Haematology Flashcards
Causes of anaemia in infancy?
physiological anaemia of infancy (6-9wks)
anaemia of prematurity
blood loss
TTTTS
haemolysis (haemolytic disease of new-born, hereditary spherocytosis, G6PD deficiency)
Anaemia of prematurity is caused by?
less time in utero receiving iron
RBC creation can not keep up with rapid growth
red. erythropoietin levels
blood tests take a significant amount of their circulating blood volume
What is Haemolytic Disease of the newborn?
cause of haemolysis and jaundice in the neonate due to an incompatibility between the rhesus antigens of the mother and the baby
Investigation for haemolytic disease of the newborn?
Direct Coomb’s Test
Causes of anaemia in older children?
iron deficiency anaemia
blood loss (menstruation)
sickle cell anaemia
thalassemia
leukaemia
hereditary spherocytosis
hereditary elliptocytosis
sideroblastic anaemia
helminth infections in developing countries
Causes of microcytic anaemia?
TAILS
thalassemia
anaemia of chronic disease
iron deficiency
lead poisoning
sideroblastic anaemia
Causes of normocytic anaemia?
3 As, 2 Hs
Acute blood loss
Anaemia of chronic disease
Aplastic anaemia
Haemolytic anaemia
Hypothyroidism
Causes of macrocytic anaemia?
megaloblastic:
Vit B12 deficiency
folate deficiency
normoblastic:
alcohol
thyroid (hypothyroid)
reticulocytotis
chronic liver disease
meds (azathioprine)
Presentation of anaemia?
fatigue
dyspnoea
headaches
dizziness
palpitations
worsening of other conditions
pica (IDA)
hair loss (IDA)
pale skin
conjunctival pallor
tachycardia
tachypnoea
koilonychia, angular stomatitis, glossitis (iron deficiency)
jaundice (haemolytic)
bone deformities (thalassemia)
Investigations for anaemia?
FBC (isolated, Hgb, MCV)
blood film
reticulocyte
ferritin
transferrin saturation
B12 and folate
bilirubin
DCT
Causes for iron deficiency anaemia?
inadequate dietary intake
loss of iron (menorrhagia)
inadequate iron absorption (Crohn’s, coeliac)
Mx of iron deficiency anaemia?
treat underlying cause
dietician input
supplementary iron
blood transfusions rarely
Types of leukaemia in children?
ALL most common
AML
CML rarely
Peak ages of leukaemia in children?
ALL 2-3yrs
AML <2yrs
RFs for leukaemia?
radiation exposure (pregnancy)
Down’s syndrome
Klinefelter syndrome
Noonan syndrome
Fanconi’s anaemia
Presentation of leukaemia?
non-specific
persistent fatigue
unexplained fever
failure to thrive
weight loss
night sweats
pallor
petechiae and abnormal bruising
unexplained bleeding
abdo pain
lymphadenopathy
bone or joint pain
hepatosplenomegaly
Diagnosis of leukaemia?
refer any child with unexplained petechiae or hepatomegaly for immediate assessment
FBC within 48hrs
FBC (anaemia, leukopenia, thrombocytopenia)
blood film (blast cells)
BM biopsy
LN biopsy
CXR
CT
LP
genetic analysis of abnormal cells
Mx of leukaemia?
MDT
chemotherapy primarily
radiation
BM transplant
Sx intervention
Complications of chemotherapy?
failure to treat leukaemia
stunted growth and development
immunodeficiency and incr. infections
neurotoxicity
infertility
secondary malignancy
cardiotoxicity
Prognosis for leukaemia?
ALL overall cure rate 80%
AML not as good
What is immune thrombocytopenic purpura?
ITP
condition characterised by spontaneous thrombocytopenia resulting in a non-blanching purpuric rash
type II hypersensitivity reaction
can be idiopathic or triggered by recent viral infection
Presentation of ITP?
children <10
usually Hx of viral illness
bleeding, gums or epistaxis
bruising
petechial or purpuric rash
Investigations for ITP?
FBC shows isolated thrombocytopenia
exclude HIT and leukaemias
Mx of ITP?
usually no mx is required
if active bleeding or platelets <10:
prednisolone
IVIG
blood transfusions
platelet transfusions not effective long-term
Advice:
avoid contact sports
avoid injections
avoid NSAIDs and blood thinners
advice on epistaxis
safety netting
Complications of ITP?
chronic ITP
anaemia
intracranial and SAH
GI bleeding
What is sickle cell anaemia?
AR genetic condition affecting RBCs, in which they are shaped like a crescent or sickle
leads to haemolytic anaemia
predisposed to sickle cell crises
Sickle cell relation to malaria?
selective advantage to having sickle cell trait in countries prone to malaria as it reduces the severity
thus sickle cell anaemia is more common in these populations
Screening for sickle cell anaemia?
pregnant women at high risk are offered prenatal testing
Complications of sickle cell anaemia?
anaemia
incr. risk of infection
CKD
sickle cell crises
acute chest syndrome
stroke
avascular necrosis
pulmonary HTN
gallstones
priapism in males
Types of sickle cell crises?
Vaso-occlusive crises
splenic sequestration crises
aplastic crises
acute chest syndrome
Mx of sickle cell crises?
low threshold for admission
treat underlying cause
keep warm
ensure hydration
analgesia
What is a vaso-occlusive crisis?
most common sickle cell crisis
‘painful crisis’
caused by sickle cell shaped RBCs clogging capillaries and causing distal ischaemia
typically affects hands, feet or penis (priapism)
What is a splenic sequestration crisis?
caused by sickle shaped RBCs blocking blood flow within the spleen
acute splenomegaly
severe anaemia and hypovolaemic shock
can lead to splenic infarction, leaving patients with hyposplenism and thus susceptible to infections
splenectomy may be considered in recurrent cases
What is aplastic crisis?
temporary absence of creation of new RBCs
usually triggered by parvovirus B19
leads to aplastic anaemia
typically resolves spontaneously within a week
What is acute chest syndrome?
when the vessels supplying the lungs become clogged with RBCs
presents with fever, shortness of breath, chest pain, cough and hypoxia
pulmonary infiltrates on CXR
supportive management
Management of sickle cell anaemia?
MDT
avoid triggers for crises
vaccinations
antibiotic prophylaxis, typically penicillin V
hydroxycarbamide (stimulates HbF)
crizanluzimab (reduces incidence of vaso-occlusive crisis)
blood transfusions
BM transplant (curative)
Diagnosis of sickle cell anaemia?
Hgb electrophoresis