Haematology Flashcards
Blood film results of iron deficiency anaemia will show..?
Microcytic hypochromic anaemia
i.e. Small, pale RBCs
High reticulocyte count
Causes of IDA in children
Immature erythropoiesis Malabsorption of dietary iron - coeliac Autoimmune haemolysis blood disorders Cancers Bleeding (uncommon, e.g. menarche)
Management of IDA
5mg/Kg/Day of oral ferrous salt
+
Vitamin C to aid absorption
Which clotting factors are affected in Haemophilia
Haemophilia A - Factor 8
Haemophilia B - Factor 9
Differences between the two Haemophilias
Haemophilia A, much more common
haemophilia B - more severe
Describe inheritance of haemophilia
x-linked recessive
Only affects boys, with carrier mothers
50% of daughters of affected fathers with be carriers
Investigations for haemophilia
Clotting studies - APTT (Not PT)
FBC, assays of other clotting factors
presentation of Henoch-Schonlein Purpura
Low grade fever
Widespread macular purpuric rash over lower trunk and lower limbs
Diarrhoea - due to vasculitis of vessels supplying bowel
Painful joints + swelling
Rarely - kidney involvement
Triggers of a sickle cell crisis?
Infection, cold, exertion, dehydration
Aplastic crisis (cessation of erythropoiesis) due to infection with Parvovirus B19
Management of acute sickle cell crisis
Analgesia, hydration
Blood transfusion if severe/life threatening/organ at risk of ischaemia
Pathophysiology of thalassaemia?
Thalassaemia Alpha or Beta (depending on haem chain affected) - misshapen dysfunctional RBCs
Can be Major, intermedia, minor - classification of severity
Alpha-major often incompatible with life
Investigations for thalassaemia
Picked up on Guthrie assay at 3-10 days of life
Microcytic hypochromic anaemia on film - that fails to respond to iron
Low Hb, ^serum ferritin/iron (so not IDA), Hb electrophoresis
Management of thalassaemia intermedia/major?
regular transfusions if necessary
+ iron chelation therapy to prevent overload
If severe, splenectomy due to splenomegaly
Genetic counselling
