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Finals > Haematology > Flashcards

Haematology Flashcards

1
Q

What is ABO blood grouping?

A

inherityed surface antigens on RBCs

A- have A antigens on RBCs, and anti-B antibodies in plasma

B- Have B antigens on RBCs, and anti-A antibodies in plasma

AB- have both A and B antigens on RBCs, neither anti-A or anti-B antibodies

O- have neither A or B antigens on RBCs, have both anti- B and anti-A antibodies

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2
Q

What blood can type A receive

A

A, O

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3
Q

What blood can type B receive

A

B, O

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4
Q

What blood can type AB receive

A

A, B, AB, O

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5
Q

What blood can type O receive

A

O

Universal donor

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6
Q

What is Rhesus types

A

C, D and E antigens on RBCs

Rhesus -ve/+ve refers to the presence or absence of the D antigen

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7
Q

why are rhesus types important in pregnancy/childbirth

A

Rhesus negative (dd) mothers who are exposed to rhesus positive cells (from +ve baby), will make IgG anti-D antigens which ccan cross the placenta and haemolyse baby’s blood

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8
Q

What are the indications of fresh frozen plasma

A
  • contains COAGULATION proteins, albumin, carrier proteins for nutrients/hormones, Ig
  • lost about half of their blood volume- major haemorrhage protocol-
  • actively bleeding with deranged clotting (PT, APTT, INR)
  • consider prophylactically before surgery if clotting is deranged
  • give units one by one and reassess coag status
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9
Q

When do you give platelets

A
  • major haemorrhage
  • thrombocytopenia (plt<30)
  • prophylaxis pre-surgery if plt <50

do not routinely offer to pt with bone marrow failure, autoimmune thrombocytopenia, herpain induced thrombocytopenia, thrombotic thrombocytopenic purpura

  • give one unit and reassess unless severely haemorrhaging
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10
Q

indications for cryoprecipitate

A
  • significant bleeding and fibrinogen level <1.5g/L
  • prophylactically for pts with fibinogen level <1g/L pre-surgery
  • adult dose= 2 ‘pools’– reassess and repeat
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11
Q

what is in cyroprecipitate

A

fibrinogen, fibronectin, factor VIII, vWF, factor XIII

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12
Q

how do you order blood

A
  • 2x group and saves from a pt taken 15 mins apart for safe blood typing
  • likely need to code the reason for the request
  • major haemorrhage– 2u of O- instant access
  • prescribe either on fluid chart or on specific blood products chart- each unit needs to be it’s own px
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13
Q

how do you give blood products

A
  • wide bore cannula to prevent haemolysis
  • give over 2-3 hours in non urgent cases
  • give within 30mins of leaving the fridge (should be warmed before administering)
  • baseline obs at 0, 15, 30mins into the transfusion
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14
Q

Complications of blood transfusions

A
  • acute haemolytic transfusion reaction- ABO incompatibility
  • Anaphylaxis
  • Transfuion related acute lung injury
  • transfusion associated circulatory overload
  • hypocalcaemia
  • post transfusion purpura
  • graft versus host disease
  • transmission of blood borne diseases
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15
Q

what is acute haemolytic reaction (ABO incombatibility)

A
  • Anti-a/b antibodies activate complement pathway, triggering inflammatory cytokine release
  • most severe is when A is given to O pt
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16
Q

ix for ?acute haemolytic reaction

A
  • blood film
  • direct antiglobulin test
  • retest both bloods (cross match and antibody screen)
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17
Q

tx acute haemolytic reaction

A
  • stop transfusion
  • fluid replacement
  • FFP/platelets to tx DIC
  • *- dopamine (vasodilation in the kidneys)
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18
Q

sx of acute haemolytic reaction

A

sx

  • fever
  • hypotension
  • abdo/chest pain
  • SOB
  • haematuria and widespread haemorrhage (DIC)
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19
Q

what is transfusion related acute lung injury , sx

A
  • not related to fluid overload (that is an immune response of unknown mechanism causing pulm oedema and ARDS)
  • more typically due to FFP/plts than with red packed cells

sx- onset is sudden and severe, occurs within 6hours of transfusion:

  • Dyspnoea
  • Hypoxaemia
  • hypotension
  • fever
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20
Q

management of transfusion related acute lung injury

A

usually resolves within 48-96hours

  • O2
  • sometime ventilation
  • fluids
  • corticosteroids sometimes
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21
Q

What is transfusion associated circulatory overload

A
  • hypervolaemia as a result of a transfusion
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22
Q

sx of transfusion assoc circulatory overload

A
  • dyspnoea
  • orthopnoea
  • peripheral oedema
  • rapid increase in BP
  • pulmonary oedema
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23
Q

RF for tranfusion assoc circulatory overload

A 
CVD
kidney disease
lung disease
hypoalbuminaemia
severe anaemia
age (<3years old and >60)
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24
Q

Management of transfusion assoc circulatory overload

A

stop transfusion
oxygen
diretics (furosemide)
other tx for - ACEI/ARB, BB

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25
Q

why can hypocalcaemia occur after a transfusion, tx

A
  • citrate (anticoagulant present in the bag) binds to free serum Ca in plasma, leaving the pt hypocalcaemic
  • rare, only in massive transfusions

tx- calcium gluconate

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26
Q

What is post transfusion purpua, why does it occur

A
  • immune reaction- antibodies to the new platelets’ antigens are produced, causing plt destruction leading to thrombocytopenia and bleeding under the skin
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27
Q

sx of post transfusion purpura

A
  • purple rash

- 5-12 days after transfusion

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28
Q

tx post transfusion purpura

A

supportive

igG IV

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29
Q

what is graft versus host disease

A

White cells in donated tissue recognise the recipient as foreign
attack of host cells

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30
Q

what are some transmission of blood-borne disease

A 
HIV
Hep B, C
syphillis
malaria
chagas disease (trypanosomiasis)- parasite
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31
Q

Name some P2Y12 receptor antagonist antiplatelets

A

clopidogrel
ticagrelor
prasugrel

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32
Q

name a COX inhibitor antiplatelet

A

aspirin

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33
Q

Name some GPIIIaIIB inhibitor antiplatelets

A

abciximab
tirofiban
eptifibatide

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34
Q

name some antithrombin anticoagulants

A 
fondaparinux
unfractionated heparin
enoxaparin- LMWH 
dalteparin- LMWH 
tinzaparin- LMWH
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35
Q

name some factor Xa inhibitor anticoagulants

A

Rivaroxiban
apixaban
DOACs

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36
Q

name a vitamins K antagonist anticoagulant

A

warfarin

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37
Q

name some plasminogen activator anticoagulants

A

alteplase
reletplase

streptokinase is also a thrombolytic but is not used widely due to high levels of anaphylaxis

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38
Q

what is the difference between antiplatelets and anticoagulants

A

Anticoagulants
- inhibit clotting factors and fibrin

Antiplatelets
- inhibit enzymes that cause platelets aggregation

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39
Q

how do p2y12 inhibitors work

A
  • ADP released from damaged endothelium and activated plts
  • binds to P2Y12 receptor on platelet (which is blocked by these drugs)
  • activates glycoproten IIB/IIa receptors, which causes fibrinogen mediated platelet cross-linking
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40
Q

how do cox inhibitors work

A
  • when platelet is acitvated, arachidonic acid is released into the plt
  • COX-1 metabs this into prostaglandin H2
  • this is metab into thromboxane A2, which is released
  • stimulates more platelets and aggregation
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41
Q

how do glycoprotein 2b.3a inhibitors work

A
  • glycoproten IIB/IIa receptors, causes fibrinogen mediated platelet cross-linking
  • administered only IV
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42
Q

contraindications for aspirin

A
  • active peptic ulceration
  • bleedgng disorders/haemophilia***
  • <16y/o- Reye’s
  • breastfeeding**

caution

  • asthmatics
  • HTN
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43
Q

what is reye’s syndrome

A
  • rapid encephalopathy, hepatitis

sx

  • vomiting
  • change in personality
  • confusion
  • seizures
  • LoC
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44
Q

SE of aspirin

A
  • gastric irritation/bleeding
  • asthmatic attack/bronchospasm
  • reye syndrome in children
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45
Q

what is dual antiplatelet therapy

A

aspirin+ticagrelor - secondary prevention of MI

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46
Q

when is aspirin used

A

MI- 300mg tx/75mg prophylactic
secondary prevention of MI/DVT
- TIA (300mg, 75mg)
- acute ischaemic stroke 300mg

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47
Q

when are p2y12 inhibitors used

A

ticagrelor/clopidogrel

  • prophylaxis during PCI
  • alternative to aspirin in stroke
  • dual antiplatelet therapt- secondary prevention post MI/vascular disease
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48
Q

What classes of drug are included in the umbrella term ‘antiplatelets’

A

COX inhibitors
P2Y12 inhibitors
Glycoprotein IIb/IIIa inhibitors

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49
Q

what class drugs are included in the umbrella term ‘anticoagulants’

A

NOACs/DOACs- factor Xa inhibs*****
Vit k antagonists - warfarin
Plasminogen activators- alteplase
Antithrombins- LMWH, unfract. hep.

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50
Q

mechanism of warfarin

A

vit K antagonism

blocks vit K oxide reductase
this usually activates vit K and uses vit k to produce factors 2 (prothrombin), 7, 9, 10

long half life- full effect not seen until a few days
may become hypercoagulable at first

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51
Q

indications for warfarin

A
  • VTE tx
  • TIA
  • AF
  • heart valve disease- rheumatic, mechanical, mitral
  • inherited, symptomatic thrombophilia
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52
Q

warfarin interaction

A

enzyme inducers (decrease INR)- CRAPS (cabamazepine, rifampicin, bArbituates/alcohol chronic, phenytoin, St johns wort/sulphonylureas)

vit K (leafy greens)

enzyme inhibitors (increase INR) Some, certain Damn Silly Compounds Annoyingly Inhibit Enzymes GRR
Sodium Val, Cipro, Dilitiazem, Sulphonamide, Cimetidine/omezop, antifungals/Amiodarone, Isoniazid, Erythro/clarithro, Grapefruit juice

AODEVICES- Allopurinol, omezoprazole, disulfiram, erythro, valproate, isoniazid, cipro, ehtanol (acute), sulphonamides

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53
Q

monitoring of warfarin

A

amount adjusted to maintain:

2-3 INR (venous embolism, AD, Mitral valve, inherited thrombophilia)

2.5-3.5- mechanical heart valves

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54
Q

how are the effects of warfarin reversed eg for emergency surgery

A
  • witholding warfarin
  • vit K (oral/IV)– phytomenadione
  • prothrombin complex concentrate- have factors 2,7,9,10
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55
Q

pt info for warfarin

A
  • take before, during or after a meal OD
  • remember to take dose
  • do NOT double dose
  • no piercings or tattoos
  • must always stick with same brand (bioavailabilities differ)
  • seek urgent medical help if theres’ blood in stool/urine/black poo/severe bruising/long nosebleeds/significant blow to the head/any signs of bleeding or prologned bleeding
  • always check new meds/OTC meds can be taken with warfarin
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56
Q

What does bridging anticoagulant medication mean

A
  • pts taking vit k antagonists at high risk of thromb emb (VTE in last 3m, AF with previous stroke/TIA, mitral mechanical valve)
  • warfarin stopped in perioperative period, so require unfractinated heparin or LMWH in interim
  • using tx dose
  • LMWH then stopped 24hours before surgery
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57
Q

How are the effects of LMWH and unfractionated herparin reversed

A

protamine

LMWH- partially (60%)
UFH- fully

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58
Q

what is polycythaemia

A

too many RBCs

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59
Q

causes of polycythaemia

A
  • Primary- Polycythaemia rubra vera (overactive bone marrow)

Secondary

  • lung disease, smoking
  • living at high altitude
  • pregnancy**
  • malignancy
  • kidney disease/RCC– too much EPO**
  • CYANOTIC heart disease
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60
Q

sx of polycythemia

A
  • plethoric face
  • thrombosis/VTE
  • pruritis
  • splenomegaly /abdo discomfort
  • unusual bleeding (nosebleeds)
  • severe headache, dizziness
  • fatigue
  • paraesthesia
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61
Q

ix for ?polycythemia rubra vera

A
  • FBC
  • JAK2 on genotyping
  • USS abdo- kidneys, spleen
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62
Q

management of polycythemia rubra vera

A
  • venesection
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63
Q

management of secondary polycythemia

A

aspirin- reduced risk of clots

  • venesection
  • bone marrow supression - hydroxycarbamide, interferon
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64
Q

Causes of anaemia

A

MCV <80

  • iron deficiency
  • thalassaemia
  • haemoglobinopathies
  • ** sideroblastic anaemia (iron cannot be incorp into RBC)***
  • chronic disease (incl. kidney, less common)
  • lead

MCV 81-95

  • blood loss
  • chronic disease (incl. kidney, more common)
  • pregnancy
  • haemolysis ***
  • combined iron/b12/folate deficiency

MCV >96– ABCDEF

  • alcoholic/ liver disease**
  • b12 deficiency
  • compensatory reticucytosis
  • drugs- cytotoxic
  • endocrine- hypothyroid
  • Folate deficiency/foetus
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65
Q

presentation of anaemia

A

fatigue, faintness, anorexia
dyspnoea
palpitations, angina
headache, tinnitus

Signs:

  • pallor- conjunctival
  • tachycardia
  • jaundice if haemolytic
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66
Q

ix for anaemia

A
  • Bloods- FBC (hb, MCV), iron studies, haematinics (B12/folate)
  • *- blood film- reticulocyte count
  • FIT, LFTs
  • *- USS spleen, kidney
  • *- direct coombs
  • genetic testing
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67
Q

management of anaemia

A

Acute anaemia/v symptomatic

  • ferinject (IV iron)
  • transfusion if Hb <70 ro <80+IHD

Chronic
- iron/folate/b12 supplements and diet advice

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68
Q

how do you interpret reticulocyte count

A

high (megaloblastic)- producing lots of, large, immature RBCs:

  • bone marrow working well to compensate for anaemia
  • loss of blood (chronic)
  • hypersplenism
  • haemolysis

Low (non-megalobastic)

  • bone marrow is working not well
  • do MCV
  • Mircocytic- iron, thalassaemia, sideroblastic
  • normocytic- kidneys, chronic, acute bleeds
  • macro- b12/folate, hypothyroid, bone marrow failure
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69
Q

causes of hypersplenic megaloblastic anaemia (and Ixs)

A
  • congestion (LFTs, portal vein doppler, abdo imaging, d-dimer)
  • infiltration (protein electrphoresis, blood film (malignancies), spleen biopsy
  • poliferation- viral serologies, blood film (RBC abnomrlaities)
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70
Q

causes of megalobastic, haemolytic anaemia (and ixs)

A

A type of macrocytic anaemia– Bone marrow producing large, immature RBC , due to increased rates of haemolysis

bilirubin and LFTs deranged

Extrinsic
Immune- direct coombs:
-drug induced (penicillin, quinine)
-autoimmune- SLE, infection, idiopathic
-Infection- malaria- blood film
DIC- coag profile
Thrombotic thrombocytopenic purpura- low plts due to clots

Intrinsic- genetic and blood film testing:
  Enzyme defects
        - G6PD (xlinked, broad beans)
         - Pyruvate Kinase, 
  Membrane defects:
         -spherocytosis
         -ellipotcytosis 
         -ovalocytosis
         -stomatocytosis
  Haemoglobinopathoes
         -sickle cell
         -thalassaemia
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71
Q

specific sx of iron deficiency anaemia

A
  • koilonychia (soft, scooped out/spoon nails)
  • angular cheilitis
  • atrophic glossitis- swollen, painful/itchy tongue
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72
Q

ix results in iron deficiency anaemia

A
  • Hb low
  • MCV <80
  • reticulocyte count low
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73
Q

tx for iron deficiency anaemia

A
  • ferrous sulphate 200mg TDS PO for at least 3 months
  • urgent 2ww if >60 or women >55 with PMB
  • consider coeliac screen, urine dipstick, stool sample for parasites, FIT
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74
Q

SE of ferrous sulfate

A

black stools
diarrhoea, constipation
nausea

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75
Q

normal hb levels

A

120-180 g/L

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76
Q

Specific sx for b12/folate deficiency

A

v. insidious
- pallor, jaundice, glossitis
- mood- irritability, depression, psychosis

NEURO:

  • sensory neuropathy- paraesthesia
  • upper and lower motor neuron signs (extensor plantar, absent reflexes, ataxia)
  • weakness
  • urinary incontinence
  • optic neuropathy
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77
Q

management of b12/folate deficiency

A

always correct b12 Before starting folate as folate can hide sx of b12 deficiency and if untx–> spinal cord degeneration

hydroxocobalamin IM then folic acid

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78
Q

what is pernicious anaemia

A
  • b12 deficiency due to autoimmune atrophic gastritis
  • parietal cells in stomach destroyed
  • reduced intrinsic factor secretion
  • reduced b12 absorption in terminal ileum
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79
Q

management of pernicious anaemia

A
  • 1mg IM hydroxocobalamin for life

3x a week for 2w, then every 2-3m

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80
Q

what is sideroblastic anaemia

A
  • ineffective erythropoeisis
  • leading to increased Iron absorption and iron loading in marrow (not in RBCs)
  • production of erythrocytes with ferritin granules in them that cannot incorporate the iron in the Hb (sideroblasts)
  • these get deposited into endocrine organs, liver and heart
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81
Q

causes of sideroblastic anaemia

A
  • congenital
  • malignancy- CML
  • alcohol

Iatrogenic:

  • chemo
  • anti-TB drugs
  • irradiation
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82
Q

sx of sideroblastic anaemia

A

pallor
fatigue
dizziness
hepatosplenomegaly

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83
Q

tx of sideroblastic anaemia

A

tx cause
blood transfusion and iron chelation
**pyridoxine (vit B6)***

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84
Q

sx of haemolytic, megaloblastic anaemias

A
  • anaemia sx
  • jaundice
  • dark urine
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85
Q

ix for ?haemolyic anaemia

A
  • malaria testing
  • Coomb’s test (autoimmune causes)
  • FBC, LFTS
  • blood film- malaria, RBC deformities
  • LDH levels- raised
  • Hb electrphoresis- sickle cell, thalassaemia
  • sickle solubility test
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86
Q

precipitants of glucose 6 phosphate deficiency

A

Broad beans
Primaquine
Sulphonamides
- Dapsone (gangrene, hermatitis, herptiformis, bascuitits)

Nitrofurantoin
Quinolones- cipro

infection
stress

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87
Q

inheritance pattern G6PD

A

x linked

recessive

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88
Q

inheritance pattern membranopathies

A

dominant

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89
Q

inheritance pattern sickle cell

A

recessive

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90
Q

what is sickle cell anaemia

A
  • Hb conatins an abnormal Hb globin chain
  • single base substitution in beta chain
  • creates HbS which changes the solubility of Hb and damages the cell containing it
  • inflexible
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91
Q

sx sickle cell disease

A

Homozygous- severe sx
Heterozygous- may have mild sx

  • from 3- 6 months of age as HbF replaced by HbSS (instead of Beta)
  • anaemia sx
  • growth restriction, delayed puberty
  • recurrent infections

Sickle crises

  • acute painful crisis due to vaso-occ
  • sequestrian crisis
  • hyperhaemolytic crisis
  • acute chest syndrome- chest pain, SOB
  • splenomegaly, asplenism
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92
Q

ix for sickle cell

A 
FBC- anaemia
LFTS- bilirubin
Blood film
sickle solubility test
Hb electrophoresis- diagnostic

neonatal screening- heel prick

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93
Q

How to avoid sickle cell crises

A
  • avoid triggers- cold temps, dehydration, exhaustion, alcohol, smoking
  • prevent infections- oral penicillin, vaccination
  • folic acid supplementation
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94
Q

tx of sickle cell

A
  • blood tranfusions
  • iron chelation
  • hydroxycarbamide (B12) OD (increase HbF)
  • stem cell/bone marrow transplant
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95
Q

management of sickle crisis

A
  • paracetemol, ibuprofen
  • opioids
  • IV fluids
  • O2 to avoid acute chest
  • empirical broad spec abx if fever
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96
Q

what is a sequestration crisis, tx

A

erythrocytes trapped in spleen– causing dangerous drop in circulating blood vol, infarction, atrophy

Acute anaemia:

  • profound pallor, SOB, weakness
  • Acute splenomegaly
  • Shock

urgent transfusion

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97
Q

what is an aplastic crisis, sx, tx

A
  • temporary cessation of erythropoesis
  • usually due to parvovirus b19

sx

  • severe anaemia- SOB, pallor, weakness
  • may present as high output congestive HF- SOB, oedema
  • tx- blood transfusion
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98
Q

What is thalassamia

A

reduced synthesis of alpha or beta chains with consequent reduction of haemoglobin

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99
Q

presentation of beta thalassaemia

A

MAJOR

  • present at 6-12 months of age (beta chains replace gamma)
  • Failure to feed
  • listless, crying
  • pale
  • anaemia
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100
Q

types of beta thalassaemia

A

major- no beta chain at all, transfusion dependent

intermedia- microcytic anaemia, can survive without transfusions

Carrier/heterozygote- clinically well

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101
Q

pathophysiology of beta thalassaemia

A

beta chain production affected

only become apparent when adult Hb replaces HbF (Y chains–> b chains)

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102
Q

ix ?thalassaemia

A

FBC- severe anaemia, low MCV- MICROCYTIC, reticuloyes low (non-megaloblastic)
blood film- large and small, irregular pale red cells
HbF >90% in foetal sample

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103
Q

A thalassaemia- pathophysiology

A
  • production of alpha chain affected, meaning HbF is affected
  • 4 alleles involved- 2 mum, 2 dad
  • generally caused by deletion mutations
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104
Q

presentation of alpha thalassaemia

A

depends on how many alleles are involved

1- asymptomatic
2- asymptomatic but low MCV
3- moderate anaemia and features of haemolysis eg hepatosplenomegaly, leg ulcers
4- Bart’s hydrops (foetal death)

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105
Q

What is thrombocytosis

A

too many platelets (>450)

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106
Q

causes of thrombocytosis

A

Primary (thrombocytopemia)- NOT thrombocytoPENIA

  • essential thrombocytopemia
  • Polycythemia rubra vera- increased RBC with also hgih WCC and plt in most cases
  • haematological malignancy (CML)
  • primary myelofibrosis - scarring of BM, can cause high or low plts

Secondary

  • chronic inflammation
  • severe systemic infection
  • bleeding/healing, clotting- surgery/trauma
  • malignancy
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107
Q

What is thrombocytopenia

A

too few platelets (<150)

108
Q

Causes of thrombocytopenia

A

Decreased production

  • congenital platelet abnormalities
  • bone marrow suppression /infiltration

Increased destruction

  • immune conditions
  • hypersplenism
  • consumption- DIC, ITP, HELLP
109
Q

hx for ?thrombocytosis

A
  • Essential
  • inflammation
  • Malignancy-sx of possible cancers:
  • bowel– habits, rectal bleeding
  • ENT/gastric/oes- dysphagia, dyspepsia
  • Resp- dyspnoea, fatigue, cough, haemoptysis
  • gynae- PMB
  • wt loss

Polycythaemia vera

110
Q

ix for ?thrombocytosis

A
  • FBC, CRP/ESR, iron studies
  • blood film
  • CXR if >40yo
  • USS pelvis if females >55yo
  • FIT
  • JAK-2 testing- essential thrombocytopemia, PRV
111
Q

Management of someone with thrombocytosis

A
  • Urgent referral if plt >1000 or 600-1000 with clinical features
  • tx the cancer
112
Q

what causes bone marrow suppression/failure (not by infiltration)

A
  • low b12/folate
  • alcoholism
  • CT, radiation
  • azathioprine. methotrexate
  • NSAIDs
  • viral infections (parvovirus- aplastic, HIV, hepatitis)
113
Q

what conditions infiltrate into bone marrow

A
  • leukaemia
  • mets
  • lymphoma
  • myeloma
  • myelofibrosis
114
Q

presentation of thrombocytopenia

A
  • usually asymptomatic until severe/found incidentally
  • bleeding/bruising
  • petechiae/purpura
  • infections
  • Fam hx
  • obstetric hx
  • screen for sx of occult malignancy
115
Q

ix for ?thrombocytopenia

A
  • FBC, UE, clotting, haematinics (b12/folate)
  • blood film
  • bone marrow biopsy if ?myeloproliferative disease
  • genetic screening
116
Q

management of thrombocytopenia

A

pt education

  • avoid contact sports
  • avoids NSAIDs (impairs platelet function)
  • maintain good dental hygiene (minimise risk of gingival bleeding/need to dental procedures)
  • avoid deep IM injections (sc when poss)
  • platelet transfusion- if severe
  • TXA
  • Prednisolone (ITP)
  • TPO agonsists- hormone that increases plt prod.
  • bone marrow transplant

Urgent referral

  • plt <20
  • severe bleeding
  • red cell fragments or blasts on film (cancer)
  • hx of b sx (fever, wt loss, sweats)
  • consider discussion with haematologist if plt <100
117
Q

What is immune thrombocytopenic purpura

A
  • immune destruction of plts

- IgG atnibodies to platelet and megakaryocyte surface protein

118
Q

presentation of ITP

A

bruising
petechiae (esp on lower legs)
bleeding- gums, nose, menorrhagia
NO splenomegaly

119
Q

ix ?ITP

A

dx of exclusion

  • bloods- isolated low plt
  • blood film - normal (exclude cancer- red cell blasts/fragments)
  • bone marrow biopsy - normal (cancer, myelofibrosis)
  • serology for hep b/c
120
Q

management of ITP

A
  • IV IG ***
  • prednisolone/dexamtethasone PO/hydrocortisone IV if severe
  • plts and TXA** if severe bleeding
  • splenectomy** if not responding to tx
  • pt education
  • tx underlying cause
  • if post-viral in children - may resolve without tx
121
Q

what is thrombotic thrombocytopenic purpura

A
  • reduction in protease enzyme (ADAMTS13) which usually breaks down VWF
  • more VWF–> clotting
  • leads to less plts, as theyre all used up and consumed
122
Q

causes/triggers of thrombotic thrombocytopenic purpura

A
  • bacterial infection
  • iatrogenic- plt inhibitors, CT, anti-virals, immunosuppressants
  • AI diseases- SLE
  • pregnancy
123
Q

presentation of thrombotic thrombocytopenic purpura

A
  • large bruises
  • weakness, lethargy
  • SOB
  • confusion
  • headache, confusion, fever
  • reduced kidney function
  • often preceded by diarrhoeal/flu-like illness
124
Q

ix ?thrombotic thrombocytopenic purpura

A
  • FBC (low plts, anaemia)

- ADAMTS13 levels (very low)

125
Q

management of thrombotic thrombocytopenic purpura

A
  • urgent plasma exchange- replaces ADAMTS13 levels and removes antibody
  • corticosteroids, rituximab
  • do NOT give blood/plts as this increases thrombosis
126
Q

pathophysiology of DIC

A 
cytokines released
activation of clotting cascade
fibrin production-- consumptive coagulopathy
thrombosis
consumption of plts

–> clotting plus bleeding plus multi-organ failure

127
Q

Causes of DIC

A
  • shock
  • sepsis
  • major trauma or burns
  • malignancies (AML)
  • snake bites
  • severe immune reactions- ABO mismatch, organ transplant rejection
  • severe organ dysfunction - acute hepatic failure, acute pancreatitis
  • obstetric emergencies- eclampsia, HELLP, placental abruption, intrauterine death, amniotic fluid embolism
128
Q

presentation of DIC

A
  • precipitating event
  • bleeding- from unusual sites (haematuria, rectum, bruising/petechiae, ears, nose, haemoptysis, sites of venepuncture/cannulation)
  • circulatory collapse- hypotension, tachycardia, cyanosis, confusion/LoC/coma

signs of microvascular thrombosis and tissue damage

  • Cerebral- new confusion or disorientation, dysphagia/dysarthria, weakness
  • chest pain
  • leg pain, necrosis, gangrene
129
Q

ix ?DIC

A
  • septic screen
  • FBC- thrombocytopenia
  • Prolonged PT, APPT, high INR, low fibrinogen
  • high d-dimers
  • ISTH scoring- uses plt count d-dimer, PT and fibrinogen
130
Q

ddx of DIC

A
  • decompensated liver failure (coagulopathy)
  • vit K defieciency/anticoag overdose
  • HELLP
  • ITP- immune thrombocytopenic purpura
  • TTP- thrombotic thrombocytopenic purpura
131
Q

management of DIC

A
  • tx cause
  • urgent haematological review
  • fresh platelets (try to maintain >50)
  • FFP (contains clotting factors)
  • Cryoprecipitate (if fibrinogen is low)
  • transfusion
  • therapeutic dose heparin
132
Q

what is haemolytic uraemic syndrome

A
  • usually due to E.coli shiga toxins (verotoxin) leading to increased thrombogenicity
  • ‘thrombotic microangiopathy’
  • kidney microvasculatrue damaged
  • increases clotting in this microvasc.
  • plt consumption–> thrombocytopenia with AKI
133
Q

the triad and presentation of haemolytic uraemic syndrome

A
  • microangiopathic haemolytic anaemia
  • thrombocytopenia (bleeding/bruising)
  • AKI- HTN, oedema, olgio/anuria
  • bloody diarrhoea
  • abdo pain
  • vomiting
  • petechiae
  • pallor
  • often in children <5
134
Q

ix ?haemolytic uraemic syndrome

A
  • FBC, UE, Clotting, LFTs
  • peripheral blood smear
  • stool culture- SHiga toxin
  • urinanalysis
135
Q

management of haemolytic uraemic syndrome

A
  • fluid restriction (AKI)
  • avoid abx, antimotitly agents, NSAIDs (kidney strain)
  • dialysis/renal transplant
136
Q

What is HELLP syndrome

A
  • haemolysis
  • elevated liver enzymes
  • low plts
  • form of pre-eclammpsia
  • assumed due to endothelial injury–> increased vWF –> consumption of plts–> widespread thrombotic microangiography
137
Q

presentation and signs of HELLP syndrome

A

often 27-37 weeks antepartum

  • new onset abdo pain (RUQ, epigastric)
  • N+V
  • headache
  • blurry vision

may lead to seizures
pre-eclampsia— HTN, proteinuria

138
Q

diagnostic criteria for HELLP

A
  • elevated transanimases (AST) twice the upper limit of normal
  • low plt count <100
  • haemolysis- elevated bilirubin, LDH elevation, schistocytes on peripheral blood smear, haematuria, worsening anaemia, low serum haptoglobin
139
Q

Management of HELLP

A
  • delivery of the baby

tx pre-eclampsia

  • labetolol, nifedipine, methyldopa
  • IV dexamethasone
  • MgSO4- prevent seizures
140
Q

what is thrombophilia

A
  • umbrella term– increased tendency to clot— hypercoagulable

(NB: thrombocytosis is high plts specifically, thrombopemia is idiopathic high plts)

141
Q

causes of thrombophilia

A

Congenital

  • Factor V leiden
  • prothrombin mutation
  • antithrombin deficiency
  • protein C and S

Acquired

  • antiphospholipid synrome- lupus anticoag, anti-cardiolipin, antibeta1-glycoprotein
  • autoimmune SLE, IBD
  • cancer
  • hyperviscosity- sickle cell, myeloproliferative, PRV, essential thrombocytosis/thrombocytopemia
  • nephrotic syndrome (urinary loss of antithrombotic proteins)
  • high oestrogens- pregnancy, OCP, obesity
  • heparin induced thrombocytopenia (type II)- immune response causing immune complexes binding to plt–> plt destruction plus plt aggregation–> thrombocytopenia plus thrombosis/thrombophilia
142
Q

presentation of thrombophilia

A
  • DVT, PE
  • thrombosis
    • venous sinus thrombosis (headache, blurred vision, movement issues, seizure, coma)
    • portal vein
      thrombosis/hepatic vein thrombosis- RUQ pain, jaundice, blood in stool
    • mesenteric vein and veins in arms
  • obstetric - recurrent miscarriage, IUGR, stillbirth, severe pre-eclampsia, placental abruption
143
Q

ix for thrombophilia

A

FBC, clotting profile
- blood film

thrombophilia screen- congen

  • antithrombin - congen deficiencies
  • Protein C and S- congen deficiency of anticoagulant factors
  • lupus anticoagulant (antiphospholipid syndrome)
  • factor V Leiden- mutation of factor V
  • prothrombin gene mutation
  • anti beta 2 glycoprotein-1 antibodies
  • anti-cadiolipin antibodies (antiphospholipid syndrome)
144
Q

Management of thrombophilia

A
  • tx undelrying cause
  • warfarin, LMWH
  • IVC filter as prophylaxis for events
145
Q

What is hyperviscosity syndrome

A
  • change in cellular or protein fractions in the blood
  • eg polycythemias, multiple myeloma, leukaemia, monoclonal gammopathies, sick cell anaemia, sepsis
  • changes viscosity of the blood—> produces this set of sx
146
Q

sx of hyperviscosity syndrome

A
  • spontaneous mucosal bleeding
  • visual disturbances due to papilloedema
  • pulm oedema- SOB, hypoxia
  • HF
  • Neuro sx- headache, vertigo, reduced GCS, seizures, coma
147
Q

ix for hyperviscosity syndrome

A

blood counts- high

plasma viscosity

148
Q

management of hyperviscosity syndrome

A
  • IV fluids
    tx cause
    haematology input
    plasmapheresis/venesection
149
Q

what is antiphospholipid syndrome

A

Autoimmune disorder

  • attacks phospholipid (fat) molecules
  • causes blood to become hypercoagulable (thrombophillic)
150
Q

presentation of antiphospholipid syndrome

A
  • DVT, stroke, TIA, PE
  • HTN, migraines, visual disurbances
  • balance and mobility issues
  • speech, memory problems
  • paraesthesia
  • fatigue
  • livedo reticularis- marbeling of the skin
  • thrombophlebitis
  • unexplained miscarriage, PROM, severe pre-eclampsia
151
Q

Blood autoantibodies suggesting antiphospholipid syndrome

A

lupus anticoagulant

anti-apolipoprotein/anti- cariolipin antibodies

152
Q

tx antiphosphlolipid syndrome

A
  • lifestyle- wt loss, stop smoking, exercise, low fat diet
  • anticoags/antiplatelets, lifelong
  • acute- SC heparin
153
Q

what is haemophilia

A
  • inherited
  • deficiency in clotting factors
  • recessive and x-linked
154
Q

types of haemophilias

A
  • A- Factor VIII
  • B- Factor IX
  • C- Factor XI
155
Q

presentation of haemophilia

A
  • spontaneous/prolonged bleeding
  • bruising
  • bleeding- gums, epistaxis, haematuria
  • joint pain (arthrosis)
156
Q

ix haemophilia

A

Blood- anaemia
clotting- normal PT/fibrinogen/vWF, prolonged APTT (intrinsic pathway)

reduced factor VIII, IX, XI activity level
in haemophilia A (factor VIII), vWF also low as they;re connected

157
Q

management if haemophilia

A

avoid competitive sports/excessive manual labour
LT/pre-procedure factor infusions
TXA
regular monitoring

158
Q

what is von willebrand disease

A
  • genetic deficiency of vWF– lack of clotting
159
Q

sx vWF disease

A
  • bruising, bleeding

- menorrhagia

160
Q

ix vWF disease

A

FBC, clotting, INR
vWF levels and activity (glycoprotein Ib binding assay)
factor VIII levels- also low, as these 2 factors are connected

161
Q

management of vWF disease

A

desmopressin- vassopressin, stimulates vwf release, may also increase factor VIII
TXA
vWF concentrate prior to surgery

162
Q

What is the extrinsic pathway of clotting

A
  • due to tissue trauma
  • thromboplastin/tissue factor is released
  • factor Vii–> Viia
  • Viia + TF+ Ca2+ activate Factor X–> prothrombin activator (Xa)
  • prothrombin (II)– Xa—> thrombin (IIa)
  • fibrinogen—IIa—> fibrin
  • crosslinked fibrin clot
163
Q

What blood test measures the extrinsic pathway

A 
Prothrombin time  (PT)
(Play Tennis OUTSIDE)

also good for common pathway

164
Q

what is the intrinsic pathway

A

damaged vessel wall

  • collagen fibres bind to vWF factor that are released from damaged endothelium
  • vWF to vWF receptors on platelets– adhesion
  • collagen also bind to collagen receptors on platelets– adhesion
Clotting cascade triggered- 
(--> XII
--> XIIa + kininogen
--> XIa + Ca 2+
--> IXa + VIIIa + Ca2+
---> Xa + Ca2+ + V)
---> prothrombin (II)
---> thrombin (IIa)
--> fibrinogen (I)
--> fibrin (Ia)
165
Q

what is the common pathway

A
  • propthrombin activator (Xa) activated prothrombin (II) into thrombin (IIa)
  • leading to fibrinogen (I) into firbin (Ia)
  • ca+ ions and fibrin stabilising factor (XIII) stabilise fibrin into fibrin polymers
  • forms mesh
  • this attracts more platelets, phospholipids
  • platelet adhesion– haemostasis, stable clot
166
Q

how does fibrinolysis occur

A

plasminogen produced by liver

  • factors XIa and XIIa make plasminogen into plasmin
  • plasmin breaks down fibrin into d-dimers
167
Q

what tests tests the intrinsic pathway

A

Activated partial thromboplastin time (APTT)

Play Table Tennis INSIDE

168
Q

what does thrombin time mean

A

how fast fibrinogen (I) is converted into fibrin (Ia) by thrombin (IIa)

169
Q

What is international normalised ratio (INR)

A

ratio of PT:normal

170
Q

what types of leucocytes are there

A

Granulocytes

agranulocytes

171
Q

what types of granulocytes are there

A

Neutrophils (most)
Eosinophils
Basophils (least)

172
Q

when are neutrophils raised

A

bacterial infections

  • stress
  • cancers
  • trauma
  • inflammation
173
Q

when are eosinophils raised

A

parasitic infetions

allergic reactions

174
Q

when are basophils raised

A

allergens

175
Q

What types of agranulocytes are there

A

Lymphocytes (most)

monocytes

176
Q

When are lymphocytes raised

A

viral infections

177
Q

when are monocytes raised

A

bacterial infecitons

178
Q

What 2 cell progenitors can a multipotent haematopoietic stemm cell/haemocytoplast turn into

A

common myeloid progenitor

common lymphoid progenitor

179
Q

what cells can a common myeloid progenitor turn into

A
  • Megakaryocyte (thrombocytes/plts)
  • erythrocytes
  • mast cells
  • myeloblasts
180
Q

what can a myeloblast become

A
  • basophil
  • neutrophil
  • eosinophil
  • monocyte (–> macrophage)
181
Q

what cells can a common lymphoid progenitor turn into

A 
Natural killer cell
Small lymphocyte (--> T and B)
182
Q

What cell type do plasma cells derive from

A

B lymphocyte

183
Q

What 2 leucocytes are phagocytic

A

neutrophils, monocytes

184
Q

function of neutrophils

A
  • phagocytosis

- attract monocytes

185
Q

function of eosinophils

A
  • produce histamine and enzymes that inactivate inflammaotry agents released by mast cells
186
Q

histological appearance of neutrophil

A
  • multilobed, purple nucleus
187
Q

histological appearance of eosinophils

A
  • bilobed, sausage shaped nucleus
188
Q

functin of basophils

A
  • allergen response
  • contain histamine granules that cause local inflammatory response via interaction with IgE (similar function to mast cells)
189
Q

appearance of basophils histologically

A

bilobed, S shaped nucleus

- lots of blue staining granules in cytoplasm

190
Q

funciton of different types of lymphocytes

A

NKC
- non specific, innate immunity

T cells

  • formed in bone marrow, mature in thymus
  • acquired/adaptive immune system
  • once active, cytotoxic t cells attack non self/infected self
  • helper t cells activate b cells and form memory t cells

B cells

  • form and mature in bone marrow
  • adaptive immune system
  • secrete antibodies
  • develop into plasma/memory cells which make antibodies
191
Q

histological appearance of lymphocytes

A
  • round, densely staining nuclei, takes up most of cytoplasmic space
192
Q

what is the funciton of monocytes

A
  • release cytokines, modulates immune response
  • can diff. into dendritic cells, which assist t cell acitvation
  • diff. into macrophages which then phagocytose microorgs
  • can transform into osteoclasts
193
Q

appearance of monocytes histologically

A
  • kidney shaped nucleus
194
Q

how many Hb molecules does one RBC have in it

A

~27 million

195
Q

structure of Hb molecule

A

4 heme groups– each composed of a ringlike porphyrin ring which an iron atom is attached and one O2 or CO2 molecule can bind to

2 beta chains, 2 alpha chains

196
Q

how many O2 molecules can one Hb molecule carry

A

4

197
Q

how is heme extracted from Hb

A

liver and spleen breakdown Hb–> bile pigments/bilirubin) exctreted in urine/faeces

198
Q

what is the Bohr effect

A

as CO2 binds to RBCs, the affinity for O2 decreases

199
Q

What is the haldane effect

A

reduced blood pH also causes a reduction in total binding capacity of Hb to O2

200
Q

What adhesion receptrs are on the surface of platelets

A

GPIIb-IIIa fibrinogen
vWF
GPIa-IIa collagen

201
Q

what agonist receptors are there on the surface of a platelet

A
  • Collagen
  • Thromboxane
  • ADP
  • Thrombin
202
Q

how is eruthropoiesus regulated

A

erythropoietin– sectreted by kidneys
– increased RBC production

reduced partial pressure of O2 is detected in interstitial peritubular cells, causing EPO production

203
Q

Where is Iron absorbed

A

Dueodenum

204
Q

Where is folate absorbed

A

Jejunum

205
Q

Where is B12 absorbe

A

Ileum

206
Q

how is iron absorbed

A
  • low pH of gastric acid in the proximal duodenum allows ferritic reductase enzyme to convert Fe3+ ferric into ferrous Fe2+
  • why PPIs reduce iron absorption
  • ferrous moves into cell
  • transferred into blood stream via ferroportin- (inhibited by hepcidin)
  • Fe2+ is reoxidised into Fe3+
  • bound to transferrin
  • transported to bone marrow for RBC production
207
Q

why does chronic disease cause anaemia

A
  1. inflammation causes liver to produce hepcidin
    hepcidin stops ferroportin from transferring ferritin into bloodstream
  2. less EPO produced from kidneys
208
Q

how is B12 aborbed

A

Parietal cells in stomach make intrinsic factors

  • this binds to B12
  • in ileum, the complex attaches to brush order in the presence of Ca2+ and alkaline pH
209
Q

what cann effect b12 absorption

A
  • abnormal ileal structure

- gastric atrophy- pernicious anaemia

210
Q

What is acute lymphoblastic leukaemia

A
  • hyperproliferation of immature lymphoblasts , affects T and B cells
211
Q

What genetic feature is assoc with acute lymphoblastic leukaemia

A
  • philidelphia chromosome
212
Q

what is the most common paediatric leukaemia

A

acute lymphoblastic leukaemia

213
Q

sx of acute lymphoblastic leukaemia

A

bone marrow:

  • infections
  • Anaemia- weak, fatigue, SOB pale
  • bleeding
  • fever
  • wt loss
  • easy bruising
  • painful bones/joints
214
Q

signs of acute lymphoblastic leukaemia

A
  • lymphadenopathy
  • hepatosplenomegaly
  • SVCO- hgih JVP, facial oedema
215
Q

Ix for ?acute lymphoblastic leukaemia

A
  • blood film
  • biopsy- marrow, node
  • PCR
216
Q

treatment of acute lymphoblastic leukaemia

A
  • bone marrow transplant
  • allopruinol
  • abx antivirals, antifungals
  • vincristine, prednisolone, methotrexate
217
Q

what is Chronic lymphoblastic leukaemia

A

accumulation of b lymphocytes in blood, marrow, nodes

218
Q

who does chronic lymphoblastic leukaemia occur in

A

elderly

219
Q

sx and signs of chronic lymphoblastic leukaemia

A 
wt loss
anorexia
sweats
infection
easy bruising (bruising easily)
anaemia- SOB, fatigue

hepatosplenomegaly
lymphadenopathy- large, rubbery, non-tender

220
Q

ix for chronic lymphblastic leukaemia

A

FBC- often incidental

221
Q

tx of chronic lymphoblastic leukaemia

A
  • fludarabine with rituximab +- cyclophosphamide
  • steroids
  • anticlonal antibodies- anti VD20, rituximab
  • bruton kinase inhibitors
  • bone marrow transplant
222
Q

what is acute myeloid leukaemia

A
  • myeloblast hyperproliferation

- rapid progression

223
Q

who does acute myeloid leukaemia affect

A

adults

224
Q

symptoms of acute myeloid luekaemia

A
  • anaemia- SOB
  • infection
  • bleeding/bruising
  • fever
  • gum hypertrophy
  • skin/CNS involvement
225
Q

ix for acute myeloid leukaemia

A
  • WCC high but poor immunity

- bone marrow biopsy- Auer rods

226
Q

tx acute myeloid leukaemia

A

fluids, plts, transfusion
infection prophylaxis
daunorubicin, cytarabine
bone marrow transplant

227
Q

what is chronic myeloid leukaemia

A

uncontrolled proliferation of myeloblasts

228
Q

who gets chronic myeloid leukaemia

A

adults

philadelphia chromosome

229
Q

sx and signs of chronic myeloid leukaemia

A
  • anaemia, bruising, bleeding
  • tried, fever, sweats, wt loss
  • gout (purine breakdown)

Hepato/splenomegaly

230
Q

ix chronic myeloid leukaemia

A
  • FBC
  • Film
  • Philadelphia chromosome t(9,22)
231
Q

tx chronic myelid leukaemia

A

imatinib

bone marrow stem cell transplant

232
Q

What is lymphoma

A

cancer of the lymphocytes in the lymphatic system (lymph glands, pleen, thymus, bone marrow)

233
Q

sx of Hodgkin’s lymphoma

A 
Large, painless lymph nodes
painful after alcohol
B sx
severe itching
anaemia- SOB, fatigue
infections
easy bruising/bleeding
234
Q

signs of Hodgkin’s lymphoma

A

facial oedema
spleno/hepatomegaly
SVCO- high JVP, distanded neck vessels

235
Q

ix ?Hodgkin’s Lymphoma

A
  • blood- lymphocytosis

- lymph node biopsy- reed Sternberg

236
Q

tx Hodgkin’s lymphoma

A

Adriamycin
Bleomycin
Vinbastine
Dacarbazine

237
Q

Sx of non-Hodgkin’s lymphoma

A

Small bowel sx:

  • diarrhoea
  • vomiting
  • wt loss
  • abdo pain
fever, night sweats
anaemia
infection
bleeding
erythroderma- severe skin erythema/swelling
lymphadenopathy
238
Q

Ix for non-Hodgkin’s lymphoma

A

marrow, node biopsy
(no reed sternberg cells)

CT +- PET

239
Q

tx non-Hodgkin’s

A

nothing- watch and wait

RT
RCHOP
- rituximab- monoclinal antibody
- cyclophosphamide
- hydroxyduanorubicin
- oncovin
- prednisolone

bone marrow transplant

240
Q

What is a myeloma

A

cancer of plasma cells (differentiated B cells)

241
Q

sx and signs of myeloma

A 
CRAB
HyperCalcaemia
- stones
- psychiatric moans
- thrones- constipation, polyuria
- abdo groans- N+V,
- Painful bones
- confusion, weakness, fatigue

Renal impairment

  • haematuria, anuria/oliguria,
  • pruritis, cramping,
  • SOB/oedema,
  • insomnia, poor appetite, N+V, lethargy, wt loss

Anaemia

Bone destruction-#, OP, spinal cord compression, pain

Bone marrow failure- bleeding, infection, anaemia

**amyloidosis- large tongue, syncope, peipheral neuropathy, GI upset, carpal tunnel

hyperviscosity- headaches, visual blurring

242
Q

ix for myeloma

A 
bone marrow aspirate
bloods
- hypercalcaemia
- high Cr
- low RBC, normal MCV, neutropenia, thrombocytopenia
- low albumin
  • serum electrophoresis- high igG monoclonal protein
  • urine electrophoresis- bence jones proteins
  • blood film- rouleaux
  • serum free light chain assay- high free light chains

consider whole body MRI

243
Q

who gets myeloma

A

older patients

244
Q

tx myeloma

A
  • monitor
  • stem cell transplant with thalidomide, dexamathasone, fludarabrine, terosulfan medications

give thalidomide with corticosteroid if not for stem cell transplant

bisphosphonates- zoledronic acid
analgesia
aspirin/LMWH
flu vaccine
pneumococcal vaccine
IVIG
acyclovir
245
Q

what precursor conditions are there to myeloma

A
  • Monoclonal gammopathy of undetermined significance (MGUS)- earliest stage, no active disease, just high Ig
  • smouldering multiple myeloma- intermediate stage, higher level of monoclonal protein and abnormal plasma cells, no CRAB criteria

monitor both with MRIs, serum electrophoresis, Cr, Ca, FBC

246
Q

What pathogens cause malaria

A 
Plasmodium falciparum
Plasmodium vivax
Plasmodium ovale
Plasmodium malariae
Plasmodium knowlesi
247
Q

what parasite causes malaria most commonly

A

Plasmodium falciparum

248
Q

what is the pathophysiology of malaria

A
  • mosquito bite
  • sporozoites in saliva of mosquito enter blood
  • to the liver
  • sporozoites mature in liver and lie dormant– hypnozoites
  • mature organisms rupture and release merozoites
  • merozoites invade RBCs and undergo asexual reprod.
  • RBCs rupture and release gametocytes
  • mosquitoes ingest these
  • parasite undergoes sexual reprod.–> sporozoites
249
Q

sx of malaria

A

swinging fever

malaise, fatigue, rigors, headache
myalgia, arthralgia
sore throat, cough, LRTI
GI upset

anorexia, jaundice
children- poor feeding

hx of travelling to high prevalence country (Africa, Asia, Central/south America, Middle East)

250
Q

signs of malaria

A 
fevere
splenomegaly
hepatomegaly
jaundice
\+- abdo tenderness
251
Q

sx/signs of severe disease (P. falciparum)

A
  • reduced GCS, seizures
  • bleeding/DIC
  • SOB, hypovolaemia, shock
  • hypoglycaemia
  • AKI- oliguria, oedema, SOB, confusion, tiredness, nausea, nephrotic syndrome
  • ARDS
252
Q

ix malaria

A
  • thick/thin blood smears - GOLD STANDARD
  • antigen detection test
  • FBC- thrombocytopenia, anaemia
  • G6PD- prior to giving primaquine
  • LFTs
  • UEs
  • hypoglycaemia if severe

if ill/unstable

  • blood gas
  • cultures
  • clotting
  • urine/stool culture
  • CXR
  • LP
253
Q

tx of malaria

A

falciparum:

  • artesunate
  • artemisinin combination therapy- artemether (artemisinin derivative) with lumfantrine

non-falciparum

  • chloroquine- overdose is fatal
  • primaquine prevents relapses (check G6PD before)

inform PHE

warn family members about sx

254
Q

Conditions that may cause paraproteinaemia

A

Cancers:

  • MGUS, myeloma(Ig, plama cells)
  • chronic lymphoblastic leukaemia (b lymphocytes)
  • non-hodgkin’s

Other:

  • autoimmune- RA, scleroderma, hasimotos
  • liver- hepatitis, cirrhosis
  • infection- TB, endocarditis
255
Q

what is paraproteinaemia

A

monoclonal immunoglobulin or light chain in blood
excess immunoglobulin M component

  • produced by B cells
  • there is a group of blood disorders that are characterised by an imbalance/disproportionate proliferation of immunoglobulin producing cells
256
Q

sx of paraproteinaemia

A

think CRAB like syndrome:

  • hypercalcaemia- stones, moans, thrones, groans
  • impaired renal function- oliguria, confusion
  • normochromic, normocytic anaemia +- pancytopenia- SOB, recurrent infection
  • bone disease- back pain, osteopenia, lytic lesions
  • malaise, fatigue
  • hyperviscosity- headaches, vision
  • cardiac failure- SOB, orthopnoea, PND
  • ESR elevation
257
Q

ix for ?paraproteinaemia

A
  • serum electrophresis
  • FBC, blood film, ESR- total protein, myeloma, Ca
  • urine- bence jones
  • bone marrow biopsy
258
Q

what is amyloidosis

A
  • deposition of abnormal amyloid fibrils
259
Q

classificaiton of amyloidosis

A

Amyloid A protein (AA)

  • acute phase reactant produced by liver
  • response to cytokines
  • chronic inflammatory diseases eg RA ank spond, maligancnies
  • kidneys, liver , spleen

Primary (AL)

  • affects every organ- mainly heart, kidney, peripheral nerves
  • monoclonal bands/asymptomatic myeloma

Transthyretin

  • inherited- autosomal dominant
  • peripheral neuropathy, cardiomyopathy, kidney damage
260
Q

sx of amyloidosis

A

fatigue, weight loss

  • easy bruising (spleen)
  • SOB (lungs, heart)
  • peripheral oedema (heart)
  • sensory changes, carpal tunnel- peripheral nerves
  • postural hypotension, early satiety- autonomic
  • oliguria- kidneys
261
Q

signs amyloidosis

A
  • renal disease- nephrotic syndrome- proteinuria
  • restrictive cardiomyopathy, HF- oedema, raised JVP
  • hepatomegaly without jaundice
  • oeseophageal varices, portal HTN
  • glove stocking neuropathy
  • interstitial sounding lungs
  • racoon eye sign- spont periorbital purpura
  • non tender goitre
262
Q

ix amyloidosis

A

Serum amyloid scintigraphy- Radiolabelled serum amyloid P component- imaging amyloid deposits, diagnostic
- organ biopsy

Organ Damage:

  • urinalysis- proteinuria
  • FBC- anaemia , thrombocytopenia
  • blood film- howell jolly bodies (splenic dysfunction)
  • LFTs- normal, clotting- abnormal
  • raised ESR
  • ECG, Echo
263
Q

Management of amyloidosis

A
  • diuretics for kidney and CHF
  • erythropoetin
  • tx malignancy, inflammation, chronic infections
  • thalidomide in AL amyloidosis
  • stem cell transplant
  • liver kidney transplant
264
Q

what drugs are contraindicated in amyloidosis

A

CCB, beta blockers, digoxin

risks of toxicity (kidneys)

265
Q

differentials of pruritis and differentiating features

A

Liver disease

  • hx alcohol
  • spider naevi, bruising, palmar erythema, gynaecomastia
  • decomp sx- juandise, ascites, encephalopathy

Fe deficiency

  • pallor
  • koilonchychia
  • atrophic glossitis
  • post cricoid webs
  • angular stomatitis

Polycythemia

  • after warm baths
  • ruddy complexion
  • gout (increased cell turnover)
  • peptic ulcer disease

CKD

  • lethargy
  • pallor
  • oedema, wt gain
  • hypertension

Lymphoma

  • night sweats
  • lymph nodes
  • spleno/hepatomegaly
  • fatigue
  • hyper/hypothyoid
  • diabetes
  • pregnancy
  • old age
  • urticaria
  • eczema, scabies, psoriasis, pityriasis rosea
266
Q

management of antiphospholipid syndrome

A
  • low dose aspirin if no hx of VTE

- warfarin if prev hx of clots with a 2-3 INR target

Finals (15 decks)

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