haem Flashcards
1
Q
Causes of pancytopenia
A
Marrow failure/infiltration:
- aplastica anaemia
- myelodysplastic syndrome
- leukaemia
- myelofribrosis
- infiltration by malignancy e.g. lymphoma
- infection
- megaloblastic anaemia
- prolonged starvation
hypersplenism
- portal hypertension
- RA
- storage disorders
2
Q
Causes of prolonged PT
A
- Warfarin
- liver disease
- vitamin K deficiency
- DIC
3
Q
causes of prolonged APTT
A
- Heparin
- haemophilia
- vWD
- DIC
- Liver disease
- Lupus anticoagulant
4
Q
Causes of low fibrinogen
A
DIC or severe liver disease
5
Q
What is polycythaemia rubra vera?
A
- Malignant proliferation of a clone from one pluripotent stem cell
- leads to excess RBCs, WBCs and platelets
- hyper-viscosity and thrombosis
- JAK2 mutation
- common >60s
- aquagenic pruritis, erythromelalgia, tinnitus, facial plethora
- can get gout
6
Q
what is essential thrombocythaemia?
A
- clonal proliferation of megakaryocytes
- persistently high platelet count >1000 x10^9
- bleeding, thrombosis, microvascular occlusion
- headache
- atypical chest pain
- erythromelalgia
7
Q
Treatment of polycythaemia rubra vera?
A
- Primary: venesection, aspirin 75mg, hydroxyurea and ruxolitumab if severe
- Secondary: vensection, aspirin 75mg, hydroxyurea
8
Q
Treat ment of essential thrombocythaemia
A
- Aspirin 75mg OD
- Hydroxycarbamide in high risk
9
Q
What is myelofibrosis?
A
- Clonal proliferation of abnormal megakaryocytes
- fibroblasts made instead of platelets
- causes bone marrow fibrosis
- haematopoiesis in speen and liver –> MEGALY
- B symptoms, anaemia, bleeding, infection
10
Q
Myelofibrosis blood film
A
- Teardrop RBCs
- leucoerythroblastic film - RBCs have nuclei
11
Q
Treatment of myelofibrosis
A
Allogenic SCT
can evolve into AML, can develop from ET or PRV
12
Q
Packed RBCs
A
- 1 unit increases Hb 10-15g/L or 1g/dL
- Transfused over 2-3 hours/unit
- Each unit prescribed separately on chart
- Special requests: irradiated, CMV negative
- Shelf life of 35 days
- Kept at 4°C
13
Q
When is irradiated blood required?
A
To prevent transfusion associated graft vs host disease in
- Hodgkin’s Lymphoma
- Congenital immunodeficiency
- Stem cell transplant
14
Q
How are platelets given?
A
- 1 unit increases platelets by 20 x10^9
- Given over 30 minutes
- Shelf life of 5 days
- Kept at room temperature
- units kept on shelves that are shaking continuously
15
Q
How is fresh frozen plasma given?
A
- Contains clotting factors
- Use within 24 hours of defrosting
- 1 year shelf life
- Kept at -30ºC
16
Q
What is Cryoprecipitate?
A
- Factor 8
- Factor 12/13
- Fibrinogen
- vWF
17
Q
What is prothrombin complex?
A
- Beriplex, octuplex
- Factors 2, 7, 9 and 10
- Protein C and S
- Dose: 30 units/kg
18
Q
Indications for RBC transfusion
A
To correct anaemia:
- Marrow failure e.g. aplastic anaemia, leukaemia
- Haemoglobinopathies e.g. thalassaemia, sickle cell
- Chronic disorders e.g. renal failure, malignancy
- Severe haemolysis e.g. haemolytic disease of the newborn
To replace blood loss
- Blood loss during surgery
- Trauma
- other haemorrhage
19
Q
Indication for platelets transfusion
A
- Decreased platelet production due to
- congenital BM failure
- acquired BM failure
- Dilutional thrombocytopenia
- functionally abnormal platelets
- platelet-destructive conditions
20
Q
Indications for fresh frozen plasma
A
- DIC
- Liver disease with abnormal clotting
- Coagulopathy of massive blood transfusion
- Factor V deficiency
- Warfarin overdose (vitamin K would be too slow)
- Thrombotic thrombocytopenia purpura
- Depletion of coagulation factors after thrombolysis
21
Q
Indication for fresh frozen plasma
A
- Same as FFP
- Haemorrhage after cardiac surgery
- hypofibrinogenemia
22
Q
Tests done before transfusion
A
- Blood grouping - test for ABO and Rh - agglutination
- Antibody screening - test for atypical antibodies
- Crossmatching - mix patients serum with donor red cells
23
Q
What is acute haemolytic transfusion reaction?
A
- When red cells from the transfusion react with the patient’s anti-A or anti-B antibodies etc
- Can be immediate or delayed
- Can stimulate complement, leading to DIC (clots everywhere) or intravascular haemolysis
24
Q
Presentation of acute haemolytic transfusion reaction
A
- haemolytic shock phase:
- urticaria, lumbar pain, headache, Chest pain, SOB, vomiting, rigors, fever, fall in BP
- Oliguric phase:
- some have necrosis of renal tubles with acute renal failure
- Diuretic phase:
- fluid and electrolyte imbalance following acute renal failure
25
Q
Treatment of acute haemolytic transfusion reaction
A
- Stop transfusion, check for ABO reactions/contamination
- Maintain BP and renal perfusion
- Saline, O2 and fluids
- 100mg hydrocortisone (IV) and antihistamines for shock
- Treat DIC - escalate, transfuse platelets or FFP
26
Q
What is febrile/afebrile non-haemolytic allergic reaction?
A
- may be due to antibody reaction targeting leukocytes in the transfusion
- antigen-antibody complexes induce complement
- symptoms: fever and rigors
- Mx: stop transfusion until haemolytic reaction ruled out
- new transfusion
- paracetamol for fever
27
Q
What is post-transfusion overload?
A
- When too much fluid volume is transfused or transfused too quickly
- caues pulmonary oedema and acute resp failure
- Management: furosemide (IV) and high-flow O2
28
Q
What is TRALI?
A
- form of acute resp distress from transfer of leucoagglutin HLA anitbodies from donor plasma
- causes endothelial and epithelial injury
- presents within 6hrs of transfusion
- sudden dyspnoea and tachypnoea, non productive cough, hypoxia, frothy sputum
- may have fevers and rigors
- CXR may show perihilar nodules with infiltration of lower lung zones
- Mx: high flor O2, IV fluids and inotropes, monitor ABGs, urgen ventilation may be needed but improves within 2-4 days
29
Q
What is graft-versus-host disease?
A
- usually immunocompromised patients
- between day 4 - 30 post transfusion
- high fever and diffuse red rash, progresses to erythroderma
- diarrhoea and abnormal liver function
- preventable with irradiated blood
30
Q
Signs and treatment of post transfusion iron overload
A
- myocardial, endocrine gland and liver damage
- Mx: chelation therapy with desferrioxamine
31
Q
What is post transfusion purpura?
A
- sever thrombocytopenia that occurs 7-10 days after any type of transfusion containing platelets, usually red cells
- patient has become sensitised to a foreign platelet antigen during pregnancy or from prior transfusion
- Sx: diffuse purpura and bleeding from mucour membranes, GI and urinary tracts
- Mx: high dose IV immunoglobulin
