PAEDIATRICS
HAEMATOLOGY
PromptDDx for a toddler with anaemia
ResponseIron deficiency - microcytic hypochromic, target cells, pencil cells
Acute lukaemia - Blasts on film
Aplastic anaemia - Low reticulocyte coung
GIT bleed - Elevated reticulocyte count
Hamoglobinopathy - Thalassemia - nuclearted RBC’s, microcytes
Congenital haemolysis - Sickle cell disease, sickle cells
Acquired haemolysis - HUS - schistocytes red cell fragments
Abnormal red cells - spherocytesosis = spherocytes
Acute lukaemia - Blasts on film
Aplastic anaemia - Low reticulocyte coung
GIT bleed - Elevated reticulocyte count
Hamoglobinopathy - Thalassemia - nuclearted RBC’s, microcytes
Congenital haemolysis - Sickle cell disease, sickle cells
Acquired haemolysis - HUS - schistocytes red cell fragments
Abnormal red cells - spherocytesosis = spherocytes
PromptPale asian 12 month old with dark urine DDx
ResponseHaemolytic uraemic syndrome
Haemolytic anaemia
Infection with dehydration
Intussusception
Hepatitis/cholecystitis/porphyria/dengue/malaria
Haemolytic anaemia
Infection with dehydration
Intussusception
Hepatitis/cholecystitis/porphyria/dengue/malaria
PromptInvestigations findings in HAEMOLYTIC anaemia
ResponseBlood count and flim - polychromasia, bite cells
Bilirubin - increased indirect
Haptoglobin - decreased
Reticulocyte count - increased
LDH - elevated
Coags
Urine + Micro - haematuria, urinary haemosiderin (severe haemolysis), Casts = Renal
G6PD level - needs to be done when WELL ie. no role in acute illness
Bilirubin - increased indirect
Haptoglobin - decreased
Reticulocyte count - increased
LDH - elevated
Coags
Urine + Micro - haematuria, urinary haemosiderin (severe haemolysis), Casts = Renal
G6PD level - needs to be done when WELL ie. no role in acute illness
PromptG6PD
Response
PromptTriggers of G6PD
ResponseDrugs - antimalarials, Trimethoprim, cipro, aspirin
Infection
Napthaline blue (mothballs)
Fava beans
Ketoacidosis
Infection
Napthaline blue (mothballs)
Fava beans
Ketoacidosis
PromptManagement
ResponseCease triggering agent
Admission
Supportive care - IV fluids (Reduce ketoacidosis/oxidative stress)
Oral folate
Unlikely to require blood transfusion unless severe
Admission
Supportive care - IV fluids (Reduce ketoacidosis/oxidative stress)
Oral folate
Unlikely to require blood transfusion unless severe
PromptHAEMOLYTIC URAEMIC SYNDROME
Remember that kid who’s mum DAMA’d from that other place and the reg called you and was like “they fine, just want 2nd opinion”
Then the only blood that didn’t clot was the CRP of 270
And then the haem reg called you at 5AM cos they came in without being called cos it so interesting.
Remember that kid who’s mum DAMA’d from that other place and the reg called you and was like “they fine, just want 2nd opinion”
Then the only blood that didn’t clot was the CRP of 270
And then the haem reg called you at 5AM cos they came in without being called cos it so interesting.
ResponseRecent gastrointestinal illness (5-10 days before onset of rash) - E. Coli or Shiga - Usually has bloody diarrhoea
Abdominal pain
Hypertension
Focal neurology
FAT RN = HUS/TTP
Fevers
Anaemia
Thrombocytopaenia
Renal failure
Neurologic symptoms
Investigations - HAEMOLYSIS
Anaemia, elevated LDH, Decreased haptoglobin, schistocytes
Low platelets
Elevated Creatanine/urea
Blood culture - ?causative organism (E.Coli 0157:H7)
Management:
Transfuse >80
Platelets (contraindicated in general) - avoid unless significant bleeding or surgery required
Fluids - judicious - risk of overload but may be dehydrated.
Electrolytes - Manage hyperkalaemia
Dialysis - anuric/oliguric/severe electrolyte imbalance/metabolic acidosis/fluid overload refractory to treatment
BP management - hypertension
Plasma exchange
Treat neurologic dysfunction - Seizures, Eculizumab improves neurologic dysfunction
Antibiotics may worsen toxin production.
- E. Coli (STEC) - 90%
- Streptococcus pneumoniae - 5-10%
- HIV, influenza
Abdominal pain
Hypertension
Focal neurology
FAT RN = HUS/TTP
Fevers
Anaemia
Thrombocytopaenia
Renal failure
Neurologic symptoms
Investigations - HAEMOLYSIS
Anaemia, elevated LDH, Decreased haptoglobin, schistocytes
Low platelets
Elevated Creatanine/urea
Blood culture - ?causative organism (E.Coli 0157:H7)
Management:
Transfuse >80
Platelets (contraindicated in general) - avoid unless significant bleeding or surgery required
Fluids - judicious - risk of overload but may be dehydrated.
Electrolytes - Manage hyperkalaemia
Dialysis - anuric/oliguric/severe electrolyte imbalance/metabolic acidosis/fluid overload refractory to treatment
BP management - hypertension
Plasma exchange
Treat neurologic dysfunction - Seizures, Eculizumab improves neurologic dysfunction
Antibiotics may worsen toxin production.
PromptTHROMBOCYTOPAENIA
Response
PromptITP
ResponseHx: Recent viral illness, otherwise well child, normal vitals on examination
Ix: ISOLATED thrombocytopaenia (60% have anti-platelet antibodies - anti-glycoprotein IgG)
Ix: ISOLATED thrombocytopaenia (60% have anti-platelet antibodies - anti-glycoprotein IgG)
PromptACUTE LUKAEMIA
Response
PromptClinical presentation of acute lukaemia
ResponseHx Constitutional symptoms, bone pain, fever, lethargy, night sweats
Splenomegaly
Ix
FBC - Leukocytosis(even in the hundreds!), anemia/thrombocytopaenia, blasts
LDH elevated
No evidence of haemolysis (haptoglobin normal, no schistocytes)
ALL ~ 80% of cases, AML ~20% in kids
Splenomegaly
Ix
FBC - Leukocytosis(even in the hundreds!), anemia/thrombocytopaenia, blasts
LDH elevated
No evidence of haemolysis (haptoglobin normal, no schistocytes)
ALL ~ 80% of cases, AML ~20% in kids
PromptDifferentials for pancytopaenia
ResponseAcquired aplastic anaemia
Drugs - NSAID’s, Anticonvulsants (Carbemazepine), Antibiotics
Infection - EBV/HIV
Immune - SLE
Bone marrow suppression from other causes/infiltration
Lymphoma
Drugs - NSAID’s, Anticonvulsants (Carbemazepine), Antibiotics
Infection - EBV/HIV
Immune - SLE
Bone marrow suppression from other causes/infiltration
Lymphoma
PromptDIC - list clinical features
ResponseHx - Rapid onset of severe illness
Febrile
Sings of distributive shock (ALOC, vital signs deranged) - poorly perfused/mottled
Echymosis
Ix
Low fibrinogen, Low platelets - High PT, D dimer.
Blood culture/LP may be positive
Febrile
Sings of distributive shock (ALOC, vital signs deranged) - poorly perfused/mottled
Echymosis
Ix
Low fibrinogen, Low platelets - High PT, D dimer.
Blood culture/LP may be positive