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Haematology P1 Flashcards

1
Q

Which factors does heparin affect?

A

2, 9, 10, 11

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2
Q

Which factors does warfarin affect?

A

2, 7, 9, 10

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3
Q

Which factors are affected in DIC?

A

1, 2, 5, 8, 11

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4
Q

Which factors are affected in liver disease?

A

1, 2, 5, 7, 9, 10, 11

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5
Q

How is APTT, PT and bleeding time affected in haemophilia?

A
  • increased APTT
  • normal PT
  • normal bleeding time
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6
Q

How is APTT, PT and bleeding time affected in von Willebrand’s disease?

A

-increased APTT -normal PT -increased bleeding time

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7
Q

How is APTT, PT and bleeding time affected in vit K deficiency?

A

-increased APTT -increased PT -normal bleeding time

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8
Q

What is acute intermittent porphyria (AIP)

A

-rare autosomal dominant -defect in porphobilinogen deaminase (enzyme in synthesis of haem) -toxic accumulation of delta aminolevulinic acid and porphobilinogen -more common in women

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9
Q

Presentation of AIP:

A

-abdominal: pain and vomiting -neuro: motor neuropathy -depression -HTN and tachycardia

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10
Q

Diagnosis of AIP:

A

-urine deep red on standing -raised urinary porphobilinogen (elevated between attacks and greater during acute) -assay of red cells for porphobilinogen deaminase -raised serum levels of delta aminolevulinic acid and porphobilinogen

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11
Q

Complications of antiphospholipid syndrome in pregnancy:

A

-recurrent miscarriage -IUGR -pre-eclampsia -placental abruption -pre-term delivery -venous thromboembolism

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12
Q

Management of antiphospholipid syndrome in pregnancy:

A

-low dose aspirin once pregnancy confirmed -LMWH once foetal heart on ultrasounds (discontinue at 24 weeks)

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13
Q

What is aplastic anaemia and peak incidence age?

A

-pancytopenia and hypo plastic bone marrow -peak incidence of acquired: 30yo

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14
Q

Features of aplastic anaemia:

A

-normochromic normocytic anaemia
-leukopenia with lymphocytes relatively spared
-thrombocytopenia
-may be presenting feature of acute lymphoblastic or myeloid leukaemia
-minority later develop paroxysmal nocturnal haemoglobinuria or myelodysplasia

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15
Q

Causes of aplastic anaemia:

A

-idiopathic
-congenital: Fanconi, dyskeratosis congenita
-drugs: cytotoxics, chloramphenicol, sulphonamides, phenytoin, gold
-toxin: benezene
-infections: parvovirus, hepatitis
-radiation

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16
Q

What test determines AIHA?

A

positive direct antiglobulin test (Coomb’s test)

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17
Q

Warm AIHA (MOA and management)

A

-antibody (usually IgG) causes haemolysis at body temperature and at extravascular sites e.g. spleen
-manage with steroids, immunosuppression and splenectomy

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18
Q

Causes of warm AIHA:

A

-autoimmune disease e.g. systemic lupus erythematosus
-neoplasia e.g. lymphoma, CLL
-drugs e.g. methyldopa

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19
Q

Cold AIHA:

A

-usually Igm
-haemolysis best at 4 degrees c
-mediated by complement and intravascular
-symptoms of Raynaud’s and acrocyanosis
-respond less well to steroids

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20
Q

Causes of cold AIHA:

A

-neoplasia e.g. lymphoma
-infections e.g. mycoplasma, EBV

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21
Q

Beta thalassaemia major: (incl features and management)

A

-absence of beta chains
-chromosome 11
-presents in first year of life with failure to thrive and hepatosplenomegaly
-microcytic anaemia
-HbA2 and HbF raised
-HbA absent
-manage with repeated transfusions (iron overload)
-infusion of desferrioxamine

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22
Q

Beta thalassaemia trait

A

-group of genetic disorders
-reduced production rate of either alpha or beta chains
-beta thalssaemia
- autosomal recessive with mild hypo chromic microcytic anaemia, usually asymptomatic (microcytosis characteristically disproportionate to anaemia)
- -HbA2 raised >3.5%

23
Q
A
  • target cells
  • sickl cell/thalassaemia
  • iron deficiency anaemia
  • hyposplenism
  • liver disease
24
Q
A
  • ‘tear drop’ poikilocytes
  • myelofibrosis
25
- spherocytes - hereditary spherocytosis - autoimmune haemolytic anaemia
26
- basophilic stippling - lead poisoning - thalassaemia - sideroblastic anaemia - myelodysplasia
27
- Howell-Jolly bodies - hyposplenism
28
- Heinz bodies - G6PD def - alpha thalassaemia
29
- shistocytes - intravascular haemolysis - mechanical heart valve - DIC
30
- pencil poikilocytes - iron deficiency anaemia
31
- burr cells (echinocytes) - uraemia - pyruvate kinase def
32
- acanthocytes - abetalipoproteinemia
33
What does hypersegmented neutrophils on a blood film indicate?
megaloblastic anaemia
34
How do blood films apear with hyposplenism (post-splenectomy, coeliac)?
- target cells - Howell-Jolly bodies - Pappenheimer bodies - siderotic granules - acanthocytes
35
How does iron def anaemia appear on a blood film?
- target cells - pencil poikilocytes - if combined with B12/folate def, dimoprhic film with mixed microcytic and macrocytic cells
36
How does myelofibrosis appear on a blood film?
tear drop poikilocytes
37
How does IV haemolysis appear on a blood film?
shistocytes
38
What are the different blood product transfusion complications?
- immunological: acute haemolytic, non-haemolytic febrile, allergic/anaphylaxis - infective (vCJD) - TRALI - tranfusion associated circulatory overload (TACO) - other: hyperkalaemia, iron overload, clotting
39
Non-haemolytic febrile reaction:
- ab react to white cell fragments in blood products and cytokines leaked during storage - causes fever, chills - stop/slow transfusion, paracetamol, monitor
40
Minor allergic reaction to transfusion:
- foreign plasma proteins - pruritus, urticaria - temporarily stop transfusion - antihistamine and monitor
41
Anaphylaxis after tranfusion:
- IgA deficiency with anti-IgA antibodies - hypotension, dyspnoea, wheezing, angioedema, stridor - stop tranfusion, IM adrenaline, ABC support - consider antihistaine, corticosteroids and bronchodilators
42
Acute haemolytics reaction:
- ABO incompatible blood - causes massive IV haemolysis, red blood cell destruction by IgM Ab - fever, abdominal pain, hypotension - stop tranfusion, confirm diagnosis (direct Coombs test and cross-matching), supportive care (generous fluid rescusciation) - complications: DIC and renal failure
43
Tranfusion associated circulatory overload (TACO)
- excessive rate of transfusion, pre-existing heart failure - pulmonary oedema, hypertension - slow or stop infusion, consider IV loop diuretics and oxygen
44
Transfusion related acute lung injury (TRALI)
- non-cardiogenic pulmonary oedema secondary to increased vascular permeability by host nuetorphils activated by blood - hypoxia, pulmonary infiltrates on CXR, fever, hypotension - stop transfusion, O2 and supportive - within 6 hours of transfusion
45
Packed red cells
- transfusion in chronic anaemia - infusion of large volumes which may result in cardiovascular compromise - centrifugation of whole blood
46
Platelet rich plasma
- thrombocytopaenia and bleeding or surgery - low speed centrifugation
47
Platelet concentrate
-prepared by high speed centrifugation and administered to patients with thrombocytopaenia
48
FFP
- clotting factors, albumin and immunoglobulin - unit usually 200-250 ml - to correct clotting deficiencies in hepatic synthetic failure about to undergo surgery - usual does 12-15ml/kg - not first line therapy for hypovolaemia
49
Cryoprecipitate
- formed from supernatant of FFP - rich source factor VIII and fibrinogen
50
SAG mannitol blood
-removal of all plasma from blood unit and substitution with NaCl, adenine, anhydrous glucose, mannitol
51
Warfarin reversal:
1. stop warfarin 2. vit K (reversal within 4-24 hours) - IV takes 4-6hrs, oral can take 24 hrs 3. FFP - not 1st line (only if prothrombin complex unavailable) 4. Human prothrombin complex - rapid action but factor 6 short half life so vit K
52
What is irradiated blood used for?
- depleted of T lymphocytes - used to avoid transfusion associated GVHD caused by engraftment of viable donor T lymphocytes - used for granlucyte transfusions, intra uterine transfusions, neonates up to 28 days post expected date, bone marrow/stem cell transplants, immunocompromised, patients with pervious/Hodgkin's
53
Prothrombin complex concentrate
- emergency reversal of anticoagulation in patients with severe bleeding or head injury with suspected intracerebral haemorrhage - prohpylacitc in patients undergoing emergency surgery

Haematology (4 decks)

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