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Paeds Haem Flashcards

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1
Q

What is ferritin ?

A

Stored iron in cells

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2
Q

What does low ferritin suggest ?

A

Iron deficiency

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3
Q

What does high ferritin suggest ?

A
  • Inflammation
  • Ferritin is released from cells when there is inflammation such as with infection or cancer
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4
Q

What happens to the TIBC and transferrin levels in IDA?

A

Increased

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5
Q

How is IDA treated ?

A
  • Ferrous sulphate or ferrous fumarate
  • Blood transfusions if necessary
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6
Q

what causes ITP?

A
  • Type II hypersensitivity reaction
  • Antibodies are produced that destroy the platelets
  • Can occur spontaneously or as a result of a VIRAL INFECTION
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7
Q

How does ITP present ?

A
  • Usually a history of a viral illness
  • Onset of symptoms is 24-48hrs
  • Bruising
  • Petechial or purpuric rash
  • Bleeding : gums, epistaxis or menorrhagia (less common)
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8
Q

Who does ITP usually effect ?

A

-Children <10

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9
Q

How is ITP diagnosed ?

A

-FBC for platelet count = isolated thrombocytopenia

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10
Q

Give 3 causes of low plts

A
  • ITP
  • Heparin induced thrombocytopenia
  • Leukaemia
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11
Q

How is ITP treated if patient is actively bleeding or if plt levels are severe (<10)

A
  • Usually resolves on own
  • Prednisolone
  • IV immunoglobulins
  • Platelet transfusion (if required)
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12
Q

Why would a plt transfusion for ITP only work temporarily ?

A

-The antibodies will destroy the transfused plts

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13
Q

What key education is given in ITP?

A
  • Avoid contact sports
  • Avoid IM injections
  • Avoid NSAIDs, aspirin and other blood thinning meds
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14
Q

Give 4 complications of ITP

A
  • Chronic ITP
  • Anaemia
  • Intracranial and SAH
  • Gastrointestinal bleeding
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15
Q

What is thalassaemia ?

A

-Autosomal recessive condition causing a genetic defect in the protein chains that make up Hb.

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16
Q

Give 8 potential signs and symptoms of thalassaemia

A
  • Microcytic anaemia
  • Fatigue
  • Pallor
  • Jaundice
  • Gallstones
  • Splenomegaly
  • Poor growth and development
  • Pronounced forehead and malar eminences
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17
Q

How is thalassaemia diagnosed ?

A
  • Pregnant screening test
  • FBC -> microcytic anaemia
  • Haemoglobin electrophoresis -> globin abnormalitis
  • DNA testing -> genetic abnormality
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18
Q

Why can thalassaemia cause iron overload?

A
  • Faulty creation of RBC
  • Recurrent transfusions
  • Increased iron absorption from the gut due to anaemia
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19
Q

Give 8 signs of iron overload

A
  • Fatigue
  • Liver cirrhosis
  • Infertility
  • Impotence
  • HF
  • Arthritis
  • DM
  • Osteoporosis and joint pain
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20
Q

How is potential iron overload monitored and then treated ?

A
  • Monitor serum ferritin
  • Treat with iron chelation (desferrioxamine) and limit transfusions
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21
Q

What chromosome is involved in alpha thalassaemia ?

A

-16
- 4 genes, 2 on each chromosome

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22
Q

How is alpha thalassaemia managed ?

A
  • Monitor FBC
  • Monitor for complications
  • Blood transfusions
  • Splenectomy can be performed
  • BM transplant can be curative
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23
Q

What chromosome is involved in beta thalassaemia and why is there 3 types ?

A

11
-Gene defect can either consist of abnormal copies that retian some function or deletion of genese where there is no function :

  • Thalassaemia minor
  • Thalassaemia intermedia
  • Thalassaemia major
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24
Q

What is thalassaemia minor ?

A
  • Carriers of abnormally functioning beta globin gene
  • 1 abnormal, one normal
  • Causes mild hypochromic microcytic anaemia and pts often only require monitoring and no active treatment
  • Raised HbA2
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25
What is thalassaemia intermedia
- 2 defective genese or one defective and one deletion gene - More significant anaemia - Need monitoring and occassional blood transfusions - May require iron chelation to prevent iron overload
26
What is thalassaemia major ?
- Homozygous for the deletion gene - Severe : presents with severe anaemia and failure to thrive in early childhood
27
What are 3 complications of thalassaemia major ?
- Severe microcytic anaemia - Hepatosplenomegaly in the 1st year of life - Bone deformities - Raised HbF and HbA2, absent HbA
28
What is sickle cell anaemia ?
- Autosomal recessive condition causing crescent shaped RBC's - They are more fragile and more easily destroyed = haemolytic anaemia
29
Explain the pathophysiology behind sickle cell anaemia
- Pts have an abnormal Hb variant - It is an autosomal recessive condition where there is an abnormal gene for beta-globin on chromosome 11 - One copy = sickle trait and usually asymptomatic (HbAS) - 2 copies = sickle cell disease (HbSS)
30
Why is having sickle cell trait a selective advantage in areas affected by malaria ?
- It reduces the severity of malaria | - Makes them more likely to survive malaria and pass on their genes
31
How is sickle cell disease diagnosed
- Newborn screening heel prick test at 5 days old or by Hb electrophoresis - Pregnant women at risk of being carriers are offered testing during pregnancy.
32
What is the general management of sickle cell anaemia ?
- Avoid dehydration and other triggers - Keep vaccines up to date - Abx prophylaxis with penicillin V - Hydroxycarbamide to stimulate HbF production -> protective effect against sickle cell crisis and acute chest syndrome - Blood transfusion for severe anaemia - Stem cell transplant can be curative
33
Give 9 complications of sickle cell disease
- Anaemia - Avascular necrosis of large joints - Pulmonary HTN - CKD - Sickle cell crisis - Acute chest syndrome - Increased risk of infection - Stroke - Priapism
34
Give 4 examples of a sickle cell crisis
- Vaso-occlusive crisis (painful) - Splenic sequestration - Aplastic - Acute chest syndrome
35
What is a vaso-occlusive crisis
- Precipitated by infection, dehydration or deoxygenation. - The sickle RBC's clog capillaries and cause distal ischaemia - Presents with pain, fever and signs of triggering infection - Associated with dehydration and raised haematocrit - Can cause priapism in med which is treated with aspiration of blood from the penis
36
What is a splenic sequestration crisis ?
- RBC's block blood flow to the spleen = enlarged and painful spleen - Pooling of blood in the spleen can lead to severe and anaemia and circulatory collapse (hypovolaemic shock) - Mx : blood transfusion, fluid resus - Recurrent crises -> splenectomy as reccurrent crisis can lead to splenic infarct - Associated with raised reticulocyte count
37
What is an aplastic crisis ?
- Temporary loss of creation of new blood cells - Usually triggered by parovovirus B19 - Leads to severe anaemia and is managed with blood transfusion if necessary - BM supression -> raised reticuloytes
38
When is acute chest syndrome diagnosed ?
-Fever or resp symptoms with NEW INFILTRATES seen on CXR and low pO2
39
What causes acute chest syndrome ?
- Infection (pneumonia, bronchiolitis) - Non-infective (pulmonary vaso-occlussion or fat emboli)
40
How is acute chest syndrome managed ?
- Abx or antivirals for infection - Blood transfusions for anaemia - Incentive spirometry for breathing - Artifical ventilation with NIV or intubation if required
41
What is haemophilia and its causative deficiencies
- X linked recessive bleeding disorder - Haemophilia A : deficiency in factor VIII - Haemophilia B : deficiency in factor IX
42
Explain the inheritance of haemophilia
- X linked recessive - Almost exclusively affects males
43
How does haemophilia present
- Excessive bleeding in response to trauma or spontaneous haemorrhage - Usually in neonates or early childhood with intracranial haemorrhage, haematomas and cord bleeding - E.g. 11mnth -> bruising, painful and swollen joint but no fever
44
Where can bleeding occur in haemophilia ?
- Joints (haemathrosis) and muscles = severe - Gums - GI tract - Urinary tract -> haematuria - Retroperitoneal space - Intracranial - Following procedures
45
How is haemophilia diagnosed ?
- 1st line = clotting screen - Isolated raised APTT
46
How is an acute episode of bleeding managed in haemophilia ?
- Infusions of the factor - Desmopressin to stimulate VWF release - Antifibrinolytics such as tranexamic acid
47
What is a complication of clotting factor IV infusions
-Abx can be made against the factors
48
What is the most common inherited cause of abnormal bleeding ?
-Von Willebrand Disease
49
What is the inheritance pattern of VWD?
- Autosomal dominant - Involves deficiency, absence or malfunctioning VWF - 3 types, type 3 most severe (autosommal recessive)
50
How does VWD present ?
- Hx of unusually easy, prolonged or heavy breathing - Bleeding gums with brushing - Epistaxis - Menorrhagia - Heavy bleeding during surgical operations - Fx of heavy bleeding or VWD
51
How is major trauma treated in VWD?
- Desmopressin to stimulate release of VWD - VWF infusion - Factor VIII
52
What are the 4 subunits of haemoglobin in the foetus (HbF)
- 2 alpha subunits - 2 gamma subunits
53
Explain the difference between adult and foetal haemoglobin, in terms of the oxygen dissociation curve
- Fetal HbF has a greater affinity for oxygen that HbA - HbF binds to oxygen more easilty and is more reliuctan to let go - It sits further left on the oxygen dissociation curve
54
When does HbF production being to decrease ?
- From 32 to 36 weeks gestation - HbA is then produced in greater quantities
55
What can be given to increase HbF production in patients with sickle cell anaemia ?
-Hydroxycarbamide
56
What is the most common cause of anaemia in infancy ?
-Physiologic anaemia of infancy
57
Give 4 other causes of anaemia in infancy?
- Anaemia of prematurity - Blood loss - Haemolysis - Twin Twin transfusion -> blood is unequally distributed between twins that share a placenta
58
Give 3 causes of haemolysis in neonates
- Haemolytic disease of the newborn (ABO or rhesus incompatibility) - Hereditary spherocytosis - G6PD deficiency
59
What is Physiologic Anaemia of Infancy and when does it occur ?
- Six to nine weeks - High oxygen delivery to the tissues due to high HbF levels at birth leads to negative feedback - There is reduced erythropoietin produced by the kidneys leading to reduced HbF by the bone marrow - High oxygen = lower haemoglobin production
60
Give 4 reasons why premature neonates become anaemic
- Less time in utero receiving iron from the mother - RBC creation cannot keep up with rapid growth in the first few weeks - Reduced erythropoietin levels - Blood tests remove a significant portion of their circulating volume
61
What is haemolytic disease of the newborn ?
-Incompatibility between rhesus antigens on the surface of RBC of the mother and fetus
62
Explain the pathophysiology behind haemolytic disease of the newborn
- In subsequent pregnancies, the mothers anti-D antibodies cross the placenta - If the fetus is resus positive, the Abx attack the RBC - This leads to haemolysis, anaemia and high bilirubin levels
63
How is immune haemolytic anaemic diagnosed ?
Direct coombs test
64
Give the 2 most common causes of anaemia in older children
- IDA -> secondary to dietary insufficiency (most common cause overall) - Blood loss -> most commonly menstruation in older girls
65
Give 6 rarer causes of anaemia in children
- Sickle cell - Thalassaemia - Leukaemia - Hereditary spherocytosis - Hereditaty eliptocytosis - Sideroblastic anaemia
66
What is a common cause of blood loss leading to IDA and chronic anaemia in developing countries ?
-Helminth infection : roundworms, hookworms or whipworms
67
How is a Helminth infection treated ?
-Single dose of albendazole or mebendazole
68
Give 5 causes of microcytic anaemia
-TAILS T: Thalassaemia A : Anaemia of chronic disease I : Iron deficiency anaemia L : Lead poisoning S : Sideroblastic anaemia
69
Give 5 causes of normocytic anaemia
-3A's, 2H's A : acute blood loss A : anaemia of chronic disease A : aplastic anaemia H : Haemolytic anaemia H : hypothyroidism
70
How can macrocytic anaemia be classified ?
- Megaloblastic : impaired DNA synthesis preventing if dividing normally = continuous growth into a large, abnormal cell. Caused by a vitamin deficiency - Normoblastic
71
Give 2 causes of megaloblastic macrocytic anaemia
- B12 deficiency - Folate deficiency
72
Give 5 causes of normoblastic macrocytic anaemia
- Alcohol - Reticulocytosis (from haemolytic anaemia or blood loss) - Hypothyroidism - Liver disease - Drugs such as azathroprine
73
Give 5 generic symptoms of anaemia
- Tiredness - SOB - Headaches - Dizziness - Palpitations
74
Give 2 symptoms specific to IDA
- Pica -> dietary cravings for abnormal things such as dirt - Hair loss
75
Give 4 general signs of anaemia
- Pale sign - Conjunctival pallor - Tachycardia - Raised resp rate
76
Give 4 specific signs of IDA
- Koilonychia -> spoon shaped nails - Angular stomatitis - Atrophic glossitis - Brittle hair and nails
77
Give 8 initial investigations for anaemia
- FBC for Hb and MCV - Blood film - Reticulocyte count (high in anaemia due to haemolysis or blood loss) - Ferritin (low in IDA) - B12 and folate - Bilirubin (raised in haemolysis) - Direct coombs test (autoimmune haemolytic anaemia) - Haemoglobin electrophoresis (haemoglobinopathies)
78
Give 3 causes of iron deficiency
- Dietary insufficiency (most common in children) - Loss of iron -> heavy menstruation - Inadequate iron absorption (e.g. crohn's)
79
Where is iron absorbed and what is required ?
- Duodenum and jejunum -> any conditions causing inflammation can cause inadequate absorption - Stomach acid to maintain is soluble ferrous (Fe2+) form. Medications such as PPI's reduced stomach acid = ferric Fe3+ form = insoluble
80
What is the total iron binding capacity ?
-Total space on the transferrin molecule for iron to bind
81
What is the transferrin saturation ?
-Proportion of transferrin molecules that iron is bound to?
82
How is the transferrin saturation calculated ?
Transferrin saturation = Serum Iron / Total Iron Binding Capacity
83
What is hereditary spherocytosis and how is it inherited ?
- RBC are sphere shaped = easily destroyed - Autosomal dominant, along woth hereditary elliptocytosis
84
How does hereditary spherocytosis present?
- Jaundice - Anaemia - Gallstones - Splenomegaly
85
What infection can trigger an aplastic crisis in hereditary spherocytosis ?
-Parovirus
86
What is an aplastic crisis
-Anaemia, haemolysis and jaundice is increased without the BM creating new RBCs
87
How is hereditary spherocytosis diagnosed and anaged ?
- Blood film : spherocytes, raised MCHC, raised reticulocytes - Manage : folate and splenectomy
88
What is. theinheritance pattern of G6PD deficiency and how can a crisis be triggered ?
- X linlked recessive - Infections, medications, fava (broad) beans - Exam presentation : jaundice following the triggers above
89
Explain the pathophysiology behond G6PD deficiency
- Deficiency in G6PD = cells more vulnerable to ROS = haemolysis of RBC - Period of increased stress = higher production of ROPS = acute haemolytic anaemia
90
How does G6PD deficiency present ?
- Neonatal jaundice - Anaemia, intermittent jaundice, gallstones, splenomegaly
91
What is seen on investigations in G6PD deficiency
- Heinz bodies on blood film - Diagnosed using G6PD enzyme assay
92
what is the inheritance of fanconi anaemia
Autosommal recessive
93
what are the 4 features of fanconi anemia
- Haematological : Aplastic anaemia with increased risk of AML - Neurological - Skeletal abnormalities : short stature, thumb/radius abnormalities - Cafe au lait spots
94
what is seen on blood results in leukaemia ?
- Pancytopenia, combination of : - Anaemia - Leukopenia - Thrombocytopenia
95
Give 4 genetic syndromes that predispose to a higher risk of developing leukaemia
Down’s syndrome Kleinfelter syndrome Noonan syndrome Fanconi’s anaemia
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