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
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2
Q

Causes of prolonged PT

A
  • Warfarin
  • liver disease
  • vitamin K deficiency
  • DIC
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3
Q

causes of prolonged APTT

A
  • Heparin
  • haemophilia
  • vWD
  • DIC
  • Liver disease
  • Lupus anticoagulant
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4
Q

Causes of low fibrinogen

A

DIC or severe liver disease

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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
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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
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7
Q

Treatment of polycythaemia rubra vera?

A
  • Primary: venesection, aspirin 75mg, hydroxyurea and ruxolitumab if severe
  • Secondary: vensection, aspirin 75mg, hydroxyurea
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8
Q

Treat ment of essential thrombocythaemia

A
  • Aspirin 75mg OD
  • Hydroxycarbamide in high risk
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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
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10
Q

Myelofibrosis blood film

A
  • Teardrop RBCs
  • leucoerythroblastic film - RBCs have nuclei
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11
Q

Treatment of myelofibrosis

A

Allogenic SCT

can evolve into AML, can develop from ET or PRV

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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
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13
Q

When is irradiated blood required?

A

To prevent transfusion associated graft vs host disease in

  1. Hodgkin’s Lymphoma
  2. Congenital immunodeficiency
  3. Stem cell transplant
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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
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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
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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
  1. Blood grouping - test for ABO and Rh - agglutination
  2. Antibody screening - test for atypical antibodies
  3. 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