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Paediatrics > Haematology > Flashcards

Haematology Flashcards

1
Q

acute lymphoblastic leukaemia prevalence

A

most common cancer in paeds
2-5 yrs
males more
trisomy 21 increases

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2
Q

acute lymphoblastic leukaemia pathop

A

infection, genetic + environment
disruptions in regulation and proliferation of lymphoid precursor cells in bone marrow…excessive production of blast cells, drop in R.B.C, W.B.C and platelets

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3
Q

history ALL

A

anaemia - lethargy
thrombocytopenia - bruising
leukopenia - infection
bone pain
weight loss
malaise
headache
seizures

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4
Q

examination ALL

A

pale
excess bruising/bleeding
lymphadenopathy
hepatosplenomegaly

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5
Q

ddx ALL

A

immune thrombocytopenia, immune deficiency, reactive lymphodenopathy

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6
Q

investigations ALL

A

FBC - pancytopenia, anaemia, thrombocytopenia
blood film - blast cells
CXR - mediastinal mass
bone marrow aspirate
LP

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7
Q

risk scoring ALL

A

1-10 yrs - better prognosis
WCC>50 - poorer
females - better
blasts within CSF - poorer
morphology and cytology - effects prognosis

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8
Q

immediate management ALL

A

resus - high WCC requires hyperhydration to prevent hyperviscosity
if mediastinus mass - steroids
if infection - abx

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9
Q

definitive ALL

A

chemo - IV, PO, intrathecally
blood products and prophylactic anti-fungal

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10
Q

prognosis ALL

A

over 90% survive
follow up for 5 years

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11
Q

complications ALL

A

infertility
avascular necrosis
peripheral neuropathy
anxiety

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12
Q

2 types of acute leukaemia

A

acute lymphoblastic leukaemia (maj)
acute myeloid leukaemia

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13
Q

acute leukaemia pathophysiology

A

blood stem cell - can be lymphoid stem cell or myeloid stem cell
lymphoid - WBC
myeloid - RBC, WBC, platelets

genetic mutations in blood progenitor cells…causes developmental arrest…causing immature cells (myeloblasts) - less healthy cells…infection, anaemia, bleeding

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14
Q

childhood AML diagnosis

A

when bone marrow has 20% or more blasts

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15
Q

classification AML

A

FAB classification
MO-M7
morphological and histochemical characteristics

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16
Q

leukaemia cells spread

A

to CNS, skin and gyms
can form solid tumour called chloroma or granylocytic sarcoma

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17
Q

risk factors AML

A

Down’s syndrome
Li-Fraumeni Syndrome
Aplastic anaemia
Myelodysplasia
Affected sibling

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18
Q

3 main pathological processes acute leukaemia

A

bone marrow failure due to infiltration by blasts
blast infiltration of other tissues
systemic effects of cytokines released by leukaemic cells and of increased plasma viscosity (leucostasis)

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19
Q

acute leukaemia investigations

A

FBC- pancytopenia, neutropenic
blood film - blasts, atypical cells, leukoerythroblastic features
CXR - lymph gland enlargement
bone marrow aspirate - definitive diagnosis
LP
biopsy of chloroma

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20
Q

acute leukaemia bone marrow exam

A

aspirate - response to chemo and risk of relapse
light microscopy - classification
immunophenotyping - identify subtype

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21
Q

initial management acute leukaemia

A

IV chemo - induction (2 cycles 4 weeks apart) and post remission consolidation

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22
Q

long term managent acute leukaemia

A

post remission therapy - further chemo
and/or allogeneic haematopoeitic stem cell transplantation
bone marrow transplant - if chemo not work or relapses

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23
Q

complications acute leukaemia

A

neutropenic sepsis
myelosuppression
N+V
mucositis
hair loss

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24
Q

chemo agent and side effect AML

A

Doxorubicin Cardiotoxicitiy
Vincristine Peripheral neuropathy
Cyclophosphamide Reduced fertility
Cytarabine Hepatotoxicity

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25
Q

prognosis AML

A

5 year survival of 66%
if poor cytogenic features or response to therapy - <20% cured

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26
Q

haemophilia genetics

A

x linked recessive
more common in males

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27
Q

haemophilia A v B

A

A - factor VIII (more common)
B - factor IX

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28
Q

pathophysiology haemophilia

A

factor VIII is one of main clotting factors that contibutes to production of thrombin via intrinsic pathway. The intrinsic pathway is activated in response to endothelial collagen exposure…therefore lack of favot VIII…deficient in thrombin…results in insufficient production of fibrin for clot stabilisation

29
Q

severity of haemophilia A

A

depends on plasma conc of factor VIII
if severe, may have no measurable factor in blood

30
Q

risk factors haemophilia

A

male
family hx

31
Q

hx haemophilia

A

prolonged, unexplained bleeding
recurrent episodes
spontaneous bleeding
joint pain, swelling, reduced ROM - haemarthrosis

32
Q

examination haemophilia

A

active bleeding
pallor
bruising
haemarthrosis

33
Q

haemophilia ddx

A

non accidental injury (social care)
von willebrand disease
vit K deficiency
ALL

34
Q

investigations haemophilia

A

FBC - anaemia
coagulation screen - prolonged APTT, normal PT, factor will be low
CT head, joint aspiration - additional

35
Q

management haemophilia

A

treating bleeds - in: one off infusion of factor
prophylaxis - regular IV infusion of factor
education
avoid contact sports
good dental hygeine

36
Q

complications haemophilia

A

chronic arthopathy
development of factor VIII or IX inhibitors
transfusion related complications

37
Q

lymphoma types

A

hodgkin and non hodgkin lymphoma

38
Q

epidemiology lymphoma

A

over 10% of childhood cancers
on boys
older children

39
Q

pathophysiology lymphoma

A

multifactorial
adults - obesity, smoking, alcohol

40
Q

history lymphoma

A

EMV
immunosuppresed - transplant, previous cancers
weight loss, night sweats, fevers

41
Q

examination lymphoma

A

non tender lymphadenopathy
if mediastinus - wheeze, difficulty breathing
SVC obstruction

42
Q

ddx lymphoma

A

reactive lymphadenopathy
leukaemia
met malignancy

43
Q

investigations lymphoma

A

FBC
UE - tumour lysis syndrome can cocur before tx begins
LDH - elevated
USS of area and nodes and biopsy
CXR - mediastinal nodes
full body CT
lymph node biopsy

44
Q

risk scoring lymphoma

A

1 - single group of nodes or organ
2 - 2 or more groups of nodes or organs on same side of diaphragm
3 - in lymph nodes or organs on both sides
4 - diffuse involvement of nodes and organs such as liver and bones
B added to stage if B symptoms present - weight loss, night swats, fevers

45
Q

immediate management haemophilia

A

airway compromise
high dose steroids
SVCO may require stenting of veins
hyperhydration in tumour lysis syndrome

46
Q

definitive management lymphoma

A

chemo and possible radiotherapy, depending on stage

47
Q

prognosis lymphoma

A

majority recover
hodgkin’s more favourable than non

48
Q

complications lymphoma

A

tumour lysis syndrome, which is seen when rapid lysis of tumour cells causes release of large amounts of phosphorus, potassium and calcium leading to potential kidney damage. This is most likely to occur when chemotherapy is first commenced, but may occur beforehand
neurotropenia
alopecia
sub fertility

49
Q

genetics sickle cell

A

a mutation in the gene encoding the haemoglobin subunit β, encoded by the beta globin gene. It is inherited in an autosomal recessive

50
Q

epidemiology sickle cell

A

1 in 2,449 in the UK with a carrier risk of 1 in 89
marked variation by region
malarial hypothesis - survival advantage against malaria
increasing prevalence of HbS allele in high income countries

51
Q

pathophysiology sickle cell

A

The HbS allele results from a single nucleic acid substitution from GAG to GTG in the beta globin gene. This causes glutamic acid to be substituted with valine. Individuals with one HbS allele (HbAS) are carriers while those who are homozygous for the HbS allele (HbSS) have sickle cell disease. This is the most common type of sickle cell disease, however it is also possible to have one HbS allele and a different faulty beta globin allele other than HbS (such as β-thalassaemia, written as HbSβ-thalassaemia
This makes it prone to sickling where it becomes rigid and distorts into crescent shape. in the deoxygenated state, HbS tetramers bind to each other and polymerise…vaso-occlusion and chronic haemolysis

52
Q

risk factors sickle cell

A

black african, black caribbean - 1 in 200 affected, 1 in 10 carriers
next common - asian
lowest - white

53
Q

sickle cells history

A

intermittent vaso-occlusive crises - severely painful, precipitated by hypoxia, infection, exercise, dehydration, acidosis
dactylitis
family history

54
Q

examination sickle cell

A

Pallor, lethargy – due to anaemia
Jaundice – due to haemolysis
Fever
Tachycardia, tachypnoea
Digital redness, swelling and pain

55
Q

ddx sickle cell

A

gout
acute abdomen

56
Q

vaso occulsive crisis >7 days

A

consider osteomyelitis or avascular necrosis

57
Q

investigations sickle cell

A

haemoglobin electrophoresis - HbA absence
high performance liquid chromatography
blood film - nucleated red cells, howell jolly bodies, sickle shaped
FBC and reticulocyte count
iron studies
X ray of long bones
CXR - acute chest syndrome

58
Q

screening sickle cell

A

antenatal screening -
heel prick test

59
Q

acute pain crisis sickle cell

A

analgesia
fluid
oxygen
abx if infection

60
Q

prophylactic tx sickle cell

A

Hydroxycarbamide – reduces frequency of painful crises
Novel and experimental treatments – including L-glutamine, crizanlizumab and Voxelotor
Regular blood transfusions – targeted to maintain HbS <30%

61
Q

complications sickle cell

A

increased susceptibility to infection - pneumococcal and salmonellavac

62
Q

vaccinations sickle cell

A

seasonal influenza vaccines – annual
pneumococcal vaccines – every 5 years
hepatitis B vaccine – if receiving regular blood transfusions
other vaccines, such as meningococcal C or haemophilus influenza type B vaccine – may be advisable if they haven’t had them before

63
Q

complications sickle cell

A

pulmonary hypertension
leg ulcers
priapism
chronic kidney disease
large-artery ischemic stroke

64
Q

aplastic crisis

A

result from infection with parvovirus B19

65
Q

prognosis sickle cell

A

median life expectancy is 58 years for HbSS and 66 years for HbSC and HbSbeta-thalassaemia
life expectancy increasing
infection or stroke - most common mortality
acute chest syndrome - adults

66
Q

poor prognostic factors sickle cell

A

earlier first presentation
high frequency of vaso-occlusive crises

67
Q
A
68
Q
A

Paediatrics (17 decks)

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