Haematology Flashcards
Coagulation disorder suspected but coagulation profile is normal - think of
Factor XIII deficiency
What does factor XIII do?
Makes crosslinks between fibrin dimers, so stabilises the clot
What kind of bleeding do you get with factor XIII deficiency?
Bleeding a few days after the injury
How is factor XIII deficiency inherited?
Autosomal recessive
How is factor XIII deficiency managed?
Either with factor XIII supplementation or cryoprecipitate infusions
Which is the most severe vWF disease?
Type 3 (autosomal recessive)
What does von willebrand factor do?
Acts as the transporter protein for factor VII and also helps platelet binding at endothelial sites
How do you test for vWF?
Partial thromboplastin time often prolonged; ristocetin cofactor activity test measures plasma vWF function
What are the coagulation screen abnormalities in factor XII deficiency?
Prolonged partial thromboplastin time
How is factor XII deficiency inherited?
Autosomal recessive
What causes allergic transfusion reactions?
Interaction between donor plasma proteins and recipient IgE antibodies
What haematological abnormality does phenytoin cause?
Folate deficiency -> macrocytosis
What haematological abnormality does hydroxyurea cause?
Macrocytosis with megaloblastic anaemia
What haematological abnormality can penicillin cause?
Haemolytic anaemia
What haematological abnormality can cotrimoxazole cause?
Aplastic anaemia
Which disease: pancytopenia, short stature, cafe au lait spots, squint, limb anomalies, mental retardation, chromosome fragility, predisposition to leukaemia
Fanconi anaemia
How is Fanconi anaemia inherited?
Autosomal recessive
What haematological abnormalities are seen in Diamond-Blackfan syndrome?
Normo- or microcytic anaemia with low reticulocyte count
Iron studies in iron deficiency anaemia
Low serum iron High TIBC Decrease in transferrin saturation Increase in serum soluble transferrin receptors Low ferritin Increased zinc protoporphyrin/haem ratio Decreased serum ferritin
Iron studies in chronic disease
Low serum iron
low TIBC
Normal transferrin saturation
Normal/increased ferritin
Iron studies in beta thalassaemia
Normal/increased serum iron
Normal TIBC
Normal transferrin saturation
Normal/increased ferritin
Iron studies in sideroblastic anaemia
Normal/high serum iron
Normal/low TIBC
100% transferrin saturation
Increased ferritin
X-ray features of osteosarcoma
Intramedullary sclerosis
‘Sunburst’ periosteal reaction
Colman’s triangle (raised periosteum away from bone)
Which kind of anaemia does B12 deficiency present with
Microcytic anaemia, neutrophil hypersegmentation
What do you see in blood during acute haemolysis?
Decreased plasma haptoglobin
Increased plasma methane albumin
Haemoglobinuria
Heinz bodies
Common causes of cold induced haemolytic anaemia?
Infectious mononucleosis, mycoplasma, CMV infection
Which antibodies indue cold haemolytic anaemia?
IgM
Which antibodies induce warm haemolytic anaemia?
IgG
What causes fifth disease?
Parvovirus B19
What is the incubation period of slapped cheek?
13-18 days; mainly infective during incubation period
Factors in ALL associated with better outcome
Female sex
cALL (early pre-B)
Remission within 1 month
Associated with gingival hypertrophy
Phenytoin
Cyclosporin
AML
Prevention for TLS in high risk children?
Aggressive hydration (2-3l/day) Rasburicase (recombinant uric oxidase)
Usual organisms for acute chest syndrome
Mycoplasma, chlamydia
What are similarities and differences between vWD and haemophilia A?
Factor VIII levels are low in both
APTT prolonged in both
vWD has mucocutaneous bleeding and prolonged bleeding time
Haemophilia A has joint bleeding and normal bleeding time + PT
Avoid in G6PD:
Fava beans Naphthalene Chloroquine Aspirin Cotrimoxazole Nitrofurantoin Nalidixic acid Ascorbic acid
X ray features of thalassaemia
Bone marrow expansion Frontal bossing Prominent maxillae Widening of bone cortex 'hair on end' or 'sun-ray' Phalanges are bilaterally convex or rectangular
Chemotherapy drug causing acute dystonic reactions
Domperidone
Chemotherapy drug causing haemorrhagic cystitis
Cyclophosphamide
Chemotherapy drug causing coagulopathy, hepatotoxicity, pancreatitis, hyperglycaemia
L asparaginase
Chemotherapy drug causing Redman syndrome (flushing)
Vancomycin
Chemotherapy drug causing mucositis, hepatitis, dermatitis, osteoporosis
Methotrexate
Chemotherapy drug causing cardiomyopathy, alopecia, mucositis
Doxorubicin
Chemotherapy drug causing headache and chest pain
Ondansetron
Chemotherapy drug causing headache, seizures, hypertension, rash, photophobia, nephrotoxicity
Ganciclovir
Chemotherapy drug causing rash, eosinophilia, biliary sludging
Ceftriaxone
Vaccines for those who have had splenectomy
Influenza
Pneumococcal polysaccharide
Haemophilus influenzae
Meningococcal
Features of ABO incompatibility vs rhesus
Occurs in first born
Presence of spherocytes
Exchange transfusion rarely needed
Dysmorphic features of Diamond-Blackfan
Webbing of neck Short stature Cleft palate Anomalies of the thumb Microcephaly Hypertelorism Micrognathia
