Haematology Flashcards
What are the risk factors for iron deficiency anaemia in children?
Preterm Low birth weight Multiple pregnancy Exclusive breastfeeding>6m Female Adolescent Poverty
What are the causes of iron deficiency anaemia?
Malabsorption: IBD, Coeliac Malnutrition Bleeding Drugs: NSAIDs, Steroids Inc demand: Growth spurt
What are the signs & symptoms of iron deficiency anaemia?
Asymptomatic Mood, cognitive, psychomotor changes Fatigue SOB Pica Pallor (conjunctival) Angular Stomatitis Koilonychia
How is iron deficiency anaemia investigated?
Bloods: Hb, MCV, MCHC, Clotting, Ferritin, B12 & Folate, CRP
How is iron deficiency anaemia treated?
1.5-2mg/kg Ferrous Sulphate daily
Response within 5-10days
Continue for 3m after Hb has normalised
What is Henoch-Schonlein purpura?
IgA mediated small vessel vasculitis
What are the signs & symptoms of Henoch-Schonlein purpura?
Usually after URTI
TRIAD: Arthritis (large joints), Abdo pain, palpable papular purpuric rash over legs/buttocks
GI: Melena, haematemesis, intussusception, appendicitis, perforation
Renal: Glomerulonephritis, IgA nephritis, proteinuria
What are the investigations for Henoch-Schonlein purpura?
Bloods: FBC, U&E
Urinalysis: Haematuria
Renal biopsy if signs
What is the management of Henoch-Schonlein purpura?
Most benign & resolve within 6weeks
NSAIDs: Arthritis
Steroids: Arthritis & GI
What is haemolytic disease?
Mother is rhesus -ve
Baby rhesus +ve
Mother previously sensitised
Foetal blood crossing placenta causes immune response
Maternal IgG attacks foetal RBC cells causing haemolysis
How does haemolytic disease present?
Antenatal: Anaemia on doppler, hydros fetalis
Postnatal: Hydrops fetalis, early jaundice, kernicterus (neuro damage), coagulopathy, hypotonia, hepatosplenomegaly, blueberry muffin
Anaemia
How is haemolytic disease investigated?
Maternal: Rh -ve, inc anti-Rh titre Cord: Dec Hb & platelets, inc reticulocytes coomb's test neonatal blood fr croup & titre SBR: 4hrly, glucose, Hb
How is haemolytic disease treated?
Supervision +/Transfusion After birth check cord bloods Phototherapy Supportive therapy Oral folic acid: 250mcg/kg/day 6mnths Perform audiology test
Prophylaxis: Anti-D IgG.
What is haemophilia?
Congenital bleeding disorder Due to defective production of coagulation factors Sex-linked recessive inheritance Types A & B Type A: Factor 8 Type B: Christmas disease, factor 9
What are the signs & symptoms of haemophilia?
A & B indistinguishable
Rarely presents in neonates
Mild: Bleed after trauma/ surgery
Mod: Persistent bleeding after venepuncture, epistaxis, haematuria
Severe: Neonatal bleeding, IC haemorrhage, sport bleeding into joints & muscles
Easy bruising
Haematuria
Joints: Localised tenderness, warmth, swelling, limited RoM, degeneration
How is haemophilia investigated?
Bloods: INR(n), APTT(i), factor 8(d), bleeding time & von Willebrand (n)
CT head: IC bleed
USS/ joint x-ray
Type B: x6 less common than type A
How is haemophilia managed?
Major bleed: Factor 8/9 Emergency: FFP, Tranexamic acid Analgesia: NOT NSAIDs (dec platelets) Minor bleed: Pressure Mouth bleed: Oral Tranexamic acid AVOID: IM injections inc Vit K at birth Other: Rest, ice, limb splinting, physio
PROPHYLAXIS: IV con injections 3/week in severe disease
What are the complications of haemophilia?
Chronic arthropathy
Blood bourne virus transmission
Factor 8/9 inhibitor development
What is the pathophysiology of sickle cell anaemia?
Autosomal recessive in homogenous sickle haemoglobin
Glutamine becomes Valine on beta global chain = HbS
Less soluble, high viscosity
Polymerises when deoxygenated
Can’t flow through small vessels (hypoxia & ischaemia)
What are the features of sickle cell anaemia?
Infancy: Dactylitis, pallor, infections, hepatosplenomegaly
Young children: Infection, vaso-occlusion, stroke, UA obstruction
Older children: Infection, vaso-occlusion
What are the complications of sickle cell anaemia?
Vaso-occlusion crisis: Excrutiating pain in bones/joints, hands/feet (dactylitis), precipitated by infections, cold, hypoxia
Mx: Analgesia, warmth, rehydration
Acute chest: SOB, chest pain, cough, yellow sputum
Sequestration: Sickled blood in spleen/lung/liver, can be fatal
Avascular necrosis: Hip
Renal impairment: Hyposthenuria
Priapism: Painful erectile dysfunction
Retinopathy: Small vessel occlusion
Crisis:
Stroke: 5-10yr, asymptomatic infarcts
How is sickle cell anaemia investigated?
Clinical suspicion Blood film Haematology: Hb 5-9 Hb electrophoresis: Sickled cells Prophylaxis: Routine screening & heel prick test
How is sickle cell anaemia managed?
Hydration 150% normal Analgesia: Paracetamol, NSAIDs, Opiates Abx: If pyrexial- Cefotaxime. Add Clarithromycin if respiratory signs Oral folate Oxygen: Sats >95% Vaccinations Blood products: Crises BM transplant
What is thalassaemia?
Types: α-thalassaemia, β-thalassaemia major & minor
Autosomal Recessive
Decreased/absence of synthesis of 1 or 2 (α or β) chains that form adult human haemoglobin (HbA, α2/β2)
Results in reduced Hb in RBCs and anaemia.
HbA, the most common form of adult Hb, has 2 α & 2 β chains.
Fetal (HbF) has 2 α & 2 γ components (predominant type of Hb before birth).
