Speciality: Haematology Flashcards
1
Q
Sickle cell presentation in babies/kids
A
- Hand-foot syndrome
- Swelling, pain and erythema of hands and feet.
2
Q
Blood Tx reactions
A
1) Allergic - facial flushing, urticaria, wheeze, hypotension - Rx w/ adrenaline + antihistamines + steroids + bronchodilators + BSC.
2) Febrile - fever, chills, anxiety, headache, tachy.- Rx w/ paracetamol.
3) Haemolytic - widespread haemolysis fever, abdo pain, hypotension = Rx w/ fluid resus
3
Q
Multiple myeloma
- Causes
- Presentation
- Ix
- Rx
A
- Neoplasm of the bone marrow plasma cells.
- CRAB = Raised calcium, Renal failure, Anaemia, Bone pain.
- Hypercoaguability so consider in CVA. - Bloods; High calcium, normal or high phosphate, normal ALP
- Monoclonal proteins in the serum/urine
- Bence-jones proteins.
- XR - osteolytic lesions. - Correct Sx
- Thalidomide
- Cyclophosphamide + thalidomide + dexa.
4
Q
Haemophilia A
- Causes
- Presentation
- Ix
- Rx
A
- X linked
- Lack of factor 8
- Most common cause of haemophilia - Haemarthroses
- Haematomas
- Bleeding propensity - Prolonged APTT
- Bleeding time, thrombin time and PT normal - IV factor 8
- Desmopressin can raise factor 8 levels.
5
Q
Iron deficiency anaemia
- Causes
- Presentation
- Ix
- Rx
A
- Excess blood loss (periods, GI bleed)
- Inadequate iron intake (veggie/vegan)
- Poor intestinal absorption (coeliac)
- Increased iron demand (pregnancy, haemolytic anaemia) - Fatigue
- SOBOE
- Palpitations
- Pallor
- Koilonychia
- Hair loss
- Atrophic glossitis - FBC - hypochromic microcytic anaemia.
- Low ferritin.
- High TIBC
- Low serum iron
- Low transferrin saturation
- Ix to find underlying cause. - Rx underlying cause
- Oral ferrous sulphate (SE incl; nausea, abdo pain, constipation and diarrhoea)
- Blood Tx prior to operations or IV iron.
6
Q
Hereditary Spherocytosis
- Causes
- Presentation
- Ix
- Rx
A
- Most common haemolytic anaemia.
- AD inherited
- Sphere shaped RBC
- Destroyed by the spleen - reduced lifespan.
- Due to haemolysis can cause gallstones. - Failure to thrive
- Jaundice and stones
- Splenomegaly
- Aplastic crisis w/ parvovirus - Patients w/ FHx, features and spherocytes do not require further Ix.
- Cryohaemolysis testing. - Folate replacement
- Splenectomy.
7
Q
Chronic myeloid leukaemia
- Causes
- Presentation
- Ix
- Rx
A
- Philadelphia chromosome present in 95%.
- Translocation between long arm of chromo 9 and 22.
- BCR-ABL gene formation.
- Increases tyrosine kinase activity. - 60-70yo
- Anaemia
- weight loss and sweats
- splenomegaly - early fullness etc
- Increased wcc at different points of development (neutrophils)
- May develop into AML/ALL via blast formation/ - FBC
- Blood film
- Genetic testing for the chromosome.
- PET - Imatinib (TKI)
- Hydroxyurea
- Bone marrow Tx
8
Q
Fresh frozen Plasma
- Indications
- Description
A
- Significant haemorrhage in patients w/ PT/APTT ratio (INR) >1.5.
- Prophylaxis to stop bleeding in invasive surgery.
- Reversal of warfarin
- Replacement of isolated factor deficiencies
- Rx of TTP. - Made from whole blood.
- ABO compatible as red cells are removed.
- Just plasma and its constituents.
9
Q
Cryoprecipitate
- Indications
- Description
A
- Uses include; DIC, liver failure, hypofibrinogenemia secondary to massive transfusion.
- Emergency in haemophiliacs and those w/ von Willebrand disease. - Contains factor 8:C, von Willebrand factor, fibrinogen, factor 13 and fibronectin.
- Further processing of FFP.
10
Q
Prothrombin complex concentrate
- Indications
- Description
A
- Emergency reversal of anticoagulation in those with severe bleeding, head injury or intracerebral haemorrhage
- Prothrombin only
11
Q
Von Willebrand disease
- Causes
- Presentation
- Ix
- Rx
A
- Most common haemophilia.
- Consider in women.
- AD inherited.
- Behaves like a platelet disorder (but isn’t)
- VWF promotes platelet adhesion to damaged endothelium (lack of this …)
- 3 types; type 1 (common) partial reduction. type 2 abnormal VWF. Type 3 is total lack (AR inherited). - Behaves like a platelet disorder.
- Bleeding from mucus membranes
- Bruising
- Epistaxis
- Heavy periods. - Prolonged bleeding time (due to lack of platelet aggregation to endothelium)
- APTT prolonged
- Normal platelets. - Tranny acid for mild bleeding
- Desmopressin can increase VWF
- Factor 8 concentrate.
12
Q
Transfusion thresholds
A
Normal bods = 70 g/L
ACS bods = 80 g/L
13
Q
Targets after blood transfusion
- Normal people
- ACS
A
- 70-90g/L
2. 80-100g/L
14
Q
Sickle cell anaemia
- Causes
- Presentation
- Ix
- Rx
A
- AR inherited
- Synthesis of abnormal Hb - HbS.
- Carriers = heterozygous and are often asymptomatic.
- In a deoxygenated state; RBC’s sickle.
- Sickle cells can block small vessels and cause infarction. - Anaemia
- Jaundice due to haemolysis
- Pallor
- Lethargy
- Weakness.
- Acute crises; vaso-occlusive, swollen painful joints, acute chest, stroke, aplastic crises, splenic sequestration. - FBC - normocytic anaemia
- Reticulocytes
- Howell-jolly bodies due to splenic breakdown
- LFT derangement due to increased break down.
- Definitive = Hb electropherisis - Avoid crises triggers (cold, dehydration, exhaustion)
- Hydroxycarbamide - increases foetal Hb concentration.
- Avoid alcohol and smoking
- Education
- Infection prophylaxis w/ penicillin.
15
Q
Sickle cell crises
A
1) Thrombotic - vaso-occlusive.
- Risks = infection, dehydration, deoxygenation.
- infarcts.
2) Sequestration - blood is trapped in the spleen or lungs.
- Exacerbates anaemia
3) Acute chest - SOB, pain and low pO2
4) Aplastic - Parvovirus infection
- Sudden drop on Hb.
