haematology Flashcards
Erythroblastosis fetalis / haemolytic disease of the newborn / rhesus disease signs and symproms
aneamia
hepatosplenomegaly
jaundice
CCF
Erythroblastosis fetalis / haemolytic disease of the newborn / rhesus disease management
anti D immunoglobulin to Rh -ve mothers
intrauterine transfusion of affected fetuses
Erythroblastosis fetalis / haemolytic disease of the newborn / rhesus disease DD
thalassaemia
infection - toxoplasmosis, CMV
Erythroblastosis fetalis / haemolytic disease of the newborn / rhesus disease diagnosis
coombs test - Rh -ve mothers tested for D antibodies
kleihauer test
Anaemia of Prematurity cause
Low birth weight infants
poor erythropoietin response
Anaemia of Prematurity signs and symptoms
apnoea poor weight gain pallor decreased activity tachycardia
iron deficiency anaemia causes
Poor intake + increased requirement.
blood loss
malabsorption
iron deficiency anaemia presentation
pallor irritability anorexia when Hb<50, tachycardia splenomegaly if recurrent, suspect bleeding
iron deficiency anaemia management
oral iron - ferrous fumarate for 3-6 months
constipation common
Sickle Cell Disease management
Prophylactic penicillin Pneumococcal, influenza, meningococcal vaccines high risk of sepsis pain management - paracetamol, NSAIDS, O2
Sickle Cell Disease complications
acute anaemia (aplastic crisis) vaso-occlusive crisis - cold, exercise, infection cardiomegaly (high output) strokes sepsis asplenia - from filtering abnormal RBC splenomegaly
Sickle Cell Disease aplastic crisis cause
parovirus B19
Sickle Cell Disease stroke management
long term transfusion therapy
chelation for iron overload if transfusion long term
β Thalassaemia Minor signs
asymptomatic
mild anaemia
low MCV
Raised Hb A2
β Thalassaemia Major signs and symptoms
Progressive Severe Anaemia low MCV, Hb F and A2 increased Jaundice Splenomegaly Failure to thrive
β Thalassaemia management
Genetic Counselling
Regular blood transfusion
G6PD presentation
Neonatal jaundice
Chronic non-spherocytic haemolytic anaemia
Intermittent episodes of intravascular haemolysis
also dark urine (urobillinogen and haemoglobulinuria)
G6PD what is it
RBC lack G6PD so exposed to oxidant induced haemolysis
hereditary spherocytosis
RBC lose part of membrane, reducing surface:volume. cells become spheroidal leads to destruction
hereditary spherocytosis presentation
autosomal dominant
jaundice
anaemia
splenomegaly
Diamond Blackfan anaemia presentation
physical anomalies- at least 50% • Cranio-facial • thumb 10-20% • Deafness • Musculoskeletal
Immune / Idiopathic Thrombocytopaenic Purpura (ITP)
platelet destruction by Ig G autoantibodes
usually post viral
acute brusing, looks dramatic rarely dangerous
von willebrand disease causes
deficiency in vwf, a carrier protein for factor 8
von willebrand disease management
Tranexamic acid
haemophilia A
Deficiency of Factor VIII
haemophilia B
Deficiency of Factor IX, ↑APTT
haemophilia A/B presentation
X linked recessive- boys Prolonged bleeding Muscle bleeds Joint bleeds > arthritis and deformity Treatment Factor VIII/IX
acute lymphoblastic leukaemia presentation
peak age 4-7 normochromic normocytic anaemia (pallor) thrombocytopenia (bleeding) leukopenia (infections) fatigue weight loss painless lumps on neck (lymphadenopathy)
acute lymphoblastic leukaemia diagnosis
bone marrow biopsy
tdt and cd10 surface markers in immunophenotyping
CT/MRI and CXR
acute lymphoblastic leukaemia complications
neutropenic sepsis - tazocin and gentamicin
poor growth
second malignancies
acute lymphoblastic leukaemia management
Induction - vincristine, dexamethasone Consolidation and CNS treatment (if known CNS disease) Intensification Maintenance (radio / chemotherapy)
fanconi anaemia
autosomal recessive
most common cause of inherited aplastic anaemia
high risk of leukaemia
bone marrow transplant needed
anaemia range
<110g/L
microcytic hypochromic
normocytic normochromic
macrocytic
ranges and causes
MCV <70 iron def, thalassaemia
MCV 81-97 haemolysis (sickle cell)
MCV >100 B12 def (pernicious/malabsorption), folate def (malnutrition)
effects of ALL treatment
fertility growth psychological puberty intellectual
ALL good prognostic factors
Age 2-10
Female
WCC<50
No CNS disease
prognosis 80%
