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Finals > Haem > Flashcards

Haem Flashcards

1
Q

Myeloma

what is it

A

cancer of the plasma cells (a type of B-lymphocyte that produces antibodies)

cancer in a specific type of plasma cell results in large quantities of a single type of antibody being produced

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2
Q

Myeloma

what is multiple myeloma

A

where the myeloma affects multiple areas of the body

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3
Q

Myeloma

what is monoclonal gammopathy of undetermined significance (MGUS)

A

where there’s an XS of a single type of antibody or antibody components without other features of myeloma or cancer

may progress to myeloma so routinely followed up

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4
Q

Myeloma

what is smouldering myeloma

A

where there is progression of MGUS with higher levels of antibodies or antibody components

premalignant and more likely to progress to myeloma than MGUS

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5
Q

Myeloma

what is Waldenstrom’s macroglobulinemia

A

a type of smouldering myeloma where there is XS IgM specifically

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6
Q

Myeloma

why are they called B cells

A

they are found in the bone marrow

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7
Q

Myeloma

pathophysiology

A

genetic mutation in plasma cells causing it to rapidly and uncontrollably multiply

they produce an abundance of immunoglobulins (usually IgG)

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8
Q

Myeloma

what is a monoclonal paraprotein

A

a single type of abnormal protein

in this case, IgG produced by all the identical cancerous plasma cells

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9
Q

Myeloma

what can be found in the urine

A

Bence Jones protein

light chains of the antibody in myeloma

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10
Q

Myeloma

why is there anaemia, neutropenia and thrombocytopenia

A

Bone marrow infiltration from the cancerous plasma cells

causes suppression of the development of other blood cell lines

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11
Q

Myeloma

how is myeloma bone disease caused

A

increased osteoclast activity and suppressed osteoblast activity

caused by cytokines release from the plasma cells and the stromal cells (other bone cells) when they are in contact with the plasma cells

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12
Q

Myeloma

where are common places for myeloma bone disease to happen

A

the skull, spine, long bones and ribs

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13
Q

Myeloma

what’s the name for patches of thin bone

A

osteolytic lesions

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14
Q

Myeloma

what can osteolytic lesions lead to

A

pathological fractures

e.g. fractured femur or vertebra from minimal force

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15
Q

Myeloma

why is there hypercalcaemia in myeloma bone disease

A

osteoclast activity causes calcium to be reabsorbed from the bone into the blood

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16
Q

Myeloma

what can pts also develop

A

plasmacytomas

individual tumours made up of the cancerous plasma cells

they occur in the bones, replacing normal bone tissue or can occur outside bones in the soft tissue of the body

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17
Q

Myeloma

why do pts develop renal impairment (4)

A
  1. high levels of Ig can block the flow through the tubules
  2. hypercalcaemia impairs renal function
  3. dehydration
  4. bisphosphonates can be harmful to the kidneys
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18
Q

Myeloma

what is the normal plasma viscosity (or internal friction in the flow of blood)

A

between 1.3 and 1.7 times that of water

blood is 1,3 to 1.7 times thicker than water

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19
Q

Myeloma

why is the plasma viscosity significantly higher

A

large amounts of Ig in the blood

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20
Q

Myeloma

what issues can raised plasma viscosity cause

A
  • easy bruising
  • easy bleeding
  • reduced or loss of sight
  • purplish palmar erythema
  • heart failure
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21
Q

Myeloma

why may there be reduced sight

A

raised plasma viscosity cause vascular disease in the eye

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22
Q

Myeloma

4 key features of myeloma for exams

A

CRAB

Calcium (elevated)

Renal failure

Anaemia (normocytic, normochromic) from replacement of bone marrow

Bone lesions/pain

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23
Q

Myeloma

RFs (5)

A
  • older age
  • male
  • black african ethnicity
  • FH
  • obesity
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24
Q

Myeloma

when should you consider myeloma

A

in anyone >60 with
- persistent bone pain esp back

  • or unexplained fractures
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25
Q

Myeloma

what initial inx would you perform if suspecting myeloma

A
  • FBC: low WCC
  • Ca: raised
  • ESR: raised
  • plasma viscosity: raised
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26
Q

Myeloma

if any of the initial inx are positive or myeloma is still suspected, what do you do

A

an urgent serum protein electrophoresis

and a urine Bence-Jones protein test

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27
Q

Myeloma

what initial inx would you perform when testing for myeloma

A

BLIP

Bence-Jones protein (request urine electrophoresis)

serum-free Light-chain assay

serum Immunoglobulins

serum Protein electrophoresis

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28
Q

Myeloma

which inx is necessary to confirm dx

A

bone marrow biopsy

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29
Q

Myeloma

what imaging is required to assess for bone lesions (in order of preference)

A
  1. whole body MRI
  2. whole body CT
  3. skeletal survey (xray images of the full skeleton)

pts only require 1 inx

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30
Q

Myeloma

xray signs

A
  • punched out lesions
  • lytic lesions
  • raindrop skull
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31
Q

Myeloma

what is raindrop skull

A

x ray sign caused by many punched out (lytic) lesions throughout the skull that give the appearance of raindrops splashing on a surface

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32
Q

Myeloma

1st line trx

A

combination of chemo:

  • Bortezomid
  • Thalidomide
  • Dexamethasone
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33
Q

Myeloma

whilst on certain chemo regimes (e.g. thalidomide), what else do pts require

A

VTE prophylaxis with aspirin or LMWH as there is a higher risk of developing a thrombus

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34
Q

Myeloma

when can stem cell transplantation be used

A

as part of a clinicial trial where pts are suitable

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35
Q

Myeloma

mnx of myeloma bone disease

A
  • bisphosphonates
  • radiotherapy: can improve bone pain
  • orthopaedic surgery: can stabilise bones or treat fractures
  • cement augmentation
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36
Q

Myeloma

what is cement augmentation

A

injecting cement into vertebral fractures or lesions and can improve spine stability and pain

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37
Q

Myeloma

complications

A
  • infection
  • pain
  • renal failure
  • anaemia
  • hypercalcaemia
  • peripheral neuropathy
  • spinal cord compression
  • hyperviscocity
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38
Q

B12 insufficiency causes

A
  • insufficient dietary intake of B12

- pernicious anaemia

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39
Q

Pernicious anaemia

what is it

A

an autoimmune condition where antibodies form against the parietal cells or intrinsic factor

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40
Q

Pernicious anaemia

why do pts become B12 deficient

A

a lack of intrinsic factor prevents the absorption of B12

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41
Q

Pernicious anaemia

what produces intrinsic factor

A

parietal cells of the stomach

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42
Q

Pernicious anaemia

what does intrinsic factor do

A

essential for the absorption of B12 in the ileum

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43
Q

Pernicious anaemia

what neurological sx do pts present with in B12 deficiency

A
  • peripheral neuropathy with numbness or paraesthesia (pins and needles)
  • loss of vibration sense or proprioception
  • visual changes
  • mood or cognitive changes
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44
Q

Pernicious anaemia

1st line inx and dx

A

test for intrinsic factor antibody

gastric parietal cell antibody can also be tested but is less helpful

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45
Q

B12 dietary deficiency mnx

A

PO cyanocobalamin

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46
Q

Pernicious anaemia

why is oral replacement inadequate

A

because the problem is with absorption rather than intake

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47
Q

Pernicious anaemia

trx

A

1mg IM hydroxycobalamin

3 times weekly for 2w, then every 3m

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48
Q

Pernicious anaemia

what do you correct first? folate or B12 deficiency

A

B12 deficiency because treating pts with folic acid can lead to subacute combined degeneration of the cord

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49
Q

Pernicious anaemia

FBC results

A
  • macrocytic anaemia
  • hypersegmented polymorphs on blood film
  • low WCC + platelets
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50
Q

Pernicious anaemia

RFs (3)

A
  • female
  • autoimmune conditions: vitiligo, thyroid, T1DM, rheumatoid, addison’s
  • blood group A
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51
Q

Blood film findings

what is Anisocytosis

A

variation in size of the RBCs

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52
Q

Blood film findings

which condition is Anisocytosis seen in

A
  • myelodysplasic syndrome

- some forms of anaemia

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53
Q

Blood film findings

what are target cells

A

have a central pigmented area, surrounded by a ring of thicker cytoplasm on the outside

‘bull’s eye target’

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54
Q

Blood film findings

when can target cells be found

A
  • in iron deficiency anaemia

- in post-splenectomy

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55
Q

Blood film findings

what are Heinz Bodies

A

individual blobs seen inside RBCs caused by denatured globin

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56
Q

Blood film findings

where are Heinz Bodies seen in

A
  • G6PD deficiency

- alpha thalassaemia

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57
Q

Blood film findings

what are Howell-Jolly bodies

A

individual blobs of DNA material seen inside RBCs

normally this DNA material is removed from the spleen during circulation of RBCs

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58
Q

Blood film findings

when are Howell-Jolly bodies seen

A
  • post-splenectomy

- severe anaemia where the body is regenerating RBCs quickly

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59
Q

Blood film findings

what are reticulocytes

A

immature RBCs

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60
Q

Blood film findings

when is there an increase in reticulocytes

A

when there is rapid turnover in RBCS e.g. haemolytic anaemia

demonstrates that the bone marrow is active in replacing lost cells

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61
Q

Blood film findings

what are schistocytes and what do they indicate

A

fragments of RBCs

RBCs are being physically damaged by trauma during their journey through the blood vessels

may indicate networks of clots in small blood vessels

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62
Q

Blood film findings

when are schistocytes seen

A
  • HUS
  • DIC
  • TTP
  • replacement metallic heart valves
  • haemolytic anaemia
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63
Q

Blood film findings

what are sideroblasts

A

immature RBCs that contain blobs of iron

they occur when the bone marrow is unable to incorporate iron into the haemoglobin molecules

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64
Q

Blood film findings

which condition may sideroblasts indicate

A

myelodysplasic syndrome

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65
Q

Blood film findings

what are smudge cells

A

ruptured WBCs that occur during the process of preparing the blood film due to aged or fragile WBCs

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66
Q

Blood film findings

what can smudge cells indicate

A

CLL

chronic lymphocytic leukaemia

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67
Q

Blood film findings

what are spherocytes

A

spherical RBCs without the normal bi-concave disk space

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68
Q

Blood film findings

what do spherocytes indicate

A
  • autoimmune haemolytic anaemia

- hereditary spherocytosis

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69
Q

Haemolytic Anaemia

what is it

A

destruction of RBCs (haemolysis) leading to anaemia

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70
Q

Haemolytic Anaemia

what can inherited haemolytic anaemias cause

A

RBCs to be more fragile and break down faster than normal

leading to chronic haemolytic anaemia

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71
Q

Haemolytic Anaemia

name the inherited haemolytic anaemias (5)

A
  • hereditary spherocytosis
  • hereditary elliptocytosis
  • thalassaemia
  • sickle cell anaemia
  • G6PD deficiency
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72
Q

Haemolytic Anaemia

what can acquired haemolytic anaemias lead to

A

increased breakdown of RBCs and haemolytic anaemia

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73
Q

Haemolytic Anaemia

name 5 acquired haemolytic anaemias

A
  • autoimmune haemolytic anaemia
  • alloimmune haemolytic anaemia (transfusions reactions and haemolytic disease of newborn)
  • paroxysmal nocturnal haemoglobinuria
  • microangiopathic haemolytic anaemia
  • prosthetic valve related haemolysis
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74
Q

Haemolytic Anaemia

features (3)

A
  • anaemia
  • splenomegaly
  • jaundice
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75
Q

Haemolytic Anaemia

why is there splenomegaly

A

the spleen becomes filled with destroyed RBCs

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76
Q

Haemolytic Anaemia

why is there jaundice

A

biliruibin is released during the destruction of RBCs

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77
Q

Haemolytic Anaemia

what would the FBC count show

A

normocytic anaemia

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78
Q

Haemolytic Anaemia

what does the blood film show

A

schistocytes (fragments of RBCs)

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79
Q

Haemolytic Anaemia

what test would be positive in autoimmune haemolytic anaemia

A

Direct Coombs test

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80
Q

Haemolytic Anaemia

what is the most common inherited haemolytic anaemia in northern Europeans

A

Hereditary Spherocytosis

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81
Q

Haemolytic Anaemia

what is the inheritance pattern in Hereditary Spherocytosis

A

autosomal dominant

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82
Q

Haemolytic Anaemia

what does hereditary spherocytosis cause

A

sphere shaped RBCs that are fragile and easily break down when passing through the spleen

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83
Q

Haemolytic Anaemia

what does hereditary spherocytosis present with

A
  • jaundice
  • gallstones
  • splenomegaly
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84
Q

Haemolytic Anaemia

Hereditary Spherocytosis: in the presence of parvovirus, what does hereditary spherocytosis present with

A

aplastic crisis: abnormal decrease in reticulocytes

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85
Q

Haemolytic Anaemia

Hereditary Spherocytosis: what is shown on the blood film

A

spherocytes

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86
Q

Haemolytic Anaemia

Hereditary Spherocytosis: dx

A
  • FH
  • clinical
  • spherocytes on blood film
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87
Q

Haemolytic Anaemia

Hereditary Spherocytosis: what would the FBC show

A

raised mean corpuscular haem conc (MCHC)

raised reticulocytes due to rapid turnover of RBCs

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88
Q

Hereditary Spherocytosis

trx

A
  • folate supplementation
  • splenectomy
  • cholecystectomy if gallstones are a problem
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89
Q

Hereditary Elliptocytosis

what is it

A

very similar to hereditary spherocytosis except that the red blood cells are ellipse shaped.

autosomal dominant

presentation + mnx the same

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90
Q

Haemolytic Anaemia

G6PD Deficiency: what is it

A

defect in the RBC enzyme, G6PD

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91
Q

Haemolytic Anaemia

G6PD Deficiency: whom is it more common in

A

Mediterranean and African patients

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92
Q

Haemolytic Anaemia

G6PD Deficiency: what is the inheritance pattern

A

autosomal recessive

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93
Q

Haemolytic Anaemia

G6PD Deficiency: what can crises be triggered by

A
  • fava beans (broad beans)
  • infections
  • medications
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94
Q

Haemolytic Anaemia

G6PD Deficiency: presentation

A
  • jaundice (usually in neonatal period)
  • gallstones
  • anaemia
  • splenomegaly
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95
Q

Haemolytic Anaemia

G6PD Deficiency: what would the blood film show

A

Heinz bodies

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96
Q

Haemolytic Anaemia

G6PD Deficiency: diagnostic inx

A

G6PD enzyme assay

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97
Q

Haemolytic Anaemia

G6PD Deficiency: what medications trigger haemolysis

A
  • primaquine (an antimalarial)
  • ciprofloaxacin
  • sulfonylureas
  • sulfasalazine
  • other sulphonamide drugs
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98
Q

patient turns jaundice and becomes anaemic after eating broad beans/ developing an infection/ given antimalarials. what is it

A

G6PD deficiency

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99
Q

Haemolytic Anaemia

Autoimmune Haemolytic Anaemia (AIHA): what is it

A

occurs when antibodies are created against the pt’s RBCs

leading to destruction of the RBCs

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100
Q

Haemolytic Anaemia

Autoimmune Haemolytic Anaemia (AIHA): what are the 2 types based on

A

the temp at which the auto-antibodies function to cause the destruction of RBCs

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101
Q

Haemolytic Anaemia

Autoimmune Haemolytic Anaemia (AIHA): what is the more common type

A

warm type

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102
Q

Haemolytic Anaemia

Autoimmune Haemolytic Anaemia (AIHA): what is warm type

A

haemolysis occurs at normal or above normal temperatures

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103
Q

Haemolytic Anaemia

Autoimmune Haemolytic Anaemia (AIHA): warm type cause

A

idiopathic

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104
Q

Haemolytic Anaemia

Autoimmune Haemolytic Anaemia (AIHA): what is cold type aka

A

cold agglutinin disease

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105
Q

Haemolytic Anaemia

Autoimmune Haemolytic Anaemia (AIHA): pathophysiology of cold type

A
  • at <10°C the antibodies attach themselves to RBCs and cause them to clump (agglutination)
  • immune system activated against them and destroys RBCs
  • they get filtered and destroyed by the spleen
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106
Q

Haemolytic Anaemia

Autoimmune Haemolytic Anaemia (AIHA): what is cold type often secondary to

A
  • lymphoma
  • leukaemia
  • SLE
  • mycoplasma, EBV, CMV, HIV
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107
Q

Haemolytic Anaemia

Autoimmune Haemolytic Anaemia (AIHA): mnx

A
  • blood transfusions
  • prednisolone
  • Rituximab
  • splenectomy
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108
Q

Haemolytic Anaemia

Autoimmune Haemolytic Anaemia (AIHA): what is Rituximab

A

a monoclonal antibody against B cells

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109
Q

Haemolytic Anaemia

Alloimmune Haemolytic Anaemia: when does it occur (2 scenarios)

A

1) haemolytic transfusion reactions

2) haemolytic disease of the newborn

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110
Q

Haemolytic Anaemia

Alloimmune Haemolytic Anaemia: what are haemolytic transfusion reactions

A

foreign RBCs circulate pt’s blood –> immune reaction –> RBCs destroyed

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111
Q

Haemolytic Anaemia

Alloimmune Haemolytic Anaemia: what is haemolytic disease of the newborn

A

foreign antibodies cross placenta from mum to fetus –> maternal antibodies target antigens on RBCs of fetus –> destruction of the RBCs in the fetus + neonate

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112
Q

Haemolytic Anaemia

what is Paroxysmal Nocturnal Haemoglobinuria

A

rare

when a specific genetic mutation in the haematopoietic stem cells in the bone marrow occurs during the pt’s lifetime

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113
Q

Haemolytic Anaemia

Paroxysmal Nocturnal Haemoglobinuria: what does the mutation result in

A

loss of proteins on the surface of RBCs that inhibit the complement cascade

so there is activation of the compliment cascade on the surface of RBCs and destruction of RBCs

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114
Q

Haemolytic Anaemia

Paroxysmal Nocturnal Haemoglobinuria: what is the characteristic presentation

A

red urine in the morning containing Hb + haemosiderin

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115
Q

Haemolytic Anaemia

Paroxysmal Nocturnal Haemoglobinuria: what are they predisposed to

A
  • thrombosis

- smooth muscle dystonia (e.g. oesophageal spasm + erectile function)

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116
Q

Haemolytic Anaemia

Paroxysmal Nocturnal Haemoglobinuria: mnx

A
  • Eculizumab

- bone marrow transplantation (curative)

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117
Q

Haemolytic Anaemia

Paroxysmal Nocturnal Haemoglobinuria: what is Eculizumab

A

a monoclonal antibody that targets complement component 5 (C5) causing suppression of the complement system

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118
Q

Haemolytic Anaemia

Microangiopathic Haemolytic Anaemia (MAHA): what is it

A

where the small blood vessels have structural abnormalities that cause haemolysis of the blood cells travelling through them

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119
Q

Haemolytic Anaemia

Microangiopathic Haemolytic Anaemia (MAHA): cause

A

secondary to:

  • haemolytic uraemic syndrome
  • DIC
  • TTP
  • SLE
  • cancer
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120
Q

Haemolytic Anaemia

Prosthetic Valve Haemolysis: how does it cause haemolytic anaemia

A

the valve churns up the cells and they break down

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121
Q

Haemolytic Anaemia

what is a key complication in prosthetic heart valves and why

A

haemolytic anaemia caused by turbulence around the valve and collision of RBCs with the implanted valve

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122
Q

Haemolytic Anaemia

Prosthetic Valve Haemolysis: mnx

A
  • monitoring
  • PO iron
  • blood transfusion if severe
  • revision surgery if severe
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123
Q

Myeloproliferative Disorders

what are 3 myeloproliferative disorders to remember

A
  • Primary myelofibrosis
  • Polycythaemia vera
  • Essential thrombocythaemia
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124
Q

Myeloproliferative Disorders

what do these conditions occur due to

A

uncontrolled proliferation of a single type of stem cell

considered a type of bone marrow cancer

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125
Q

Myeloproliferative Disorders

what is primary myelofibrosis

A

proliferation of the haematopoietic stem cell

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126
Q

Myeloproliferative Disorders

what is Polycythaemia vera

A

proliferation of the erythroid cell line

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127
Q

Myeloproliferative Disorders

what is essential thrombocythaemia

A

proliferation of the megakaryocytic cell line

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128
Q

Myeloproliferative Disorders

what do they have the protentional to progress and transform into

A

acute myeloid leukaemia

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129
Q

Myeloproliferative Disorders

mutations in which genes?

A
  • JAK2
  • MPL
  • CALR
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130
Q

Myeloproliferative Disorders

what is ruxolitinib

A

JAK2 inhibitor

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131
Q

Myeloproliferative Disorders

what is myelofibrosis

A

where the proliferation of the cell line leads to fibrosis of the bone marrow

the bone marrow is replaced by scar tissue

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132
Q

Myeloproliferative Disorders

what can cause myelofibrosis

A
  • primary myelofibrosis
  • polycythaemia vera
  • essential thrombocythaemia
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133
Q

Myeloproliferative Disorders

why does fibrosis occur in the bone marrow in myelofibrosis

A

in response to cytokines that are released from the proliferating cells

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134
Q

Myeloproliferative Disorders

which particular cytokine is released from proliferating cells in myelofibrosis

A

fibroblast growth factor

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135
Q

Myeloproliferative Disorders

why can myelofibrosis lead to anaemia and leukopenia *(low WCC)

A

the fibrosis affects the production of blood cells

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136
Q

Myeloproliferative Disorders

Myelofibrosis: what is extramedullary haematopoiesis

A

when the bone marrow is replaced with scar tissue, the production of blood cells (haematopoiesis) starts to happen in other areas such as the liver and spleen

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137
Q

Myeloproliferative Disorders

Myelofibrosis: what can extramedullary haematopoiesis lead to

A

hepatomegaly and splenomegaly –> portal HTN

if occurs at spine –> spinal cord compression

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138
Q

Myeloproliferative Disorders

presentation

A

initially asymptomatic

systemic sx:

  • fatigue
  • weight loss
  • night sweats
  • fever
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139
Q

Myeloproliferative Disorders

signs and sx of underlying complications

A
  • Anaemia (except in polycythaemia)
  • Splenomegaly (abdominal pain)
  • Portal hypertension (ascites, varices and abdominal pain)
  • Low platelets (bleeding and petechiae)
  • Thrombosis common in polycythaemia and thrombocythaemia
  • Raised RBCs (thrombosis and red face)
  • Low WCC (infections)
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140
Q

Myeloproliferative Disorders

Polycythaemia vera: 3 key signs on examination

A
  1. conjunctival plethora
  2. ruddy complexion
  3. splenomegaly
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141
Q

Myeloproliferative Disorders

Polycythaemia vera: what is conjunctival plethora

A

excessive redness to the conjunctiva in the eyes

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142
Q

Myeloproliferative Disorders

FBC findings in polycythaemia vera

A

raised Hb
>185 in men
>165 in women

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143
Q

Myeloproliferative Disorders

FBC findings in primary thrombocythaemia

A

raised platelet count

>600 x 10^9/l

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144
Q

Myeloproliferative Disorders

FBC findings due to primary MF or secondary to PV or ET

A

can give variable findings

  • Anaemia
  • Leukocytosis or leukopenia (high or low white cell counts)
  • Thrombocytosis or thrombocytopenia (high or low platelet counts)
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145
Q

Myeloproliferative Disorders

what will the blood film show in myelofibrosis

A
  • teardrop-shaped RBCs
  • poikilocytosis
  • blasts
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146
Q

Myeloproliferative Disorders

blood film: what is poikilocytosis

A

varying sizes of red blood cells

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147
Q

Myeloproliferative Disorders

blood film: what are blasts

A

immature red and white cells

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148
Q

Myeloproliferative Disorders

what is the test of choice to establish a dx

A

bone marrow biopsy

testing for JAK2, MPL and CALR genes can help guide management.

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149
Q

Myeloproliferative Disorders

what will bone marrow aspiration show

A

usually ‘dry’ as the bone marrow has turned to scar tissue

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150
Q

Myeloproliferative Disorders

Primary Myelofibrosis: mnx of mild disease with minimal sx

A

monitored but not actively treated

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151
Q

Myeloproliferative Disorders

Primary Myelofibrosis: mnx

A
  • Allogeneic stem cell transplantation: potentially curative but carries risks
  • Chemo: slow progression
  • supportive: anaemia, splenomegaly, portal HTN
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152
Q

Myeloproliferative Disorders

Management of Polycythaemia Vera

A

1st line: Venesection (keep Hb in normal range)

aspirin: reduce thrombus formation
chemo: control the disease

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153
Q

Myeloproliferative Disorders

Management of Essential Thrombocythaemia

A

aspirin: reduce thrombus formation
chemo: control the disease

154
Q

Myelodysplastic Syndrome

what is it

A

myeloid bone marrow cells not maturing properly and therefore not producing healthy blood cells

155
Q

Myelodysplastic Syndrome

what does it cause

A

low levels of blood components that originate from the myeloid cell line:

  • anaemia
  • neutropenia
  • thrombocytopenia
156
Q

Myelodysplastic Syndrome

whom is it more common in

A
  • > 60 years

- pts that have had pevious trx w/ chemo or radio

157
Q

Myelodysplastic Syndrome

what is there an increased risk of it transforming into

A

acute myeloid leukaemia

158
Q

Myelodysplastic Syndrome

presentation

A

may be asymptomatic and incidentally diagnosed based on FBC

anaemia: fatigue, pallor, SOB
neutropenia: frequent/severe infections
thrombocytopenia: purpura or bleeding

159
Q

Myelodysplastic Syndrome

inx

A

FBC: anaemia, neutropenia, thrombocytopenia

blood film: blasts

160
Q

Myelodysplastic Syndrome

diagnosis

A

bone marrow aspiration and biopsy

161
Q

Myelodysplastic Syndrome

mnx

A
  • watchful waiting
  • blood transfusions if severely anaemic
  • chemo
  • stem cell transplantation
162
Q

Lymphoma

what are they

A

a group of cancers that affect the lymphocytes inside the lymphatic system

these cancerous cells proliferate within the lymph nodes and cause the lymph nodes to become abnormally large (lymphadenopathy)

163
Q

Hodgkin’s Lymphoma

what is it caused by

A

proliferation of lymphocytes

164
Q

Hodgkin’s Lymphoma

at what age does it peak

A

bimodal age distribution

20 and 75 years

165
Q

Hodgkin’s Lymphoma

RFs (4)

A
  • HIV
  • epstein-barr virus
  • autoimmune conditions: RA, sarcoidosis
  • FH
166
Q

Hodgkin’s Lymphoma

what is the key presenting symptom

A

lymphadenopathy

167
Q

Hodgkin’s Lymphoma

describe the lymph nodes

A

may be enlarged in the neck, axilla, inguinal area

non-tender
rubbery

168
Q

Hodgkin’s Lymphoma

what may some pts experience when drinking alcohol

A

pain in the lymph nodes

169
Q

Hodgkin’s Lymphoma

what are the systemic symptoms

A

B symptoms:

  • fever
  • weight loss
  • night sweats
170
Q

Hodgkin’s Lymphoma

sx (excluding the B sx)

A
  • fatigue
  • itching
  • cough
  • SOB
  • abdo pain
  • recurrent infections
171
Q

Hodgkin’s Lymphoma

what blood test is often raised

A

lactate dehydrogenase (LDH)

172
Q

Hodgkin’s Lymphoma

why is raised LDH not diagnostic

A

because it’s not specific and can be raised in other cancers and many non-cancerous diseases

173
Q

Hodgkin’s Lymphoma

what is the key diagnostic test

A

lymph node biopsy

174
Q

Hodgkin’s Lymphoma

what is the key finding from lymph node biopsy

A

Reed-Sternberg cells

175
Q

Hodgkin’s Lymphoma

what are Reed- Sternberg cells

A

abnormally large B cells

that have multiple nuclei

that have nucleoli inside them

appearance of an owl with large eyes

176
Q

Hodgkin’s Lymphoma

what inx can be used for diagnosing and staging

A

CT, MRI, PET

177
Q

what staging is used for lymphomas

178
Q

Lymphomas

Ann Arbor: Stage 1

A

confined to 1 region of lymph nodes

179
Q

Lymphomas

Ann Arbor: Stage 2

A

> 1 region but on the same side of the diaphragm

180
Q

Lymphomas

Ann Arbor: Stage 3

A

affects lymph nodes both above and below the diaphragm

181
Q

Lymphomas

Ann Arbor: Stage 4

A

widespread involvement inc non-lymphatic organs such as the lungs or liver

182
Q

Hodgkin’s Lymphoma

key trx

A

chemo and radio

183
Q

Hodgkin’s Lymphoma

what does chemo create a risk of

A

leukaemia and infertility

184
Q

Hodgkin’s Lymphoma

what is the risk of radiotherapy

A
  • creates a risk of cancer
  • damage to tissues
  • hypothyroidism
185
Q

Non-Hodgkin’s Lymphoma

what is Burkitt lymphoma associated with

A
  • Epstein-Barr virus
  • malaria
  • HIV
186
Q

Non-Hodgkin’s Lymphoma

what does MALT lymphoma affect

A

the mucosa-associated lymphoid tissue, usually around the stomach

187
Q

Non-Hodgkin’s Lymphoma

what is MALT lymphoma associated with

A

H.pylori infection

188
Q

Non-Hodgkin’s Lymphoma

how does diffuse large B cell lymphomas often present as

A

a rapidly growing painless mass in pts over 65 years

189
Q

Non-Hodgkin’s Lymphoma

RFs (6)

A
  • HIV
  • Epstein-Barr Virus
  • H.pylori (MALT lymphoma)
  • Hep B or C infection
  • exposure to pesticides
  • FH
190
Q

Non-Hodgkin’s Lymphoma

which specific chemical is a RF

A

trichloroethylene used in several industrial processes

191
Q

Non-Hodgkin’s Lymphoma

mnx

A
  • watchful waiting
  • chemo
  • monoclonal antibodies: rituximab
  • radiotherapy
  • stem cell transplantation
192
Q

Leukaemia

what is it

A

cancer of a particular line of the stem cells in the bone marrow.

193
Q

Leukaemia

what are the 4 main types

A
  • Acute myeloid leukaemia
  • Acute lymphoblastic leukaemia
  • Chronic myeloid leukaemia
  • Chronic lymphocytic leukaemia
194
Q

Leukaemia

pathophysiology

A

genetic mutation in one of the precursor cells in the bone marrow

excessive production of a single type of abnormal white blood cell

suppression of the other cell lines causing underproduction of other cell types.

pancytopenia

195
Q

Leukaemia

what are the progressive ages of the different leukaemias from 45-75

A

“ALL CeLLmates have CoMmon AMbitions”

<5 and >45 - ALL

> 55 - CLL

> 65 - CML

> 75 - AML

196
Q

Leukaemia

presentation

A

non-specific:

  • fatigue
  • fever
  • failure to thrive
  • pallor
  • petechiae, abnormal bruising
  • abnormal bleeding
  • lymphadenopathy
  • hepatosplenomegaly
197
Q

Leukaemia

what is petechiae caused by

A

bleeding under the skin due to thrombocytopenia (low platelets)

198
Q

Leukaemia

Ddx of petechiae, a non-blanching rash

A
  • Leukaemia
  • Meningococcal septicaemia
  • Vasculitis
  • Henoch-Schonlein Purpura
  • ITP
  • Non-accidental injury
199
Q

Leukaemia

initial inx

A

FBC within 48h of suspected leukaemia

200
Q

Leukaemia

diagnostic inx

A

bone marrow biopsy

201
Q

Leukaemia

what blood test is often raised

A

lactate dehydrogenase (LDH)

202
Q

Leukaemia

what may CXR show

A

infection or mediastinal lymphadenopathy

203
Q

Leukaemia

what does bone marrow aspiration involve

A

taking a liquid sample full of cells from within the bone marrow

204
Q

Leukaemia

what does bone marrow trephine involve

A

taking a solid core sample of the bone marrow

provides a better assessment of the cells and structure.

205
Q

Leukaemia

what does a bone marrow biopsy involve

A

local anaesthetic and a specialist needle. Taken from iliac crest

206
Q

Leukaemia

what is the difference between Bone marrow trephine and aspiration

A

Samples from bone marrow aspiration can be examined straight away

a trephine sample requires a few days of preparation.

207
Q

Acute Lymphoblastic Leukaemia

which type of lymphocyte is usually acutely proliferated

A

B-lymphocytes

208
Q

Acute Lymphoblastic Leukaemia

epidemiology

A
  • most common cancer in children
  • peaks around 2-4y
  • can affect adults >45
209
Q

Acute Lymphoblastic Leukaemia

what condition is it associated with

A

Down’s syndrome

210
Q

Acute Lymphoblastic Leukaemia

what would the blood film show

A

blast cells

211
Q

Acute Lymphoblastic Leukaemia

which chromosome is it associated with

A

Philadelphia chromosome (t(9:22) translocation)

212
Q

Chronic Lymphocytic Leukaemia

what is it

A

chronic proliferation of B-lymphocytes

213
Q

Chronic Lymphocytic Leukaemia

what condition can it cause

A

warm autoimmune haemolytic anaemia

214
Q

Chronic Lymphocytic Leukaemia

what can it transform into

A

high-grade lymphoma (Richter’s transformation)

215
Q

Chronic Lymphocytic Leukaemia

what would the blood film show

A

‘smear’ or ‘smudge’ cells

occurs during the process of preparing the blood film where aged or fragile white blood cells rupture and leave a smudge on the film.

216
Q

Chronic Myeloid Leukaemia

what are the 3 typical phases

A

chronic phase

accelerated phase

blast phase

217
Q

Chronic Myeloid Leukaemia

what happens in the chronic phase

A

can last 5y

asymptomatic

diagnosed incidentally with a raised WCC

218
Q

Chronic Myeloid Leukaemia

what happens in the accelerated phase

A

occurs when the abnormal blast cells take up a high proportion of the cells in the bone marrow and blood (10-20%)

symptomatic: anaemia, thrombocytopenia and become immunocompromised

219
Q

Chronic Myeloid Leukaemia

what happens in the blast phase

A

an even higher proportion of blast cells and blood (>30%).

severe sx: pancytopenia

often fatal

220
Q

Chronic Myeloid Leukaemia

what is the cytogenetic change that is characteristic of CML

A

Philadelphia chromosome (t(9:22) translocation)

221
Q

what is the most common acute leukaemia in adults

A

Acute Myeloid Leukaemia

222
Q

Acute Myeloid Leukaemia

it can be the result of a transformation from which conditions

A

myeloproliferative disorder such as polycythaemia ruby vera or myelofibrosis.

223
Q

Acute Myeloid Leukaemia

what will blood film show

A

a high proportion of blast cells

Auer rods inside cytoplasm

224
Q

Leukaemia

mnx

A

chemo + steroids primarily

radiotherapy, bone marrow transplant, surgery

225
Q

Leukaemia

complications of chemo

A
  • Failure
  • Stunted growth and development in children
  • Infections due to immunodeficiency
  • Neurotoxicity
  • Infertility
  • Secondary malignancy
  • Cardiotoxicity
  • Tumour lysis syndrome
226
Q

Leukaemia

what is tumour lysis syndrome caused by

A

the release of uric acid from cells that are being destroyed by chemotherapy

227
Q

Leukaemia

how does tumour lysis syndrome cause AKI

A

The uric acid can form crystals in the interstitial tissue and tubules of the kidneys

228
Q

Leukaemia

mnx of tumour lysis syndrome

A
  • Allopurinol or rasburicase: reduced uric acid levels

- monitor K and phosphate as these are released

229
Q

Leukaemia

tumour lysis syndrome: what can high phosphate lead to

A

low calcium

230
Q

what is the most common inherited cause of abnormal bleeding (haemophilia)

A

Von Willebrand Disease

231
Q

Von Willebrand Disease

cause

A
  • many different underlying genetic causes
  • most are autosomal dominant
  • absence or malfunctioning of VWF, a glycoprotein
232
Q

Von Willebrand Disease

what are the different types and which one is most severe

A

Type 1,2 and 3

3 is most severe

233
Q

Von Willebrand Disease

presentation

A

unusually easy, prolonged or heavy bleeding:

  • bleeding gums with brushing
  • epistaxis
  • menorrhagia
  • heavy bleeding during surgical operations
234
Q

Von Willebrand Disease

dx

A

combination of:

  • hx of abnormal bleeding
  • FH
  • bleeding assessment tools
  • lab inx
235
Q

Von Willebrand Disease

mnx

A

does not require day to day treatment. Mnx for response to major bleeding or trauma:

  • desmopressin
  • IV VWF
  • Factor VIII
236
Q

Von Willebrand Disease

how may women with VWD that suffer from heavy periods be managed

A
  • Tranexamic acid
  • Mefanamic acid
  • Norethisterone
  • COCP
  • Mirena coil
  • Hysterectomy if severe
237
Q

Thrombocytopenia

what is it

A

low platelet count.

238
Q

Thrombocytopenia

what can it be split into

A

problems with production or destruction

239
Q

Thrombocytopenia

causes (problems with production)

A
  • sepsis
  • B12 or folic acid deficiency
  • liver failure causes reduced thrombopoietin production in the liver
  • leukaemia
  • myelodysplastic syndrome
240
Q

Thrombocytopenia

causes (problems with destruction)

A
  • medications (valproate, methotrexate, isotretinoin, antihistamines, PPI)
  • alcohol
  • ITP
  • TTP
  • HIT
  • HUS
241
Q

Thrombocytopenia

presentation

A
  • easy or spontaneous bruising

- prolonged bleeding times

242
Q

Thrombocytopenia

what is a platelet count below 10 x 10^9/L high risk for

A

spontaneous bleeding:

  • Spontaneous intracranial haemorrhage
  • GI bleeds
243
Q

Thrombocytopenia

Ddx of abnormal or prolonged bleeding

A
  • thrombocytopenia
  • Haemophilia A and B
  • VWD
  • DIC (usually secondary to sepsis)
244
Q

Thrombocytopenia

what is Immune Thrombocytopenic Purpura (ITP) aka

A

autoimmune thrombocytopenic purpura

idiopathic thrombocytopenic purpura

primary thrombocytopenic purpura.

245
Q

Thrombocytopenia

what is ITP

A
  • antibodies are created against platelets
  • this causes an immune response against platelets
  • resulting in destruction of platelets and a low platelet count
246
Q

Thrombocytopenia

mnx options of ITP

A
  • prednisolone
  • IV IG
  • Rituximab
  • splenectomy
247
Q

Thrombocytopenia

safety net advice for pts with ITP

A

seek help if signs of bleeding:

  • persistent headaches
  • melaena
  • control BP
  • suppress menstrual periods
248
Q

Thrombocytopenia

what is Thrombotic Thrombocytopenic Purpura (TTP)

A

tiny blood clots develop throughout the small vessels (microangiopathy)

using up platelets and causing thrombocytopenia, bleeding under the skin and other systemic issues

249
Q

Thrombocytopenia

why do blood clots develop in TTP

A

problem with the protein, ADAMTS13

250
Q

Thrombocytopenia

TTP: what does the protein, ADAMTS13 normally do

A
  • inactivates vWF
  • reduced platelet adhesion to vessel walls
  • reduces clot formation
251
Q

Thrombocytopenia

TTP: why is there haemolytic anaemia

A

The blood clots in the small vessels break up red blood cells

252
Q

Thrombocytopenia

TTP: what can deficiency in ADAMS13 be due to

A
  • inherited genetic mutation

- autoimmune: antibodies are created against the protein

253
Q

Thrombocytopenia

TTP: trx

A
  • plasma exchange
  • steroids
  • rituxumab
254
Q

Thrombocytopenia

what is Heparin induced thrombocytopenia (HIT)

A

the development of antibodies against platelets in response to exposure to heparin

255
Q

Thrombocytopenia

HIT: which protein on the platelets do the heparin induced antibodies specifically target

A

platelet factor 4 (PF4)

they become anti-PF4/heparin antibodies.

256
Q

Thrombocytopenia

heparin with low platelets forms unexpected blood clots. What could this be

257
Q

Thrombocytopenia

HIT: dx

A

testing for the HIT antibodies in the patients blood

258
Q

Thrombocytopenia

HIT: mnx

A
  • stop heparin

- use alternative anticoagulant

259
Q

Haemophilia

what is Haemophilia A caused by

A

a deficiency in factor VIII

260
Q

Haemophilia

what is Haemophilia B caused by

A

caused by a deficiency in factor IX.

261
Q

Haemophilia

what is Haemophilia B aka

A

Christmas Disease

262
Q

Haemophilia

what is the mode of inheritance for A and B

A

X linked recessive

263
Q

Haemophilia

who does it usually affect and why

A

males because men only require one abnormal copy as they only have one X chromosome.

Women require abnormal copies on both their X chromosomes

264
Q

Haemophilia

presentation

A

most cases present in neonates or early childhood

  • spontaneous haemorrhage
  • intracranial haemorrhage, haematomas and cord bleeding
  • abnormal bleeding
265
Q

Haemophilia

how may severe haemophilia present

A

Spontaneous bleeding into joints (haemoathrosis) and muscles

266
Q

Haemophilia

dx

A
  • bleeding scores
  • coagulation factor assays
  • genetic testing
267
Q

Haemophilia

mnx

A

replace clotting factor 8 or 9 by IV infusion

prophylactically or in response to bleeding

268
Q

Haemophilia

complication of factor 8 or 9 IV infusion mnx

A

formation of antibodies against the clotting factor resulting in the treatment becoming ineffective

269
Q

Haemophilia

mnx of acute episodes of bleeding or prevention of excessive bleeding during surgical procedure

A
  • Infusions of the affected factor (VIII or IX)
  • Desmopressin to stimulate the release of von Willebrand Factor
  • Antifibrinolytics such as tranexamic acid
270
Q

Anaemia

causes of microcytic anaemia

A

TAILS

  • Thalassaemia
  • Anaemia of chronic disease
  • Iron deficiency anaemia
  • Lead poisoning
  • Sideroblastic anaemia
271
Q

Anaemia

causes of normocytic anaemia

A

3As and 2Hs

  • Acute blood loss
  • Anaemia of chronic disease
  • Aplastic anaemia
  • Haemolytic anaemia
  • Hypothyroidism
272
Q

Anaemia

types of macrocytic anaemia

A

megaloblastic or normoblastic

273
Q

Anaemia

what is megaloblastic anaemia the result of

A

vitamin deficiency causes

impaired DNA synthesis preventing the cell from dividing normally

Rather than dividing it keeps growing into a larger, abnormal cell

274
Q

Anaemia

causes of megaloblastic anaemia

A
  • B12 deficiency

- folate deficiency

275
Q

Anaemia

causes of normoblastic macrocytic anaemia

A
  • alcohol
  • hypothyroidism
  • reticulocytosis (usually from haemolytic anaemia or blood loss
  • liver disease
  • drugs e.g. azathioprine
276
Q

Anaemia

generic sx

A
  • Tiredness
  • SOB
  • Headaches
  • Dizziness
  • Palpitations
  • Worsening of other conditions (angina, HF or peripheral vascular disease)
277
Q

Anaemia

sx specific to iron deficiency anaemia

A
  • Pica

- hair loss

278
Q

Anaemia

what is pica

A

dietary cravings for abnormal things such as dirt

279
Q

Anaemia

generic signs of anaemia

A
  • pale skin
  • conjunctival pallor
  • tachycardia
  • raised resp rate
280
Q

Anaemia

specific signs of iron deficiency

A
  • Koilonychia
  • Angular chelitis
  • Atrophic glossitis
  • brittle hair and nails
281
Q

Anaemia

what is Koilonychia

A

spoon shaped nails

282
Q

Anaemia

what is atrophic glossitis

A

a smooth tongue due to atrophy of the papillae

283
Q

Anaemia

what is a sign in haemolytic anaemia

284
Q

Anaemia

what sign may there be in thalassaemia

A

bone deformities

285
Q

Anaemia

what can indicate CKD

A

Oedema, hypertension and excoriations on the skin

286
Q

Anaemia

initial inx

A
  • Haemoglobin
  • MCV
  • B12
  • Folate
  • Ferritin
  • Blood film
287
Q

Anaemia

further inx for a GI cause of unexplained iron deficiency anaemia

A

urgent cancer referral for suspected GI cancer

OGD

288
Q

Anaemia

further inx if the cause is unclear

A

bone marrow biopsy

289
Q

Iron Deficiency Anaemia

where is iron mainly absorbed

A

in the duodenum + jejunum

290
Q

Iron Deficiency Anaemia

what is required to keep the iron in the soluble form
ferrous (Fe2+)

A

acid from the stomach

291
Q

Iron Deficiency Anaemia

why can PPIs interfere with iron absorption

A

When the acid drops it changes to the insoluble ferric (Fe3+) form

292
Q

Iron Deficiency Anaemia

how can coeliac disease or Crohn’s disease cause inadequate iron absorption

A

they causes inflammation of the duodenum or jejunum which is where iron is mainly absorbed

293
Q

Iron Deficiency Anaemia

causes

A
  • blood loss (most common in adults)
  • dietary insufficiency
  • poor iron absorption
  • increased requirements in pregnancy
294
Q

Iron Deficiency Anaemia

how does iron travel around the blood

A

as ferric ions (Fe3+) bound to a carrier protein called transferrin

295
Q

Iron Deficiency Anaemia

what is Total iron binding capacity (TIBC)

A

the total space on the transferrin molecules for the iron to bind

thus directly related to the amount of transferrin in the blood

296
Q

Iron Deficiency Anaemia

what is transferrin saturation

A

the proportion of the transferrin molecules that are bound to iron

= Serum Iron / Total Iron Binding Capacity

297
Q

Iron Deficiency Anaemia

what is ferritin

A

the form that iron takes when it is deposited and stored in cells

298
Q

Iron Deficiency Anaemia

when is extra ferritin released from cells

A

in inflammation e.g. infection or cancer

299
Q

Iron Deficiency Anaemia

what does low ferritin in the blood suggest

A

iron deficiency

300
Q

Iron Deficiency Anaemia

what does high ferritin in the blood suggest

A

difficult to interpret but likely to be related to inflammation rather than iron overload

301
Q

Iron Deficiency Anaemia

can a patient with a normal ferritin still have iron deficiency anaemia

302
Q

Iron Deficiency Anaemia

why is serum iron on its own not a very useful measure

A

Serum iron varies significantly throughout the day with higher levels in the morning and after eating iron containing meals

303
Q

Iron Deficiency Anaemia

what can be used as a marker for how much transferrin is in the blood

A

Total iron binding capacity (easier test to perform than measuring transferrin)

304
Q

Iron Deficiency Anaemia

do TIBC and transferrin increase or decrease in iron deficiency

305
Q

Iron Deficiency Anaemia

do TIBC and transferrin increase or decrease in iron overload

306
Q

Iron Deficiency Anaemia

what gives a good indication of the total iron in the body

A

transferrin saturation

307
Q

Iron Deficiency Anaemia

what 2 things can increase the values of all serum ferritin, iron, TIBC and transferrin saturation

A
  • Supplementation with iron

- Acute liver damage (lots of iron is stored in the liver)

308
Q

Iron Deficiency Anaemia

inx in an adult without a clear underlying cause

A

OGD and colonoscopy

309
Q

Iron Deficiency Anaemia

mnx from least invasive to most invasive

A
  1. PO iron e.g. ferrous sulfate 200mg TDS
  2. iron infusion e.g. cosmofer
  3. blood transfusion
310
Q

Iron Deficiency Anaemia

SEs of ferrous sulfate

A
  • constipation
  • black coloured stool

unsuitable where malabsorption is the cause of the anaemia

311
Q

Iron Deficiency Anaemia

SEs of iron infusion

A
  • anaphylaxis

avoid during sepsis as iron ‘feeds’ bacteria

312
Q

Thalassaemia

what does normal Hb consist of

A

2 alpha and 2 beta-globin chains.

313
Q

Thalassaemia

what does defect in alpha-globin chains lead to

A

alpha thalassaemia

314
Q

Thalassaemia

what does defects in beta-globin chains lead to

A

beta thalassaemia

315
Q

Thalassaemia

mode of inheritance

A

autosomal recessive

316
Q

Thalassaemia

why is there splenomegaly

A

RBCs are more fragile and break down more easily

the spleen collects all the destroyed RBCs and swells

317
Q

Thalassaemia

why do pts have a pronounced forehead and malar eminences (cheekbones).

A

The bone marrow expands to produce extra RBCs to compensate for the chronic anaemia

which causes a susceptibility to fractures and prominent features

318
Q

Thalassaemia

potential signs + sx

A
  • Microcytic anaemia
  • Fatigue
  • Pallor
  • Jaundice
  • Gallstones
  • Splenomegaly
  • Poor growth and development
  • Pronounced forehead and malar eminences
319
Q

Thalassaemia

dx

A
  • FBC: microcytic anaemia
  • Haemoglobin electrophoresis: globin abnormalities
  • DNA testing: genetic abnormality
320
Q

Thalassaemia

who are offered a screening test for thalassaemia

A

pregnant women at booking

321
Q

Thalassaemia

why does iron overload occur

A
  • faulty creation of RBCs
  • recurrent transfusions
  • increased absorption of iron in response to anaemia
322
Q

Thalassaemia

mnx of iron overload

A
  • monitoring serum ferritin
  • limiting transfusions
  • iron chelation
323
Q

Thalassaemia

effects of iron overload

A

similar to haemochromatosis:

  • fatigue
  • liver cirrhosis
  • infertility + impotence
  • HF
  • arthritis
  • diabetes
  • osteoporosis + joint pain
324
Q

Thalassaemia

what is alpha-thalassaemia and what Ch is affected

A

defects in alpha-globin chains.

The gene coding for this protein is on Ch16.

325
Q

Thalassaemia

mnx of alpha-thalassaemia

A
  • monitor FBC
  • monitor for complications
  • blood transfusions
  • splenectomy may be performed
  • bone marrow transplant can be curative
326
Q

Thalassaemia

what is beta-thalassaemia and what Ch is affected

A

defects in beta-globin chains.

The gene coding for this protein is on Ch11

327
Q

Thalassaemia

the gene defects in beta-thalassemia can either consist of….

A

abnormal copies that retain some function or deletion genes where there is no function in the beta-globin protein at all

328
Q

Thalassaemia

what are the 3 types of beta-thalassaemia

A
  • Thalassaemia minor
  • Thalassaemia intermedia
  • Thalassaemia major
329
Q

Thalassaemia

what is beta thalassaemia minor

A

carriers: 1 abnormal + 1 normal gene

330
Q

Thalassaemia

sx of beta thalassaemia minor

A

mild microcytic anaemia

331
Q

Thalassaemia

mnx of beta thalassaemia minor

A

require monitoring and no active treatment.

332
Q

Thalassaemia

what is beta thalassaemia intermedia

A

two abnormal copies of the beta-globin gene

can either be 2 defective genes

or 1 defective gene and 1 deletion gene

333
Q

Thalassaemia

sx of beta thalassaemia intermedia

A
  • more significant microcytic anaemia
  • monitoring
  • occasional blood transfusions
  • If they need more transfusions they may require iron chelation to prevent iron overload.
334
Q

Thalassaemia

what is beta thalassaemia major

A

homozygous for the deletion genes. They have no functioning beta-globin genes at all.

335
Q

Thalassaemia

sx and signs of beta thalassaemia major

A
  • severe microcytic anaemia
  • splenomegaly
  • bone deformities
  • failure to thrive in early childhood
336
Q

Thalassaemia

mnx of beta thalassaemia major

A
  • regular transfusions
  • iron chelation
  • splenectomy
  • Bone marrow transplant can potentially be curative.
337
Q

Sickle Cell Anaemia

at around 6w of age, HbF is replaced with what

338
Q

Sickle Cell Anaemia

what abnormal variant of Hb do pts have

A

HbS which causes RBCs to be an abnormal sickle shape

339
Q

Sickle Cell Anaemia

mode of inheritance

A

autosomal recessive

340
Q

Sickle Cell Anaemia

what Ch is affected

A

there is an abnormal gene for beta-globin on Ch11

341
Q

Sickle Cell Anaemia

what is sickle-cell trait

A

1 copy of the abnormal gene for beta-globin on Ch11

usually asymptomatic

342
Q

Sickle Cell Anaemia

what is sickle-cell disease

A

2 abnormal copies of the abnormal gene for beta-globin on Ch11

343
Q

Sickle Cell Anaemia

who is sickle cell disease more common in

A

pts from areas traditionally affected by malaria such as Africa, India, the Middle East and the Caribbean

344
Q

Sickle Cell Anaemia

why is it more common in pts from areas affected by malaria

A

having sickle cell trait reduces the severity of malaria this there is a selective advantage

345
Q

Sickle Cell Anaemia

who is offered a sickle cell gene test

A
  • pregnant women at risk of being carriers

- newborn screening heel prick test at 5d of age

346
Q

Sickle Cell Anaemia

complications

A
  • anaemia
  • increased risk of infection
  • stroke
  • avascular necrosis in large joints
  • pulmonary hypertension
  • priapism
  • CKD
  • sickle cell crises
  • acute chest syndrome
347
Q

Sickle Cell Anaemia

general mnx

A
  • avoid dehydration + other triggers of crisis
  • ensure vaccines are up to date
  • penicillin V prophylaxis
  • hydroxycarbamide
  • blood transfusion for severe anaemia
  • bone marrow transplant can be curative
348
Q

Sickle Cell Anaemia

general mnx: why is hydroxycarbamide given

A

to stimulate production HbF.

it does not lead to sickling of RBCs.

This has a protective effect against sickle cell crises and acute chest syndrome.

349
Q

Sickle Cell Anaemia

what is sickle cell crises

A

an umbrella term for a spectrum of acute crises related to the condition

350
Q

Sickle Cell Anaemia

what can sickle cell crises be triggered by

A
  • infection
  • dehydration
  • cold or significant life events
351
Q

Sickle Cell Anaemia

mnx of sickle cell crises

A

supportive:

  • low threshold for admission to hospital
  • treat any infection
  • keep warm
  • keep well hydrated
  • paracetamol + ibuprofen
  • penile aspiration in priapism
352
Q

Sickle Cell Anaemia

what is vaso-occlusive crisis (aka painful crisis)

A

sickle shaped blood cells clogging capillaries causing distal ischaemia

353
Q

Sickle Cell Anaemia

signs + sx of vaso-occlusive crisis (aka painful crisis)

A
  • dehydration
  • raised haematocrit
  • pain
  • fever
  • priapism (emergency)
354
Q

Sickle Cell Anaemia

what is splenic sequestration crisis

A

RBCs blocking blood flow within the spleen

355
Q

Sickle Cell Anaemia

signs + sx of splenic sequestration crisis

A
  • enlarged + painful spleen
  • severe anaemia
  • circulatory collapse (hypovolaemic shock)
356
Q

Sickle Cell Anaemia

mnx of splenic sequestration crisis

A

blood transfusions and fluid resuscitation to treat anaemia and shock

357
Q

Sickle Cell Anaemia

what prevents splenic sequestration crisis

A

splenectomy

358
Q

Sickle Cell Anaemia

what is aplastic crisis

A

a temporary loss of the creation of new blood cells

359
Q

Sickle Cell Anaemia

what is aplastic crisis commonly triggered by

A

parvovirus B19

360
Q

Sickle Cell Anaemia

sign of aplastic crisis

A

significant anaemia

361
Q

Sickle Cell Anaemia

mnx of aplastic crisis

A

supportive

blood transfusions if necessary

usually resolves spontaneously within a week

362
Q

Sickle Cell Anaemia

a diagnosis of acute chest syndrome requires___ (2)

A
  1. fever or resp sx

2. with new infiltrates seen on CXR

363
Q

Sickle Cell Anaemia

what can acute chest syndrome be due to

A

infection (pneumonia or bronchiolitis) or

non-infective causes (pulmonary vaso-occlusion or fat emboli)

364
Q

Sickle Cell Anaemia

mnx fo acute chest syndrome

A
  • abx or antivirals for infections
  • blood transfusions for anaemia
  • incentive spirometry (machine that encourages effective + deep breathing)
  • artificial ventilation
365
Q

VTE

what is Budd-Chiari syndrome

A

where a blood clot (thrombosis) develops in the hepatic vein, blocking the outflow of blood

it causes acute hepatitis

366
Q

VTE

how does Budd-Chiari syndrome present

A
  1. abdo pain
  2. hepatomegaly
  3. ascites
367
Q

VTE

mnx of Budd-Chiari Syndrome

A
  • anticoagulation (heparin or warfarin)
  • investigating for the underlying cause of hyper-coagulation
  • treating hepatitis
368
Q

why may Rasburicase (recombinant xanithine oxidase) be given before starting chemo

A

to protect against urate build up from tumour lysis syndrome

369
Q

hypochromic, microcytic anaemia but high serum ferritin and iron levels. What is it

A

sideroblastic anaemia

370
Q

Positive green birefringence of rectal tissue with Congo red staining

what is it

A

amyloidosis

371
Q

what are roleaux

A

stacks of aggregated red blood cells

372
Q

when does roleaux occur

A

when the plasma protein concentration is high, such as in multiple myeloma

373
Q

“white out” on a chest x-ray following a blood transfusion. What is it

A

Transfusion-related acute lung injury (TRALI)

374
Q

what are burr cells

A

red blood cells with small, regularly distributed projections across the cell surface.

375
Q

causes of burr cells

A
  • liver disease
  • vitamin E deficiency
  • end-stage renal disease
  • pyruvate kinase deficiency
376
Q

what is non-haemolytic febrile transfusion reaction

A

occurs around 30-60 mins after transfusion has begun and causes fever and shivers

377
Q

why is there splenomegaly and thrombocytopenia in oesophageal varices

A

portal hypertension results in splenic enlargement and hyperfunction, and thus platelet sequestration

378
Q

what is the most common inherited thrombophilia

A

factor V Leiden

379
Q

what is factor V Leiden caused by

A

mutation in clotting factor V, which becomes resistant to inactivation by protein C

380
Q

features of factor V leiden

A

Homozygosity increases the risk of VTE 50-fold and heterozygosity increases the risk 5-fold

381
Q

woman has essential thrombocytopenia but is asthmatic so can’t take aspirin as trx. What trx is next

A

Hydroxyurea: suppresses the bone production of platelets in the bone marrow which should reduce her risk of VTE

382
Q

what would FBC show in normoblastic macrocytic anaemia

A

low platelets because of liver disease in alcoholism?

Finals (47 decks)

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