hypersplenism
Oxidant substance
DIC
Severe burns
Renal failure
44
Q

What is autoimmune haemolytic anaemia

A

Decompensated acquired haemolysis caused by the hosts immune system reacting to its own red cell anitgens

45
Q

What is DAT in autoimmune haemolytic anaemia

A

Positive

46
Q

What are the 2 types of autoimmune haemolytic anaemia

A

Cold

Warm

47
Q

What is warm AIHA

A

Caused by autoantibodies of igG which binds to the red blood cells at body temp

48
Q

What does the blood film in warm AIHA show

A

Spherocytes

Macrocytes

49
Q

What are the causes of warm AIHA

A

Idiopathic

Secondary to: maligannt, infection, immune (sle), drugs

50
Q

What is the treatment of warm AIHA

A 
Treat underyling cause
Prenisolone (steroid)
Rituximab
Immunosupressive
Splenectomy
Transfusion
51
Q

What is cold AIHA

A

Caused by autoantibodies usually igM that bind to the rbc at cooler temperature

52
Q

What will the blood film show in cold AIHA

A

Red cell agglutination

53
Q

What will the DAT be positive for in cold AIHA

A

C3d

54
Q

What is the cause for cold AIHA

A

Primary

Seconday to malignancy, infection, autoimmune disorders

55
Q

What is the treatment for cold AIHA

A 
Keeping the paitent warm
Transfuse the blood by a blood warmer
Folic acid 
Treat the underlying casue
Rituximab
56
Q

What is an alloimmune haemolytic anaemia

A

When the red cells are destroyed by antibodies that are foreign

57
Q

Name an example of alloimmune haemolytic anaemia

A

Haemolytic disese of the new born

58
Q

Why does haemolytic disease of the newborn happen

A
  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
Q

Give an example of a non immune acquired haemolytic anaemia

A

Microangiopathic haemolytic anaemia (MAHA)

60
Q

What is MAHA

A

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
Q

What are the causes of MAHA

A 
Haemolytic uraemic syndrome
Thrombotic thrombocytopenia 
Pre eclampsia in pregenancy 
Disseminated carcinoma
Collagen vascular disease
Medication
Malignant hypertension
62
Q

What is thrombotic thrombocytopenia

A
  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
Q

What will the blood film in thrombotic thrombocytopenia show

A

RBC fragments

64
Q

What is the treatment of thrombotic thrombocytopenia

A 
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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