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3a > Paediatrics: Haem/Onc > Flashcards

Paediatrics: Haem/Onc Flashcards

1
Q

describe how fetal and adult Hb have a different affinity for oxygen ?

A

HbF has a greater affinity for oxygen at any given partial pressure of oxygen

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2
Q

describe the structure of fetal and adult Hb ? number of subunits ? what subunits ?

A

both have 4 subunits
- HbA: 2 alpha + 2 beta subunits
- HbF: 2 alpha + 2 gamma subunits

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3
Q

when does the product of HbF decrease and HbA increase ? what is the ratio at birth

A

from 32-36 weeks gestation: production of HbF decrease and HbA increases so at birth about 50:50

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4
Q

when is most HbF gone ?

A

by 6 months there is v little HbF (mostly HbA)

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5
Q

Why does sickle cell disease not affect HbF ?

A

genetic abnormality => sickling of beta subunit (=> so no sickling in HbF as no beta unit)

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6
Q

what is anaemia ?

A

low Hb conc secondary to underlying disease

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7
Q

what are some causes of anaemia in infancy ? (5)

A
  • physiologic anaemia of infancy
  • anaemia of prematurity
  • blood loss
  • Haemolysis
  • twin-twin transfusion
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8
Q

what is physiologic anaemia of infancy ? describe the physiology - as a result of what ?

A

there is a normal dip in Hb at around 6-9 weeks
- high oxygen at birth => -ve feedback => reduced EPO from kidneys => low Hb production by bone marrow

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9
Q

name things that would cause anaemia as a result of Haemolysis ? (3)

A
  • haemolytic disease of the the Newborn
  • sickle cell disease
  • hereditary spherocytosis
  • G6PD deficiency
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10
Q

what is haemolytic disease of the newborn ?

A

rhesus antigen incompatibility => Haemolysis, anaemia + increased bilirubin

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11
Q

what is the investigation to do for haemolytic disease of the newborn ?

A

direct Coombs test (DCT) used to check fo immune haemolytic anaemia

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12
Q

causes of anaemia in older children ?

A
  • Iron deficiency
  • Blood loss (mesntruaiton)

less common: sickle cell, thalassaemia, leukaemia

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13
Q

how is anaemia categorised ?

A

based on size (MCV)

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14
Q

causes of microcytic anaemia ? acronym ?

A

TAILS
- Thalassaemia
- Anaemia of chronic disease
- Iron deficiency
- Lead poisoning
- Sideroblastic anaemia

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15
Q

cause of narmacytic anaemia ? acronym ?

A

AAAHH
- Acute blood loss
- Anaemia of chronic disease
- Aplastic anaemia
- Haemolytic anaemia
- hypothyroidism
(and pregnancy ?)

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16
Q

causes of macrocytic anaemia ?

A
  • B12 + folate deficiceiy
  • alcohol
  • Reticulocytosis
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17
Q

symptoms of anaemia specific to iron deficiency ? (2)

A
  • Pica
  • Hair loss
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18
Q

why is iron important ? in the body

A

bone marrow require iron to make haemoglobin

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19
Q

what could cause iron deficiency ? (3)

A
  • dietary insufficiency
  • Loss of iron (heavy mesntruation)
  • inadequate iron absorption (crohns, coeliac)
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20
Q

where is most iron absorbed ? name a condition required for this ?

A
  • Duodenum + jejenum
  • required acid for absorption
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21
Q

how does crohns and coeliac cause iron deficiency ? PPI use ?

A
  • PPI => reduce acid => reduce iron absorption
  • Coeliac + crohns => jejunal/duodenal inflam => inadequate absorption
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22
Q

what is leukaemia ? cancer of what ? leads to unregulated what ?

A

cancer of a particular line of stem cells of bone marrow (bone marrow/blood cancer) => unregulated production of certain types of blood cells (different overproduction depending on which cell lineage involved))

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23
Q

depending on what two factors is leukaemia classified ?

A
  • how rapidly they progress (chronic, acute)
  • the cell line affected (myeloid, lymphoid)
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24
Q

what cells can lymphoid stem cells give rise to ? (2)

A

(WBC)
- Lymphocytes
- Plasma cells

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25
Q

what cells can myeloid stem cells give rise to ? (3)

A
  • WBC
  • RBC
  • Platelet
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26
Q

what types of leukaemia are there ? from most to least common in children ?

A
  • Acute lymphoblastic leukaemia (ALL): most common
  • Acute myeloid leukaemia (AML)
  • Chronic myeloid leukaemia (CML): rare
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27
Q

whats the peak ages for AML and ALL? prognosis for each ?

A

ALL: peaks at 2-3 yrs, 90% cure rate, associated with down syndrome
AML: peaks <2 yrs, prognosis not so good (67%)

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28
Q

What is the pathophysiology of leukaemia ?

A

genetic mutation in one precursor cell in bone marrow => excessive production of a single type of abnormal WBC => suppression of other cell lines = > pancytonpenia => anaemia, leukopenia, thrombocytopenia

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29
Q

what is the term for the abnormal WBC in leukeamia ?

A

lympho/myeloblasts (immature WBC)

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30
Q

leukaemia presentation ?

A

symptoms associated with pancytopenia, non-specific
- Fatigue, Fever, FTT, Weight loss, night sweats
- Pallor
- Petichiea + abnormal bruising
- Lymphadenopathy
- heptospelenomegaly

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31
Q

leaukaemia presentaiton is due to what 3 pain pathological processes ?

A
  • Bone marrow failure (=> pancytopenia)
  • Blast infiltration of other tissues
  • Systemic effects
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32
Q

what causes the systemmic effects associated with leukaemia ? (2)

A
  • Cytokines from leukeamic cells
  • increased plasma viscosity due to high WCC
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33
Q

leukaemia investigations ? how diagnosed ? how staged ?

A
  • FBC: pancytopenia + high number of WBC
  • Bone marrow biopsy + blood film (diagnostic): to distinguish cell type
  • Staging: CXR, CT, LP
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34
Q

blood film shows auer rods. What disease is this ?

A

AML
(leukaemia)
(auger looks red in my mind like the possible myeloid lineage)

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35
Q

leukaemia managment ? immediate and long term ?

A
  • Immediate: any child with high WCC requierd hyperhydration (to prevent hyperviscosity)
  • Ongoing: mainly chemo
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36
Q

complicaitons of chemotherapy ?

A
  • failure of treatment
  • stunted growth
  • Immunodeficiency
  • infertility
  • Peripheral neuropthy
  • Avascular necrosis
  • Anxiety
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37
Q

bruising differential (4)

A
  • ITP
  • Leukaemia
  • trauma
  • NAI
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38
Q

What is ITP ? plus what does it stand for ?

A

Idiopathic thrombocytopenic purpura
- Spontaneous low platelet count causing a purpuric rash

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39
Q

describe the pathophysioloyg of ITP ?

A

type II hypersensitivity reaction: caused by Ab that target + destroy platelets
- Can happen spontaneously or triggered by infection

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40
Q

ITP presentation ? typical age + Hx ?

A

children <10yrs with Hx of recent viral illness
- Bleeding (gums, nosebleeds, menorrhagia), bruising, petechia/purpura rash (caused by bleeding under the skin)

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41
Q

ITP management ? how diagnosed ?

A

most patients will remit spontaneously, depends on how low platelets levels are
- diagnosis: FBC (everything normal except low platelet count)
- Prednisolone, IVIG, blood transfusion

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42
Q

complications of ITP ?

A
  • Chronic ITP
  • Anaemia
  • IC or SAH
  • GI bleeding
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43
Q

What is sickle cell anaemia ? causes what type of anaemia ?

A

genetic condition that causes sickle (crescent) shaped RBC => more fragile => easily destroyed => haemolytic anaemia + prone to sickle cell crisis

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44
Q

what kind of inheritance is sickle cell anaemia ? affecting what subunit ? what chromosome ?

A

autosomal recessive condition affecting gene for beta globin on chromosome 11

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45
Q

What would one abnormal sickle cell variant mean ? 2 ?

A
  • 1 abnormal variant (HbAS): carrier
  • 2 abnormal variants (HbSS): sickle shaped RBC
46
Q

describe sickle cell anaemia relation to malaria ? which genotype ?

A

having one copy of the gene (sickle cell trait) => reduces the severity of malaria => selective advantage

47
Q

what screening is ther for sickle cell anaemia ? (2)

A
  • During pregnancy: women at high risk are offered screening
  • Newborn blood spot screening (at around 5 days)
48
Q

complications of sickle cell anaemia ? most common in kids ?

A
  • anaemia, infection, stroke (most common)
  • sickle cell crisis
  • Acute chest syndrome
49
Q

general managment of sickle cell anaemia ? lifestyle, medical, curative ?

A
  • Avoid triggers for crisis
  • Hydroxycarbonide (stimulate HbF)
  • Bone marrow transplant: can be curative
50
Q

what is sickle cell crisis? triggered by what ? management ?

A

spectrum of acute exacerbations of sickle disease
- Can be triggered by: dehydration, infection, stress, cold weather
- management: supportive

51
Q

what is vaso-occlusive crisis ? with what condition is the associated ?

A

complication of sickle cell disease
- sickle shaped RBC => clog capillaries => distal ischaemia => pain, fever, swelling, priapsim

52
Q

what is acute chest syndrome ? with what condition is this associated ?

A

compliacation of sickle cell disease
- sickled RBC clog vessels supplying the lungs
- Fever, sob, chest pain PLUS pulmonary ifiltrates on CXR

53
Q

What is lymphoma ? cancer of what ?

A

yep of cancer affecting the lymphocytes inside the lymphatic system

54
Q

what sign is lymphoma characterised by ? why is this ?

A

cancerous cells proliferate in lymph nodes => lymphadenopathy

55
Q

what are the 2 types of lymphoma ?

A
  • Hodgkins lymphoma (specific disease)
  • Non-hodgkin lymphoma (all other types)
56
Q

lymphoma definitive diagnosis ?

A

lymph node biopsy

57
Q

what is seen on lymph node biopsy for Hodgkin lymphoma ?

A

Reed-Sternberg cells

58
Q

what is the stage for lymphoma ? describe it

A

Stage 1: one single lymph node
Stage 2: 2 or more lymph nodes on same side of diaphragm
Stage 3: 2 or more lymph nodes on both sides of diaphragm
Stage 4: diffuse involvement of lymph nodes and organs such as the liver and bones

59
Q

What is haemophilia ?

A

severe inherited bleeding disorders

60
Q

what are the two types of haemophilia ? what clotting factor is affected for each ? what inheritance type is each ?

A

VIII, IV, X
- Haemophilia A: deficiency o Factor VIII
- Haemophilia B: deficiency of factor IV
- Both are X-linked recessive inheritance

61
Q

features of haemophilia

A

severe
- can bleed excessively in response to minor trauma
- Risk of bleeding without any trauma

62
Q

how is haemophilia diagnosed ?

A
  • Bleeding scores
  • Coagulation factor assay
  • Genetic testing
63
Q

haemophilia management ?

A

IV infusion of affected clotting factors

64
Q

What is Thalassaemia ? what type of anaemia ? and what MCV ?

A

genetic defect in the protein chains that make up haemoglobin => varying degrees of anaemia
(haemolytic anaemia)
microcytic anaemia

65
Q

what are the 2 types of thalassaemia ? describe what makes each one ?

A
  • Defects in alpha globin chains => alpha thalassaemia
  • Defect in beta globin chains => beta thalassaemia
66
Q

what inheritance is alpha Thalassaemia ? beta ?

A

both are automsoal recessive

67
Q

describe he pathophysiology of thalassaemia ?

A

thalassaemia RBC are more fragile => break down more easily

68
Q

explain how thalassaemia leads to splenomegaly and increased infection risk ?

A
  • Spleen has to destroy all these extra RBC => splenomegaly
  • Bone marrow expands to make more RBC to compensate for anaemia => susceptible to infection
69
Q

signs and symptoms of thalassaemia ?

A

fatigue, pallor, jaundice, gallstones, splenomegaly, pronounced forehead + taller promincnes

70
Q

how is thalssaemia diagnosed ?

A
  • FBC (microcytic anaemia)
  • Haaemoglobin electrophoresis (to diagnose globin abnormalities)
  • DNA testing (for genetic abnormality)
71
Q

thalassaemia management ?

A
  • Monitoring
  • Blood transfusions
  • Bone marrow transplant (can be curative)
72
Q

what is a complication of thalassaemia management ?

A

iron overload
- at risk due to transition + increased gut absorption due to anaemia

73
Q

What is hereditary spherocytosis ? leads to what ? be specific

A

genetic condition where RBC are sphere shaped => fragile + easily destroyed spleen => haemolytic anaemia

74
Q

what inheritance does hereditary spherocytosis display ?

A

autosomal dominant condition

75
Q

hereditary spherocytosis presentation ? (5)

A
  • Jaundice
  • Anaemia
  • Gallstones
  • Splenomegaly
  • Haemolytic crisis
76
Q

what usually triggers a aplastic crisis ? what does this lead to

A

parvovirus can trigger an aplastic crisis
- Reduced bone marrow response during a haemolytic crisis => low RBC)

77
Q

how is hereditary spherocytosis diagnosed ?

A
  • FHx
  • Clinical Hx
  • Spherocytosis on blood flip
78
Q

hereditary spherocytosis management ?

A
  • Folate supplementaiton
  • possible splenectomy
79
Q

What is G6PD deficiency ?

A

genetic condition where there is defect in G6PD enzyme found in all cells in the body

80
Q

how does G6PD deficiency cause a haemolytic anaemia ?

A

G6PD usually prevents cellular damage from reactive oxygen species (ROS)
- RBC have high conc of ROS so highly susceptable to damage

81
Q

what could triggers a crisis in G6PD deficiency ? (3)

A
  • Infection
  • Medication (trimethoprim, nitrofurantoin, sulphonuylureas)
  • Fava beans
82
Q

G6PD deficiency presentation? what on blood film ?

A
  • Neonatal jaundice
  • anaemia
  • Heinz bodes on blood film
83
Q

G6PD deficiency management ?

A

avoid triggers

84
Q

what is von willebrands disease ? what inheritance type ?

A

the most common inherited cause of abnormal and prolonged bleeding
- autosomal dominant

85
Q

von willebrands disease presentation ?

A
  • bleeding gums with brushing
  • epistaxis (nose bleeds)
  • easy bruising
  • Menorrhagia
86
Q

von willebrands disease diagnosis ?

A

Hx + FHx

87
Q

von willebrands disease management ?

A

doesn’t really need daily treatment
- tranexamic acid
- Von Willebrand factor infusion
- mirena coil if menorrhagia is an issue

88
Q

What is the most common type of primary brain tumour in children ?

A

astrocytoma

89
Q

how might a child with a primary brain tumour present ? what kinda causes each symptom

A
  • Headache
  • N&V (due to raised ICP)
  • Behavioural change (due to tumours in frontal lobe)
  • Polyuria/polydypsia (can stop ADH production)
90
Q

What is the diagnostic investigation for a brain tumour ?

A

usually MRI
(if not then contrast enhanced CT)

91
Q

What is the most common form of brain tumour ?

A

secondary
metasteses

92
Q

What is neuroblastoma ? typically which cells ? from where in the body (2) ?

A

paediatric cancer derived from neural crest cells
- typically from adrenal glands or above sympathetic cells

93
Q

in which age group is neuroblastoma most common ?

A

<1 yrs

94
Q

what causes neuroblastoma (pathophys) ?

A

arises from poorly differentiating embryonic cells (blasts) - neural crest cells (derived from developing ectoderm

95
Q

where are most neuroblastoma located ?

A

the adrenal medulla

96
Q

neuroblastoma presentation ? (3)

A
  • Bloating
  • Weight loss
  • Abdo masshow
97
Q

How is neuroblastoma diagnosed ? what test ?

A

urine HVA/VMA

98
Q

what is the most common paediatric primary bone cancer ? second most common ?

A

most common: osteosarcoma
- second: Ewing sarcoma

99
Q

which bone is most commonly affected in osteosarcoma ?

A

(most common primary bone cancer)
- Femur

100
Q

what is thought to cause osteosarcoma ? how does this reflect in the age group most affected ?

A

DNA mutations occur in rapidly dividing osteoblasts (lie during pubertal growth spurts) => malignant transformation
- age: teens/young adults (10-20)

101
Q

osteosarcoma presentation ? most common

A
  • Persistent bone pain (worse at night) (most common)
  • bone swelling
  • palpable mass
  • restricted joint movements
102
Q

how is osteosarcoma diagnosed ? what would indicate urgent assessment ?

A

Xray within 48hrs (for kids with bone pain or swelling)

103
Q

osteosarcoma management ?

A

surgical retention of th lesion (often with a limb amputation)

104
Q

osteosarcoma complicaitons ? (2)

A
  • pathological bone fractures
  • Metastesis
105
Q

What is retinoblastoma ? what age group ?

A

eye cancer that begins in the retina (most commonly affects young children)

106
Q

retinoblastoma presentaiton ?

A
  • squint
  • eye redness
  • eye swelling
  • white reflection reflux
107
Q

retinoblastoma investigations ?

A
  • red reflex
  • USS
  • MRI
108
Q

What could count as b symptoms ? (4)

A
  • Unintentional weight loss
  • Fever
  • Night sweats
  • Lethargy
109
Q

Bilateral cheek erythema with perioral pallor in a child who is a known case of sickle cell disease. What serious complication can occur as a consequence? (what complication and what is it causing)

A

Description of rash is suggestive of Slapped cheek appearance of parvo B19. It can lead to an aplastic crisis.

110
Q

What condition is associated with neuroblastoma ? why is this ?

A
  • hirschpurngs disease
  • both are disease of the neural crest cells
111
Q

What is the commonest cause of death among children with sickle cell disease? (2)

A

stroke or infection

112
Q
A

3a (43 decks)

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