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Haematology Flashcards

1
Q

Name 3 types of anaemia

A

Iron deficiency
Haemolysis - membrane/enzyme/immune
Haemoglobinopathies
Aplasia

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2
Q

Name a type of white blood cell haematology problem

A

Leukaemia

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3
Q

Name 2 causes of thrombocytopenia

A

Immune-ITP
Neonatal autoimmune
Marrow failure

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4
Q

Name 2 problems with clotting factors

A

Haemophilias

Hypercoagulable states

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5
Q

What is the normal range for Hb, MCV and WBC at birth?

A 
Hb = 149-237
MCV = 100-135
WBC = 10-26
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6
Q

What is the normal range for Hb, MCV and WBC at 2 weeks?

A 
Hb = 134-198
MCV = 88-120
WBC = 6-21
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7
Q

What is the normal range for Hb, MCV and WBC at 2 months?

A 
Hb = 94-130
MCV = 84-105
WBC = 6-18
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8
Q

What is the normal range for Hb, MCV and WBC between 2 and 6 years old?

A 
Hb = 115-135
MCV = 75-87
WBC = 5-17
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9
Q

What is the normal range for Hb, MCV and WBC between 7-12 years old?

A 
Hb = 115-155
MCV = 77-95
WBC = 4.5-14.5
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10
Q

What is the normal range for Hb, MCV and WBC between 12 and 18 years old in boys?

A 
Hb = 120-160
MCV = 78-95
WBC = 4.5-13
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11
Q

What is the normal range for Hb, MCV and WBC between 12-18 for girls?

A 
Hb = 130-160
MCV = 78-95
WBC = 4.5-13
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12
Q

Why is there a drop in normal Hb level at 2 months?

A

The HbF production has stopped but adults Hb production has not reached full level so there is a gap

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13
Q

What happens with haematopoiesis from birth to childhood?

A

Production of blood cells varies with age
By birth, virtually all bone marrow cavities are actively haematopoietic
In childhood, haematopoiesis moves to central bones - vertebrae, sternum, ribs, pelvis

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14
Q

What to pluripotent stem cells develop into in terms of haematology?

A

Mature erythrocytes
Monocytes
Megakaryocytes
Lymphocytes

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15
Q

What is stem cell development regulated by?

A

Cytokines

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16
Q

What is important in differentiating between anaemia types?

A

Mechanism
Red cell size/colour
Aetiology
Reticulocyte count

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17
Q

What mechanisms can lead to anaemia?

A

Decreased production
Increased consumption
Increased loss

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18
Q

What are the different sizes of red blood cells in anaemia?

A

Microcytic
Macrocytic
Normocytic

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19
Q

What are the different colours of red blood cells in anaemia?

A

Hypochromic

Normochromic

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20
Q

What are the 2 aetiologies associated with anaemia?

A

Congenital

Acquired

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21
Q

What are the 2 types of reticulocyte count and what do they mean?

A 
Low = lack of production
High = haemolysis/blood loss
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22
Q

What types of anaemia are related to low reticulocyte count?

A

Hypochromic, microcytic
Normochromic, microcytic
Macrocytic

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23
Q

What can cause hypochromic microcytic anaemia?

A 
Iron deficiency
Thalassaemia
Chronic inflammatory disease
Copper deficiency
Sideroblastic anaemia
Aluminium, lead intoxication
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24
Q

What can cause iron deficiency anaemia?

A

Chronic blood loss
Poor diet
Cow’s milk protein intolerance
Menstruation

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25
What can cause normochromic normocytic anaemia?
``` Chronic inflammatory disease Recent blood loss Malignancy/marrow infiltration Chronic renal failure Transient erythroblastopenia of childhood Marrow aplasia/hypoplasia HIV infection Haemophagocytic syndrome ```
26
What can cause chronic inflammatory disease?
Infection Collagen-vascular disease IBD
27
What can cause macrocytic anaemia?
``` B12/folate deficiency Hypothyroidism Oroticaciduria Chronic liver disease Lesch-Nyhan syndrome Down syndrome Marrow failure ```
28
What can cause B12 deficiency?
``` Pernicious anaemia Ileal resection Strict vegetarian Abnormal intestinal transort Congenital intrinsic factor or transcobalamin deficiency ```
29
What can cause folate deficiency?
``` Malnutrition Malabsorption Antimetabolite Chronic haemolysis Phenytoin Tremethoprime ```
30
What are the 2 causes of raised reticulocyte count?
Loss | Haemolysis
31
What can cause RBC loss?
Bleeding Burns Splenomegaly - overactive
32
What are the two types of haemolysis?
Intrinsic - which part of the RBC is faulty? | Extrinsic - what's causing destruction?
33
What are the causes of intrinsic haemolysis?
Membranopathies - hereditary spherocytosis, elliptocytosis, ovalocytosis Enymopathies - G6PD deficiency, pyruvate kinase deficiency Haemoglobinopathies - sickle cell disease, thalassaemias
34
What are the causes of extrinsic haemolysis?
Immune - autoimmune, alloimmune (transplant/haemolytic disease of the newborn), drug-induced Others - DIC, HUS, TTP, burns, Wilson's disease, vitamin E deficiency
35
What can cause severe anaemia at birth?
Haemolytic disease of the newborn | Bleeding from umbilical cord, internal haemorrhage
36
What is erythroblastosis fetalis?
Rh negative mother previously sensitised to Rh +ve cells Transplacental passage of antibodies Haemolysis of Rh +ve foetal cells
37
What are the S&S of erythroblastosis fetalis?
Severe anaemia | Compensatory hyperplasia and enlargement of blood forming organs - spleen and liver
38
What is the treatment of erythroblastosis fetalis?
Prevention of sensitisation with Rh immune globin | Intrauterine transfusion of affected foetus
39
What causes physiological anaemia of the newborn?
Fall in Hb from birth - Decreased RBC production - Plasma dilution associated with increasing blood volume - Shorter lifespan on neonatal RBCs (50-70 days) - More fragile RBCs - Switch from HbF to HbA - decreases around 3% per week, greater unloading of O2 to tissues for HbF as higher affinity for O2 than HbA
40
What causes anaemia of prematurity?
Low birth weight infants have poor erythropoietin response Protein content of breast milk may not be sufficient for haematopoiesis in the premature infant Hb level rapidly declines after birth to a low of 7-10g/dl at 6 weeks
41
What are the S&S of anaemia of prematurity?
``` Apnoea Poor weight gain Pallor Decreased activity Tachycardia ```
42
Why is iron deficiency so common in children?
Poor intake and increased requirement
43
What can cause iron deficiency anaemia?
Breast feeding | Infection
44
Where do children get iron from?
``` Iron from vegetables poorly absorbed - Only 1% from rice and spinach - 5% from wheat - 10% from soya beans - 10-20% from animal sources - Up to 30% of iron in animal sources can be absorbed in latent iron deficiency Children - 30% diet, 70% recycled red cells Adults - 5% diet, 95% recycled red cells ```
45
What are the most common causes of iron deficiency anaemia in childhood?
Low birth weight Dietary - excessive cow's milk intake Occult GI bleed eg hookworm Cow's milk intolerance
46
How does iron deficiency anaemia present?
``` Pallor Irritability Anorexia when Hb < 50 Tachycardia Cardiac dilatation Murmur Possibly splenomegaly ```
47
What does the blood film in iron deficiency anaemia look like?
Microcytic Hypochromic Low-normal reticulocytes
48
What do the iron bloods look like in IDA?
Low ferritin and serum iron Increased TIBC High ZPP
49
How do you treat IDA?
Oral therapy mainstay - 6mg/kg/day of elemental iron - Reticulocytosis in 72hr, Hgb responds at 10g/L per week, iron stores replenished by 3 months - Treatment 3-6 months to prevent relapse - Constipation common S/E if not responding check that they are taking iron as sometimes won't due to S/E - Address cause IV iron if enteral not possible RBC only if unstable - v rare needed
50
What does haemolysis lead to?
Increased RBC turnover Shortened RBC lifespan RBC's fragile - especially abnormal ones
51
What is the pathology of haemolysis?
Spleen filters out and breaks down senescent RBCs, and must work overtime, and can result in effective asplenia (sickle cell) RBC degradation products must be handled
52
What can lead to intra-corpuscular increased destruction of RBCs?
Haemoglobinopathies Enzymopathies Membranopathies
53
What can lead to extra-corpuscular increased destruction of RBCs?
Autoimmune Fragmentation Hypersplenism Plasma factors
54
Name 2 types of haemoglobinopathies
Sickle cell disease Thalassaemia (Both recessive)
55
What is normal haemoglobin like?
HbA - 2x alpha, 2x beta | HbF - 2x alpha, 2x gamma
56
What does sickle cell haemoglobin look like?
HbSS
57
What does sickle cell carrier haemoglobin look like?
HbSA
58
What does severe sickled beta thalassaemia haemoglobin look like?
HbSbeta0
59
How are haemoglobinopathies diagnosed?
High performance liquid chromatography or Hb electrophoresis
60
How is sickle cell anaemia diagnosed?
FHx key Antenatal and neonatal screening by Hb electrophoresis Followed by specialist
61
What interventions should you make sure are given to children with sickle cell disease?
Pneumococcal, influenza, meningococcal vaccines and prophylactic penicillin as functional asplenia therefore high risk for sepsis
62
What is sickle cell disease?
Multi-system disease with severe and chronic complications
63
What problems can occur with sickle cell disease?
Anaemia - cardiomegaly (high output), low pulse Ox, high WBC Infarction - low O2 -> sickling due to Hb structure changes, pain crises, strokes Infection/sepsis - asplenia from filtering abnormal RBCs, fever a serious sign Splenic sequestration Acute chest - infection/infarction Aplastic crisis - parvovirus B19 infection Iron overload - need for chelation Stem cell transplantation - curative if good donor found Reserved for severe cases
64
What blood results might you get in sickle cell disease?
Hb 55-95g/L Reticulocyte count raised 12% Chronic anaemia, elevated WBC count, increases with vaso-occlusive event to 18-22 (in absence of fever)
65
What should you do with a fever in a sickle cell patient?
``` Serious, patient seek help for any fever Seek source, blood cultures, CXR IV fluids, antipyretics Hospitalise for any pneumonia Outpatient if not toxic, reliable family, follow up of cultures ```
66
What should you do for pain in a sickle cell patient?
Frequent occurrence, treat mild with paracetamol and NSAIDs, patient and family know pain patterns Trust patient and family and treat pain Fluids, pain control - analgesia may need intranasal/IV morphine strong opiates O2 if required +/- IV fluids - dehydration can make pain worse
67
What should you do for acute chest syndrome in a sickle cell patient?
Infection/infarction 25% of premature deaths 25% after surgery Signs - pain crisis, respiratory distress, hypoxia, fever, neurological manifestations Admit - avg around 10 day stay, 2-3 days in ICU Aggressive physiotherapy/spirometry Transfusion (top-up/exchange), IV fluids but avoid overload, O2, antibiotics, incentive spirometry
68
What should you do for a stroke in a sickle cell patient?
Long-term transfusion therapy exchange | Will need chelation for iron overload if transfused > 1 year
69
What should you do for aplastic crisis in a sickle cell patient?
Remember parvovirus B19 - switches off bone marrow RBC production so become severely anaemic quite quickly due to lower RBC lifespan Can be post-op, need good hydration, O2 Low reticulocytes
70
What should you do for splenic sequestration in a sickle cell patient?
Blood can pool in spleen causing hypovolaemia | Fluids - transfuse only to 80 or 90 g/L
71
How can you treat sickle cell disease?
Hydroxycarbamide - Increases HbF which carries O2 at lower O2 tension, good efficacy but teratogenic effects in pregnancy Transfusion programmes - prevent strokes for those at highest risk Stem cell transplants - Patients with multiple strokes, frequent crises, if long term transfusion therapy needed possible GVHD Gene therapies, other new treatments Prevention of crises and infection
72
What is the prognosis of sickle cell disease?
Prevention of crises and infection
73
What is thalassaemia?
Reduced globin chain synthesis
74
Why do babies often present later with beta thalassaemia?
HbF contains no beta chains only alpha and gamma chains
75
How can carriers present and what can be a differential for this?
Mild microcytic hypochromic anaemia | Iron deficiency
76
What is the presentation of alpha thalassaemia?
Loss of 1/2 asymptomatic Loss of 3/4 - HbH Loss of 4/4 death in utero - incompatible with life
77
How does beta thalassaemia minor present?
Asymptomatic Mild anaemia Low MCV Raised Hb A2
78
How does beta thalassaemia major present?
``` Progressive severe anaemia, low MCV, HbF and A2 increased Jaundice Splenomegaly Failure to thrive Skeletal deformity Delayed puberty Death early teens/adulthood Regular blood transfusions mainstay of treatment ```
79
How is beta thalassaemia treated?
``` Genetic counselling, AN diagnosis Regular transfusions Complications of iron overload - liver, heart, pancreas, endocrinopathy - prevented by iron chelation Bone marrow transplant Genetic therapies ```
80
What is transfusion dependent thalassaemia, how does it present?
Progressive severe anaemia requiring frequent transfusions Bone marrow expansion to compensate, extramedullary haematopoiesis Can also get hepato and splenomegaly
81
What is iron overload?
Long term haemolysis and/or transfusions leading to iron overload affecting all organs Ferritin to monitor and imaging Chelation when necessary
82
How does haemolytic anaemia present?
``` Hydrops fetalis Neonatal hyperbilirubinaemia Neonatal ascites Anaemia/failure to thrive Splenomegaly Cholecystitis/gall stones Hyperbilirubinaemia Leg ulcers Aplastic crisis Thromboembolism ```
83
What is G6PD?
X-linked recessive enzymopathy | Glucose-6-phosphate dehydrogenase deficiency
84
How does G6PD present?
Neonatal jaundice Chronic non-spherocytic haemolytic anaemia Intermittent episodes of intravascular haemolysis Acute sever haemolysis
85
What is acute severe haemolysis?
Intravascular haemolysis typically induced by drugs, fava (broad) beans, fever, acidosis
86
How does acute severe haemolysis present?
Haemoglobinuria Rigors Fever Back pain
87
How is acute severe haemolysis treated?
Stop precipitant Transfusion Renal support
88
How common is hereditary spherocytosis and how does it present?
Commonest hereditary anaemia in Europeans 1/5000 Typically autosomal dominant but no family history in 25% cases Heterogeneous - deficiencies of spectrin, ankyrin Clinical effects vary from mild to transfusion dependent - similar within families
89
What is marrow failure?
Impaired production
90
What can cause marrow failure?
Parvovirus Transient erythroblastopenia of childhood Diamond-blackfan anaemia (usually < 1) Inherited/congenital - Fanconi's anaemia, dyskeratosis Acquired aplastic anaemia Bone marrow infiltration - leukaemia/lymphoma, neuroblastoma, other solid tumours, Langerhan's cell histiocytosis, osteopetrosis
91
How does diamond-blackfan anaemia present?
``` Craniofacial abnormalities Thumb abnormalities 10-20% Deafness MSK Renal Cardiac Growth retardation ```
92
What are the symptoms of fanconi anaemia and what is it?
Autosomal/x linked recession - chromosomal fragility Bone marrow failure, leukaemia, solid tumours Absent/abnormal thumbs, cafe au lait patches, growth failure
93
What are the requirements for haemostasis?
Platelets - numbers and function Coagulation factors Vascular integrity
94
What are the two types of ITP?
Acute and chronic - acute often following viral illness and resolving in 1-3 months Acute rarely needs treatment as recover spontaneously in weeks to months Chronic may have associated bleeding eg GI, nose, gingivae
95
In whom does ITP most commonly occur?
Young children
96
Name 2 bleeding disorders
Haemophilia | VWD
97
Name 2 hypercoagulable states
``` Antithrombin Protein C Protein S FVL PT mutation APS ```
98
What is VWD and what is the pathology behind it?
Bleeding disorder caused by abnormalities of vWF - carrier protein for factor VII Can range from almost undetectable to severe bleeding propensity vWF binds on platelets to its specific receptor glycoprotein Ib and acts as an adhesive bridge between the platelets and damaged subendothelium at the site of vascular injury - causes platelets to stick vWF also protects FVIII from degradation
99
What are the different types of vWD and how do they present?
T1 (70-80%) - quantitative deficiency - Autosomal dominant, variable penetrance - Generally mild, can be asymptomatic and vary with time T2A and 2B (15%) have qualitatively abnormal vWF - Autosomal dominant - Moderate severity T3 - most severe, low vWF and FVIIIc in plasma, vWF absent on platelets - Autosomal recessive, consanguinity an issue
100
What is the history of VWD like?
Often mild bleeding - bruising, epistaxis, primary menorrhagia
101
What investigations should you do for VWD?
Clotting screen - may be normal/APTT increased, vWF and FVIII - variably decreased
102
How is VWD treated?
For bleeds/surgery, tranexamic acid, DDAVP usually increases vWF and factor VIII, factor VIII/vWF plasma concentrates for severe
103
What is haemophilia?
Deficiency of factor VII/IX, increased APTT X linked recessive - boys Prolonged bleeding Muscle bleeds and joint bleeds - arthritis and deformity
104
How is haemophilia treated?
Regular infusions of factor VIII/IX - complications of treatment
105
How do neonates with haemophilia present?
FHx Cephalohaematoma/ICH Iatrogenic bleeding Umbilical cord bleeding
106
How do children in early childhood with haemophilia present?
Classically < 2 once mobile Easy bruising/soft tissue haematomas Mouth bleeds - often trauma related Muscle/joint bleeds
107
How do moderate/mild cases of haemophilia present?
May present later following trauma/bleeding with surgery/dental extractions Depends on level of factor
108
What is the most common type of leukaemia in children?
ALL 85% | AML 13%
109
What are the symptoms of ALL?
``` Anaemia Infection Bleeding Systemic symptoms Organ infiltration 2/3 have symptoms for < 4 weeks May have had more non-specific prodrome of several months ```
110
In what age group is ALL most common?
4-7
111
What investigations should you do for ALL?
Blood count - anaemia, WCC up or down, neutropaenia, thrombocytopaenia, blast cells Bone marrow aspirate LP
112
What is the prognosis of ALL and what are the good prognostic factors?
``` Prognosis > 90% cure Good prognostic factors - Age 2-10 - Female - WCC < 50 - No CNS disease ```
113
What is the treatment for ALL?
``` Stratified into risk groups A B C Induction Consolidation and CNS treatment Intensification Maintenance girls 2 years, boys 3 years ```
114
What are the late effects of treatment for leukaemia in children?
``` Psychological Family and social Growth particularly CNS RT Endocrine Puberty Fertility Intellectual Second malignancies ```

Paediatrics (21 decks)

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