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Cancer Care > pass med haematology > Flashcards

pass med haematology Flashcards

1
Q

heparin factors affected

A

prevents activation of 2, 9, 10, 11

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2
Q

warfarin factors affected

A

affects synthesis of 2,7, 9, 10

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3
Q

DIC factors affected

A

factors 1, 2, 5, 8, 11

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4
Q

liver disease factors affected

A

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

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5
Q

increased APTT, normal PT, normal bleeding time

A

haemophilia

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6
Q

increased APTT, normal PT, increased bleeding time

A

von willebrand’s disease

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7
Q

increased APTT, increased PT, normal bleeding time

A

vit K deficiecny

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8
Q

acute intermittent porphyria genetics

A

aut dom
a defect in porphobilinogen deaminase, an enzyme involved in the biosynthesis of haem. The results in the toxic accumulation of delta aminolaevulinic acid and porphobilinogen

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9
Q

clinical fx acute intermittent porphyria

A

abdominal: abdominal pain, vomiting
neurological: motor neuropathy
psychiatric: e.g. depression
hypertension and tachycardia common

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10
Q

gender and age AIP

A

female
20-40 yr olds

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11
Q

diagnosis AIP

A

classically urine turns deep red on standing
raised urinary porphobilinogen (elevated between attacks and to a greater extent during acute attacks)
assay of red cells for porphobilinogen deaminase
raised serum levels of delta aminolaevulinic acid and porphobilinogen

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12
Q

mx AIP

A

avoid triggers
in acute attacks - IV haematin/haem arginate
IV glucose as alternative

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13
Q

acute myeloid leukaemia how common

A

most common form of leukaemia in adults

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14
Q

primary or secondary AML

A

both, can be secondary transformation from a myeloproliferative disorder

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15
Q

clinical fx of AML

A

anaemia: pallor, lethargy, weakness
neutropenia: whilst white cell counts may be very high, functioning neutrophil levels may be low leading to frequent infections etc
thrombocytopenia: bleeding
splenomegaly
bone pain

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16
Q

poor prognostic fx AML

A

> 60 years
20% blasts after first course of chemo
cytogenetics: deletions of chromosome 5 or 7

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17
Q

25 yr old with DIC/thrombocytopenia

A

type of AML called acute promyelocytic leukaemia
Auer rods (seen with myeloperoxidase stain)
fusion of PML and RAR alpha genes
good prognosis

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18
Q

classification of AML

A

French-American-British (FAB)
MO - undifferentiated
M1 - without maturation
M2 - with granulocytic maturation
M3 - acute promyelocytic
M4 - granulocytic and monocytic maturation
M5 - monocytic
M6 - erythroleukaemia
M7 - megakaryoblastic

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19
Q

acute promyelocytic leukaemia what

A

M3 subtype of AML

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20
Q

APML genes

A

translocation which causes fusion of PML and RAR alpha genes

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21
Q

antiphospholipid syndrome definition

A

an acquired disorder characterised by a predisposition to both venous and arterial thromboses, recurrent fetal loss and thrombocytopenia. It may occur as a primary disorder or secondary to other conditions, most commonly SLE

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22
Q

pregnancy complications anti-phospholipid syndrome

A

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

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23
Q

mx of antiphospholid syndrome pregnancu

A

low dose aspirin - when preg confirmed on urine then LMWH when fetal heart is seen on USS..discontinued at 34 weeks gestation

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24
Q

aplastic anaemia characteristics

A

pancytopenia and hypoplastic bone marrow

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25
Q

peak incidence of acquired aplastic anaemia

A

30 yrs old

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26
Q

clinical fx aplastic anaemia

A

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

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27
Q

causes aplastic anaemia

A

idiopathic
congenital: Fanconi anaemia, dyskeratosis congenita
drugs: cytotoxics, chloramphenicol, sulphonamides, phenytoin, gold
toxins: benzene
infections: parvovirus, hepatitis
radiation

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28
Q

categories autoimmune haemolytic anaemia

A

warm and cold types depending on what temp the antibodies best cause haemolysis

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29
Q

primary or secondary autoimmune haemolytic anaemia

A

most commonly idiopathic
secondary to lymphoproliferative disorder, infection or drugs

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30
Q

investigations autoimmune haemolytic anaemia

A

general features of haemolytic anaemia
anaemia
reticulocytosis
low haptoglobin
raised lactate dehydrogenase (LDH) and indirect bilirubin
blood film: spherocytes and reticulocytes
specific features of autoimmune haemolytic anaemia
positive direct antiglobulin test (Coombs’ test).

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31
Q

warm AIHA

A

most common type
the antibody (usually IgG) causes haemolysis best at body temperature and haemolysis tends to occur in extravascular sites, for example the spleen

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32
Q

warm AIHA causes

A

idiopathic
autoimmune disease: e.g. systemic lupus erythematosus*
neoplasia
lymphoma
chronic lymphocytic leukaemia
drugs: e.g. methyldopa

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33
Q

warm autoimmune haemolytic anaemia mx

A

treatment of any underlying disorder
steroids (+/- rituximab)

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34
Q

cold AIHA

A

usually IgM and causes haemolysis best at 4 deg C. Haemolysis is mediated by complement and is more commonly intravascular. Features may include symptoms of Raynaud’s and acrocynaosis. Patients respond less well to steroids

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35
Q

causes of cold AIHA

A

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

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36
Q

beta thalassaemia major gene

A

chromosome 11
absence of beta globulin chains

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37
Q

clinical fx beta thalassaemia major

A

presents in the first year of life with failure to thrive and hepatosplenomegaly
microcytic anaemia - fx of anaemia

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38
Q

beta thalassaemia major haemoglobin

A

HbA2 & HbF raised
HbA absent

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39
Q

beta thalassaemia major mx

A

repeated tranfusion
alongside iron chelation therapy due to possible iron overload resulting in organ failure

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40
Q

beta thalassaemia trait define

A

a reduced production rate of either alpha or beta chains

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41
Q

beta thalassaemia trait genetics

A

aut recessive

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42
Q

type of anaemia beta thalassaemia trait

A

mild hypochromic, microcytic anaemia - microcytosis is characteristically disproportionate to the anaemia
HbA2 raised (> 3.5%)

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43
Q

beta thalassaemia trait clinical fx

A

trick q - usually asx

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44
Q

possible causes target cells

A

Sickle-cell/thalassaemia
Iron-deficiency anaemia
Hyposplenism
Liver disease

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45
Q

tear drop poikilocytes possible causes

A

myelofibrosis

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46
Q

spherocytes possible causes

A

Hereditary spherocytosis
Autoimmune hemolytic anaemia

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47
Q

basophilic stippling possible causes

A

Lead poisoning
Thalassaemia
Sideroblastic anaemia
Myelodysplasia

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48
Q

jowell jolly bodies possible causes

A

hyposplenism

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49
Q

heinz bodies possible causes

A

G6PD deficiency
Alpha-thalassaemia

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50
Q

schistocytes possible causes

A

Intravascular haemolysis
Mechanical heart valve
Disseminated intravascular coagulation

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51
Q

pencil poikilocytes possible causes

A

iron def anaemia

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52
Q

burr cells possible causes

A

uraemia
pyruvate kinase deficiency

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53
Q

acanthocytes possible causes

A

Abetalipoproteinemia

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54
Q

hypersegmented neutrophils possible causes

A

megaloblastic anaemia

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55
Q

causes of hyposplenism

A

post splenectomy
coeliac disease

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56
Q

blood films hyposplenism

A

target cells
Howell-Jolly bodies
Pappenheimer bodies
siderotic granules
acanthocytes

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57
Q

iron def anaemia blood films

A

target cells
‘pencil’ poikilocytes

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58
Q

iron def anaemia + b12/folate def blood film

A

‘dimorphic’ film occurs with mixed microcytic and macrocytic cells

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59
Q

blood product transfusion complications
- immune
- infective
-lung
- heart
- other

A

immunological: acute haemolytic, non-haemolytic febrile, allergic/anaphylaxis
infective
transfusion-related acute lung injury (TRALI)
transfusion-associated circulatory overload (TACO)
other: hyperkalaemia, iron overload, clotting

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60
Q

non haemolytic febrile reaction patho

A

Thought to be caused by antibodies reacting with white cell fragments in the blood product and cytokines that have leaked from the blood cell during storage

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61
Q

non haemolytic febrile reaction clinical fx

A

Fever, chills

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62
Q

non haemolytic febrile reaction mx

A

Slow or stop the transfusion

Paracetamol

Monitor

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63
Q

minor allergic reaction patho

A

caused by foreign plasma proteins

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64
Q

clinical fx mild allergic reaction

A

pruritus, urticaria

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65
Q

mild allergic reaction mx

A

Temporarily stop the transfusion

Antihistamine

Monitor

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66
Q

anaphylaxis patho

A

patients with IgA deficiency who have anti-IgA antibodies

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67
Q

anaphylaxis clinical fx

A

Hypotension, dyspnoea, wheezing, angioedema

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68
Q

anaphylaxis mx

A

Stop the transfusion

IM adrenaline

ABC support
oxygen
fluids

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69
Q

acute haemolytic reaction patho

A

results from a mismatch of blood group (ABO) which causes massive intravascular haemolysis. This is usually the result of red blood cell destruction by IgM-type antibodies.

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70
Q

clinical fx acute haemolytic reaction

A

fever
abdo pain
hypotension

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71
Q

mx of acute haemolytic reaction

A

Stop transfusion

Confirm diagnosis
check the identity of patient/name on blood product
send blood for direct Coombs test, repeat typing and cross-matching

Supportive care
fluid resuscitation with saline

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72
Q

TACO patho

A

Excessive rate of transfusion, pre-existing heart failure

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73
Q

TACO clinical fx

A

Pulmonary oedema, hypertension

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74
Q

mx TACO

A

Slow or stop transfusion

Consider intravenous loop diuretic (e.g. furosemide) and oxygen

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75
Q

TRALI patho

A

Non-cardiogenic pulmonary oedema thought to be secondary to increased vascular permeability caused by host neutrophils that become activated by substances in donated blood

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76
Q

TRALI clinical fx

A

Hypoxia, pulmonary infiltrates on chest x-ray, fever, hypotension within 6 hours of transfusion

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77
Q

TRALI mx

A

Stop the transfusion

Oxygen and supportive care

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78
Q

packed red cells key points

A

Used for transfusion in chronic anaemia and cases where infusion of large volumes of fluid may result in cardiovascular compromise. Product obtained by centrifugation of whole blood.

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79
Q

platelet rich plasma key points

A

Usually administered to patients who are thrombocytopaenic and are bleeding or require surgery. It is obtained by low speed centrifugation.

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80
Q

platelet concentrate key points

A

Prepared by high speed centrifugation and administered to patients with thrombocytopaenia.

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81
Q

fresh frozen plasma key points

A

Prepared from single units of blood.
Contains clotting factors, albumin and immunoglobulin.
Unit is usually 200 to 250ml.
Usually used in correcting clotting deficiencies in patients with hepatic synthetic failure who are due to undergo surgery.
Usual dose is 12-15ml/Kg-1.
It should not be used as first line therapy for hypovolaemia.

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82
Q

cyroprecipitate key points

A

Formed from supernatant of FFP.
Rich source of Factor VIII and fibrinogen.
Allows large concentration of factor VIII to be administered in small volume.

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83
Q

SAG-Mannitol blood key points

A

Removal of all plasma from a blood unit and substitution with:
Sodium chloride
Adenine
Anhydrous glucose
Mannitol
Up to 4 units of SAG M Blood may be administered. Thereafter whole blood is preferred. After 8 units, clotting factors and platelets should be considered.

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84
Q

which blood products must be cross matched

A

Packed red cells
Fresh frozen plasma
Cryoprecipitate
Whole blood

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85
Q

which blood products can be ABO incompatible in adults

A

platelets

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86
Q

cell saver devices definition

A

These collect patients own blood lost during surgery and then re-infuse it. There are two main types:
Those which wash the blood cells prior to re-infusion. These are more expensive to purchase and more complicated to operate. However, they reduce the risk of re-infusing contaminated blood back into the patient.
Those which do not wash the blood prior to re-infusion.

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87
Q

adv cell saver devices

A

avoid the use of infusion of blood from donors into patients and this may reduce risk of blood borne infection. It may be acceptable to Jehovah’s witnesses

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88
Q

contraindicated cell saver devices

A

in malignant disease for risk of facilitating disease dissemination.

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89
Q

immediate or urgent surgery in pts taking warfarin
4 options

A
  1. Stop warfarin
  2. Vitamin K (reversal within 4-24 hours)
    IV takes 4-6h to work (at least 5mg)
    Oral can take 24 hours to be clinically effective
  3. Fresh frozen plasma
    Used less commonly now as 1st line warfarin reversal
    30ml/kg-1
    Need to give at least 1L fluid in 70kg person (therefore not appropriate in fluid overload)
    Need blood group
    Only use if human prothrombin complex is not available
  4. Human Prothrombin Complex (reversal within 1 hour)
    Bereplex 50 u/kg
    Rapid action but factor 6 short half life, therefore give with vitamin K
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90
Q

CMV negative products requirement

A

Cytomegalovirus (CMV) is transmitted in leucocytes. As most blood products (except granulocyte transfusions) are now leucocyte depleted CMV negative products are rarely required.

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91
Q

irradiated blood products indication

A

Irradiated blood products are depleted of T-lymphocytes and used to avoid transfusion-associated graft versus host disease (TA-GVHD) caused by engraftment of viable donor T lymphocytes.

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92
Q

granulocyte transfusions

A

CMV negative and irradicated blood products required

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93
Q

intrauterine transfusions

A

CMV negative and irradicated

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94
Q

neonates up to 28 days post expected date of delivery

A

CMV negative and irradicated

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95
Q

Pregnancy: Elective transfusions during pregnancy (not during labour or delivery)

A

CMV negative

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96
Q

bone marrow/stem cell transplants

A

irradiated blood products

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97
Q

immunocompromised

A

irradiated blood products

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98
Q

patients with/prev hodgkin lymphoma

A

irradiated blood products

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99
Q

FFP indications

A

‘clinically significant’ but without ‘major haemorrhage’ in patients with a prothrombin time (PT) ratio or activated partial thromboplastin time (APTT) ratio > 1.5
can be used prophylactically in patients undergoing invasive surgery where there is a risk of significant bleeding

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100
Q

universal donor of FFP

A

AB - as lacks any anti-A or anti-B antibodies

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101
Q

cryoprecipitate consists

A

concentrated Factor VIII:C, von Willebrand factor, fibrinogen, Factor XIII and fibronectin, produced by further processing of Fresh Frozen Plasma (FFP).

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102
Q

cryoprecipitate indication

A

most suited for patients for ‘clinically significant’ but without ‘major haemorrhage’ who have a fibrinogen concentration < 1.5 g/L, eg: DIC, liver failure, hypofibrinogenaemia secondary to massive transfusion, emergency for haemophiliacs or VWB disease
an be used prophylactically in patients undergoing invasive surgery where there is a risk of significant bleeding where the fibrinogen concentration < 1.0 g/L

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103
Q

prothrombin complex concentrate indication

A

emergency reversal of anticoagulation in patients with either severe bleeding or a head injury with suspected intracerebral haemorrhage
can be used prophylactically in patients undergoing emergency surgery depending on the particular circumstance

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104
Q

transfusion threshold for red cells
- patient with
- patient without ACS

A

without - 70g/L
with - 80g/L

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105
Q

target after transfusion
- patient without ACS
- patient with ACS

A

without- 70-90g/L
with - 80-100g/L

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106
Q

red cells stored

A

at 4 degrees prior to infusion

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107
Q

non urgent scenario how long unit of red cell transferred

A

90-120 mins

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108
Q

burkitt’s lymphoma type of cancer

A

high grade B cell neoplasm

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109
Q

burkitt’s lymphoma 2 major forms

A

endemic (African) form: typically involves maxilla or mandible
sporadic form: abdominal (e.g. ileo-caecal) tumours are the most common form. More common in patients with HIV

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110
Q

gene burkitt’s lymphoma

A

c-myc gene translocation, usually t(8:14)

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111
Q

infection associated with which type of burkitts lymphoma

A

EBV - african form

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112
Q

microscopic findings burkitt’s

A

‘starry sky’ appearance: lymphocyte sheets interspersed with macrophages containing dead apoptotic tumour cells

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113
Q

burkitt’s mx

A

chemo

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114
Q

complication of tx of burkitt’s and how prevented

A

tumour lysis syndrome
Rasburicase - increases renal excretion given before chemo

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115
Q

complications of tumour lysis syndrome

A

hyperkalaemia
hyperphosphataemia
hypocalcaemia
hyperuricaemia
acute renal failure

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116
Q

chronic lymphocytic leukaemia complications

A

anaemia
hypogammaglobulinaemia leading to recurrent infections
warm autoimmune haemolytic anaemia in 10-15% of patients
transformation to high-grade lymphoma (Richter’s transformation)

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117
Q

richter’s transformation

A

occurs when leukaemia cells enter the lymph node and change into a high-grade, fast-growing non-Hodgkin’s lymphoma. Patients often become unwell very suddenly.

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118
Q

richter’s transformation sx

A

lymph node swelling
fever without infection
weight loss
night sweats
nausea
abdominal pain

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119
Q

chronic lymphoctic leukaemia caused by

A

a monoclonal proliferation of well-differentiated lymphocytes which are almost always B-cells (99%

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120
Q

CLL how common

A

most common form of leukaemia in adults

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121
Q

CLL clinical fx

A

often none: may be picked up by an incidental finding of lymphocytosis
constitutional: anorexia, weight loss
bleeding, infections
lymphadenopathy more marked than chronic myeloid leukaemia

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122
Q

CLL FBC

A

lymphocytosis
anaemia: may occur either due to bone marrow replacement or autoimmune hemolytic anaemia (AIHA)
thrombocytopenia: may occur either due to bone marrow replacement or immune thrombocytopenia (ITP)

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123
Q

CLL blood film

A

smudge cells (also known as smear cells)

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124
Q

immunophenotyping CLL

A

most cases can be identified using a panel of antibodies specific for CD5, CD19, CD20 and CD23

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125
Q

chronic myeloid leukaemia genetics

A

Philadelphia chromosome is present in more than 95% of patients with chronic myeloid leukaemia (CML). It is due to a translocation between the long arm of chromosome 9 and 22 - t(9:22)(q34; q11). This results in part of the ABL proto-oncogene from chromosome 9 being fused with the BCR gene from chromosome 22. The resulting BCR-ABL gene codes for a fusion protein that has tyrosine kinase activity in excess of norma

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126
Q

CML clinical fx

A

at 60-70 yrs
anaemia: lethargy
weight loss and sweating are common
splenomegaly may be marked → abdo discomfort
an increase in granulocytes at different stages of maturation +/- thrombocytosis
decreased leukocyte alkaline phosphatase
may undergo blast transformation (AML in 80%, ALL in 20%)

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127
Q

mx options CML

A

imatinib is now considered first-line treatment
inhibitor of the tyrosine kinase associated with the BCR-ABL defect
very high response rate in chronic phase CML
hydroxyurea
interferon-alpha
allogenic bone marrow transplant

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128
Q

cryoglobulinaemia what

A

Immunoglobulins which undergo reversible precipitation at 4 deg C, dissolve when warmed to 37 deg C. One-third of cases are idiopathic

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129
Q

three types cryoglobulinaemia

A

type I (25%):
monoclonal - IgG or IgM
associations: multiple myeloma, Waldenstrom macroglobulinaemia
type II (25%)
mixed monoclonal and polyclonal: usually with rheumatoid factor
associations: hepatitis C, rheumatoid arthritis, Sjogren’s, lymphoma
type III (50%)
polyclonal: usually with rheumatoid factor
associations: rheumatoid arthritis, Sjogren’s

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130
Q

clinical fx cryoglobulinaemia

A

Raynaud’s only seen in type I
cutaneous
vascular purpura
distal ulceration
ulceration
arthralgia
renal involvement
diffuse glomerulonephritis

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131
Q

cryoglobulinaemia investigations

A

low complement - esp C4
high ESR

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132
Q

cryoglobulinaemia mx

A

treatment of underlying condition e.g. hepatitis C
immunosuppression
plasmapheresis

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133
Q

what is cryoprecipitate

A

blood product made from plasmacy

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134
Q

cryoprecipitate indications

A

massive haemorrhage and uncontrolled bleeding due to haemophilia

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135
Q

composition of cryoprecipitate

A

factor VII
fibrinogen
von willebrand factor
factor XIII

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136
Q

DVT mx
- first line
- active cancer

A

(DOACs) as first-line treatment for most people with VTE, including as interim anticoagulants before a definite diagnosis is made
the use of DOACs in patients with active cancer, as opposed to low-molecular weight heparin as was the previous recommendation

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137
Q

DVT cancer screening

A

routine cancer screening is no longer recommended following a VTE diagnosis

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138
Q

suspected DVT what do

A

a two-level DVT Wells score should be performed
if >2 likely
if 1 or less unlikely

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139
Q

if DVT likely…

A

proximal leg vein USS within 4 hours
if +ve = diagnosis made
-ve = D dimer arranged, if this is -ve alternative diagnosis should be considered
if proximal leg USS cannot be carried out within 4 hours…a D dimer performed and intermit therapeutic anticoagulation (DOAC) started while waiting for USS (which should be performed within 24 hours)
if scan is -ve but D dimer +ve = stop intermit therapeutic anticoag and offer repeat proximal leg USS 6-8 days later

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140
Q

if DVT is unlikley

A

perform D dimer within 4 hours, if not intermit anticoag used in meantime
if -ve = alternative diagnosis considered
if +ve = proximal vein USS within 4 hours, if this USS can not be carried out within 4 hours - intermit anticoag…USS carried out within 24 hours

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141
Q

D dimer tests how work

A

a point-of-care (finger prick) or laboratory-based test
age-adjusted cut-offs should be used for patients > 50 years old

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142
Q

how long anticoag DVT

A

if provoked (obvious precipitating event) - only 3 months, 6 if active cancer
if unprovoked - 6 mths

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143
Q

how assess risk of bleeding

A

ORBIT score

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144
Q

DOAC indications

A
  • prevention of stroke in non valvular AF with one of the following risk factors -
    prior stroke or transient ischaemic attack
    age 75 years or older
    hypertension
    diabetes mellitus
    heart failure
  • prevention of VTE following hip/knee surgery
  • tx of DVT and PE
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145
Q

dabigatran
- MOA
- excretion
- reversal

A

direct thrombin inhibitor
majority renal
Idarucizumab

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146
Q

rivaroxaban
- MOA
- excretion
- reversal

A

direct factor Xa inhibitor
majority liver
Andexanet alfa

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147
Q

apixaban
- MOA
- excretion
- reversal

A

direct factor Xa inhibitor
majority faecal
andexanet alfa

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148
Q

edoxaban
- MOA
- excretion
- reversal

A

direct factor Xa inhibitor
majority faecal
no authorised reversal

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149
Q

normal clotting

A

he activation of the coagulation cascade yields thrombin that converts fibrinogen to fibrin; the stable fibrin clot being the final product of hemostasis. The fibrinolytic system breaks down fibrinogen and fibrin. Activation of the fibrinolytic system generates plasmin (in the presence of thrombin), which is responsible for the lysis of fibrin clots. The breakdown of fibrinogen and fibrin results in polypeptides (fibrin degradation products). In a state of homeostasis, the presence of plasmin is critical, as it is the central proteolytic enzyme of coagulation and is also necessary for fibrinolysis.

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150
Q

DIC pathophysiology

A

In DIC, the processes of coagulation and fibrinolysis are dysregulated, and the result is widespread clotting with resultant bleeding. Regardless of the triggering event of DIC, once initiated, the pathophysiology of DIC is similar in all conditions. One critical mediator of DIC is the release of a transmembrane glycoprotein (tissue factor =TF). TF is present on the surface of many cell types (including endothelial cells, macrophages, and monocytes) and is not normally in contact with the general circulation, but is exposed to the circulation after vascular damage.
Upon activation, TF binds with coagulation factors that then triggers the extrinsic pathway (via Factor VII) which subsequently triggers the intrinsic pathway (XII to XI to IX) of coagulation.

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151
Q

DIC septic conditions

A

TF is released in response to exposure to cytokines (particularly interleukin 1), tumour necrosis factor, and endotoxin.

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152
Q

DIC extensive trauma

A

TF is also abundant in tissues of the lungs, brain, and placenta.

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153
Q

causes of DIC

A

sepsis
trauma
obstetric complications e.g. aminiotic fluid embolism or hemolysis, elevated liver function tests, and low platelets (HELLP syndrome)
malignancy

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154
Q

DIC blood picture

A

↓ platelets
↓ fibrinogen
↑ PT & APTT
↑ fibrinogen degradation products
schistocytes due to microangiopathic haemolytic anaemia

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155
Q

heparin blood results

A

normal PT
prolonged APTT
normal bleeding time
normal platelet

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156
Q

aspirin blood results

A

normal PT
normal APTT
prolonged bleeding time
normal platelet

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157
Q

warfarin blood results

A

prolonged PT
normal APTT
normal bleeding time
normal platelet

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158
Q

most common inherited thrombophilis

A

factor V Leiden (activated protein C resistance)

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159
Q

factor V leiden patho

A

gain of function mutation in factor V leiden protein
missense mutation resulting in activated factor V becoming inactivated more slowly by activated protein C than normal

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160
Q

factor V leiden risk

A

heterozygote - 4/5 fold risk of venous thrombosis
homozygotes - 10 fold risk

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161
Q

different inherited thombophilias

A

factor V leiden
prothrombin gene mutation
protein C defiency
protein S deficency
antithrombin III deficiency

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162
Q

fanconi anaemia inheritance

A

aut recessive

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163
Q

clinical fx fanconi anaemia

A

aplastic anaemia
neurological fx
short stature
thumb/radius abnormalities
cafe au lait spots

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164
Q

fanconi anaemia increased risk

A

acute myeloid leukaemia

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165
Q

G6PD deficiency geographical risk

A

mediterranean and africa

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166
Q

G6PD deficiency inheritence

A

x linked recessive

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167
Q

G6PD deficiency crisis precipitating (causing haemolysis)

A

drugs (anti-malarials: primaquine
ciprofloxacin
sulph- group drugs: sulphonamides, sulphasalazine, sulfonylureas)

infections
broad (fava) beans

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168
Q

G6PD deficiency patho

A

G6PD is the first step in the pentose phosphate pathway, which converts glucose-6-phosphate→ 6-phosphogluconolactone
this reaction also results in nicotinamide adenine dinucleotide phosphate (NADP) → NADPH
NADPH is important for converting oxidizied glutathine back to it’s reduced form
reduced glutathine protects red blood cells from oxidative damage by oxidants such as superoxide anion (O2-) and hydrogen peroxide
↓ G6PD → ↓ reduced NADPH → ↓ reduced glutathione → increased red cell susceptibility to oxidative stress

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169
Q

clinical fx G6PD deficiency

A

neonatal jaundice is often seen
intravascular haemolysis
gallstones are common
splenomegaly may be present

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170
Q

blood film G6PD def

A

heinz bodies
bite and blister cells

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171
Q

diagnosis G6PD defiency

A

G6PD enzyme assay
- checked 3 months after an acute episode of haemolysis

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172
Q

drugs thought to be safe G6PD deficiency

A

penicillins
cephalosporins
macrolides
tetracyclines
trimethoprim

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173
Q

comparison of G6PD def and hereditary spherocytosis

A

see table

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174
Q

causes of graft versus host disease

A

allogeneic bone marrow transplant
solid organ transplant
transfusion in immunocompromised

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175
Q

graft versus host disease patho

A

T cells in donor tissue mount an immune response towards host cells

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176
Q

GVHD versus Rejection

A

It is not to be confused with transplant rejection (in which recipient immune cells activate an immune response toward the donor tissue

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177
Q

graft v host disease prognosis

A

poor

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178
Q

diagnosis of GVHD criteria

A

Billingham:
The transplanted tissue contains immunologically functioning cells
The recipient and donor are immunologically different
The recipient is immunocompromised

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179
Q

risk factors graft v host

A

Poorly matched donor and recipient (particularly HLA)
Type of conditioning used prior to transplantation
Gender disparity between donor and recipient
Graft source (bone marrow or peripheral blood source associated with higher risk than umbilical cord blood)

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180
Q

acute GVHD
- onset
- affects what
- prognosis

A

onset within 100 days of transplantation
skin, liver, GI
if multi organ - worse prognosis

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181
Q

chronic GVHD
- onset
- what affect

A

can arise de novo or following acute disease
after 100 days following transplant
v varied - lung, eye, skin, GI, or other

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182
Q

clinical fx acute GVHD

A

Painful maculopapular rash (often neck, palms and soles), which may progress to erythroderma or a toxic epidermal necrolysis-like syndrome
Jaundice
Watery or bloody diarrhoea
Persistent nausea and vomiting
Can also present as a culture-negative fever

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183
Q

clinical fx chronic GVHD

A

Skin: Many manifestations including poikiloderma, scleroderma, vitiligo, lichen planus
Eye: Often keratoconjunctivitis sicca, also corneal ulcers, scleritis
GI: Dysphagia, odynophagia, oral ulceration, ileus. Oral lichenous changes are a characteristic early sign (2)
Lung: my present as obstructive or restrictive pattern lung disease

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184
Q

investigations GVHD

A

LFT - cholestatic jaundice or USS to rule out other causes
abdo USS - ribbon sign
lung function
biopsy of affected tissue

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185
Q

mx GVHD

A

IV steroids, 2nd line 0 anti TNF
(can be topical if just cutaneous)

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186
Q

granulocyte colony stimulating factors use

A

increase neutrophil counts in patients who are neutropenic secondary to chemotherapy or other factors

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187
Q

granulocyte colony stimulating factors examples

A

filgrastim
perfilgrastim

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188
Q

philadelphia chromosome

A

t(9;22)
present in > 95% of patients with CML
this results in part of the Abelson proto-oncogene being moved to the BCR gene on chromosome 22
the resulting BCR-ABL gene codes for a fusion protein which has tyrosine kinase activity in excess of normal
poor prognostic indicator in ALL

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189
Q

t(15;17)

A

seen in acute promyelocytic leukaemia (M3)
fusion of PML and RAR-alpha genes

190
Q

t(8;14)

A

seen in Burkitt’s lymphoma
MYC oncogene is translocated to an immunoglobulin gene

191
Q

t(11;14)

A

Mantle cell lymphoma
deregulation of the cyclin D1 (BCL-1) gene

192
Q

t(14;18)

A

follicular lymphoma
increased BCL-2 transcription

193
Q

EBV risk

A

Hodgkin’s and Burkitt’s lymphoma, nasopharyngeal carcinoma

194
Q

HTLV-1 risk

A

acute T cell leukaemia/lymphoma

195
Q

HIV-1 risk

A

high grade B cell lymphoma

196
Q

H.pylori risk

A

gastric Lymphoma MALT

197
Q

malaria risk

A

burkitt’s lymphoma

198
Q

hereditary causes of hareditary haemolytic anaemia

A

membrane: hereditary spherocytosis/elliptocytosis
metabolism: G6PD deficiency
haemoglobinopathies: sickle cell, thalassaemia

199
Q

acquired haemolytic anaemias immune causes

A

immune causes (Coombs-positive)
autoimmune: warm/cold antibody type
alloimmune: transfusion reaction, haemolytic disease newborn
drug: methyldopa, penicillin

200
Q

acquired haemolytic anaemias non immune causes

A

(Coombs-negative)
microangiopathic haemolytic anaemia (MAHA): TTP/HUS, DIC, malignancy, pre-eclampsia
prosthetic heart valves
paroxysmal nocturnal haemoglobinuria
infections: malaria
drug: dapsone
Zieve syndrome
rare clinical syndrome of Coombs-negative haemolysis, cholestatic jaundice, and transient hyperlipidaemia associated with heavy alcohol use, typically following a binge
typically resolves with abstinence from alcohol

201
Q

intravascular haemolysis how work

A

free haemoglobin is released which then binds to haptoglobin. As haptoglobin becomes saturated haemoglobin binds to albumin forming methaemalbumin (detected by Schumm’s test). Free haemoglobin is excreted in the urine as haemoglobinuria, haemosiderinuria

202
Q

causes of intravascular haemolysis

A

mismatched blood transfusion
G6PD deficiency*
red cell fragmentation: heart valves, TTP, DIC, HUS
paroxysmal nocturnal haemoglobinuria
cold autoimmune haemolytic anaemia

203
Q

causes of extravascular haemolyssi

A

haemoglobinopathies: sickle cell, thalassaemia
hereditary spherocytosis
haemolytic disease of newborn
warm autoimmune haemolytic anaemia

204
Q

haemophilia inheritance

A

x linked recessive

205
Q

haemophilia a and b

A

a - def of factor VIII
b - factor IX

206
Q

clinical fx haemophilia

A

haemoarthroses
haematomas
prolonged bleeding after surgery or trauma

207
Q

blood tests haemophilia

A

prolonged APTT
bleeding time, thrombin time, prothrombin time normal

208
Q

hereditary angioedema inheritence

A

aut dom

209
Q

hereditary angioedema patho

A

low plasma levels of C1 inhibitor protein. this is a multifunctional serine protease inhibitor…uncontrolled release of bradykinin…oedema of tissues

210
Q

inv hereditary angioedema

A

C1-INH level is low during an attack
low C2 and C4 levels are seen, even between attacks. Serum C4 is the most reliable and widely used screening tool

211
Q

clinical fx hereditary angioedema

A

attacks may be proceeded by painful macular rash
painless, non-pruritic swelling of subcutaneous/submucosal tissues
may affect upper airways, skin or abdominal organs (can occasionally present as abdominal pain due to visceral oedema)
urticaria is not usually a feature

212
Q

mx hereditary angioedema

A

acute - IV C1-inhibitor concentrate, fresh frozen plasma (FFP) if this is not available
prophylaxis: anabolic steroid Danazol may help

213
Q

epidemiology hereditary spheroctyosis

A

northern european

214
Q

inheritance hreditary spherocytosis

A

aut dom

215
Q

hered spherocytosis patho

A

the normal biconcave disc shape is replaced by a sphere-shaped red blood cell
red blood cell survival reduced as destroyed by the spleen

216
Q

presentation hereditary spherocytosis

A

failure to thrive
jaundice, gallstones
splenomegaly
aplastic crisis precipitated by parvovirus infection
degree of haemolysis variable
MCHC elevated

217
Q

diagnosis hereditary spherocytosis

A

‘patients with a family history of HS, typical clinical features and laboratory investigations (spherocytes, raised mean corpuscular haemoglobin concentration [MCHC], increase in reticulocytes) do not require any additional tests
if the diagnosis is equivocal the BJH recommend the EMA binding test and the cryohaemolysis test
for atypical presentations electrophoresis analysis of erythrocyte membranes is the method of choice

218
Q

mx herediatry spherocytosis
- acute and long term

A

acute haemolytic crisis:
treatment is generally supportive
transfusion if necessary
longer term treatment:
folate replacement
splenectomy

219
Q

hodgkins lymphoma def

A

a malignant proliferation of lymphocytes characterised by the presence of the Reed-Sternberg cell

220
Q

hodgkins age affects

A

30 and 70 - bimodal

221
Q

type of hodgkins
most to least common

A

nodular sclerosing - good prog
mixed cellularity - good prog
lymphocyte predominant - best prog
lymphocyte depleted - worst prog

222
Q

nodular sclerosing more common in

A

women

223
Q

nodular sclerosing associated

A

with lacunar cells

224
Q

mixed cellularity associated with

A

large number of reed-sternerg cells

225
Q

hodgkins worse prognosis B sx

A

‘B’ symptoms also imply a poor prognosis
weight loss > 10% in last 6 months
fever > 38ºC
night sweats

226
Q

hodgkins poor prognosis other sx

A

age > 45 years
stage IV disease
haemoglobin < 10.5 g/dl
lymphocyte count < 600/µl or < 8%
male
albumin < 40 g/l
white blood count > 15,000/µl

227
Q

risk factors hodgkins

A

HIV
EBV

228
Q

clinical fx hodgkins

A

lymphadenopathy (75%)
most commonly in the neck (cervical/supraclavicular) > axillary > inguinal
usually painless, non-tender, asymmetrical
alcohol-induced lymph node pain is characteristic of Hodgkin’s lymphoma but is seen in less than 10% of patients
systemic - ‘B symptoms’ (25%)
weight loss
pruritus
night sweats
fever (Pel-Ebstein)
other possible presentations include a mediastinal mass
may be symptomatic (e.g. cough) or found incidentally on a chest x-ray

229
Q

diagnosis hodgkins

A

normocytic anaemia
may be multifactorial e.g. hypersplenism, bone marrow replacement by HL, Coombs-positive haemolytic anaemia etc
eosinophilia
caused by the production of cytokines e.g. IL-5
LDH raised
lymph node biopsy
Reed-Sternberg cells are diagnostic: these are large cells that are either multinucleated or have a bilobed nucleus with prominent eosinophilic inclusion-like nucleoli (thus giving an ‘owl’s eye’ appearance)

230
Q

staging hodgkins

A

Ann-Arbor staging of Hodgkin’s lymphoma
I: single lymph node
II: 2 or more lymph nodes/regions on the same side of the diaphragm
III: nodes on both sides of the diaphragm
IV: spread beyond lymph nodes

A = no systemic symptoms other than pruritus
B = weight loss > 10% in last 6 months, fever > 38c, night sweats (poor prognosis)

231
Q

hodgkins main mx and types

A

chemo - 2 combos
ABVD (doxorubicin, bleomycin, vinblastine, and dacarbazine): considered the standard regime
BEACOPP (bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, procarbazine, and prednisone): alternative regime with better remission rates but higher toxicity

232
Q

hodgkins other modes of tx

A

radiotherapy
combined modality therapy (CMT)
chemotherapy followed by radiotherapy
hematopoietic cell transplantation
may be used for relapsed or refractory classic Hodgkin lymphoma

233
Q

complications of tx hodgkins/prognosis

A

complications of treatment are therefore more of an issue for these patients as prognosis good
secondary malignancies are a risk, in particular solid tumours: breast and lung

234
Q

causes of hyposplenism

A

splenectomy
sickle-cell
coeliac disease, dermatitis herpetiformis
Graves’ disease
systemic lupus erythematosus
amyloid

235
Q

blood film hyposplenism

A

Howell-Jolly bodies
siderocytes

236
Q

ITP patho

A

an immune-mediated reduction in the platelet count. Antibodies are directed against the glycoprotein IIb/IIIa or Ib-V-IX complex.

237
Q

ITP adults v child

A

an acute thrombocytopenia that may follow infection or vaccination. In contrast, adults tend to have a more chronic condition

238
Q

ITP epidemology

A

older females

239
Q

clinical fx ITP

A

may be detected incidentally following routine bloods
symptomatic patients may present with
petechiae, purpura
bleeding (e.g. epistaxis)
catastrophic bleeding (e.g. intracranial) is not a common presentation

240
Q

investigations ITP

A

full blood count: isolated thrombocytopenia
blood film

241
Q

mx ITP

A

oral prednisolone
pooled normal human immunoglobulin (IVIG) may also be used
it raises the platelet count quicker than steroids, therefore may be used if active bleeding or an urgent invasive procedure is required
splenectomy is now less commonly used

242
Q

evan’s syndrome

A

ITP in association with autoimmune haemolytic anaemia (AIHA)

243
Q

iron def anaemia patho

A

Iron is needed to make the haemoglobin in red blood cells, therefore a deficiency of iron leads to a reduction in red blood cells/haemoglobin i.e. anaemia

244
Q

peak age iron def anaemka

A

pre school age

245
Q

causes iron def anaemia

A

blood loss - menorrhagia or GI bleed in colon cancer
inadequate diet - vegans and beg
poor gi absoprtion - coliac
increased iron requirements - pregnancy

246
Q

clinical fx iron def anaemia

A

Fatigue
Shortness of breath on exertion
Palpitations
Pallor
Nail changes: this includes koilonychia (spoon-shaped nails)
Hair loss
Atrophic glossitis
Post-cricoid webs
Angular stomatitis

247
Q

iron def anaemia inv

A

FBC - hypochromic microcytic anaemia
low serum ferritin
(can be false negative as rises in inflammation)
TIBC/transferin will be high
blood film - anisopoikilocytosis, target cells and pencil poikilocytes
endoscopy

248
Q

referral when iron def anaemia

A

Post-menopausal women with a haemoglobin level ≤10 and men with a haemoglobin level ≤11 should be referred to a gastroenterologist within 2 weeks.

249
Q

iron def anaemia mx

A

underlying causes managed
oral ferrous sulfate
iron rich diet - dark green leafy veg, meat, iron foritifed bread

250
Q

common s/e of iron supplements

A

nausea
abdo pain
constipation
diarrhoea

251
Q

iron def anaemia v anaemia of chronic disease

A

serum iron
<8 in IRON, <15 in ACD
TIBC
high in IRON, low in ACD
Transferrin saturation
low in IRON, low in ACD
ferritin
low in IRON, high in ACD

252
Q

idiopathic thrombocytopenia purpura patho

A

an immune mediated reduction in the platelet count. Antibodies are directed against the glycoprotein IIb-IIIa or Ib complex

253
Q

idiopathic thrombocytopenia purpura inv

A

antiplatelet autoantibodies (usually IgG)
bone marrow aspiration shows megakaryocytes in the marrow.

254
Q

idiopathic thrombocytopenia purpura rule out

A

leukaemia

255
Q

mx idiopathic thrombocytopenic purpura

A

oral prednisolone (80% of patients respond)
splenectomy if platelets < 30 after 3 months of steroid therapy
IV immunoglobulins
immunosuppressive drugs e.g. cyclophosphamide

256
Q

decreased haptoglobin

A

intravascular haemolysis

257
Q

MCHC increased

A

hereditary spherocytosis
autoimmune haemolytic anaemia

258
Q

MCHC decreased

A

microcytic anaemia

259
Q

abdo pain and neuro signs ddx

A

acute intermittent porphyria
lead poisoning

260
Q

lead poisoning patho

A

defective ferrochelatase and ALA dehydratase function.

261
Q

lead poisoning clinical fx

A

abdominal pain
peripheral neuropathy (mainly motor)
neuropsychiatric features
fatigue
constipation
blue lines on gum margin

262
Q

inv lead poisoning

A

blood level - >10 mcg/dl
microcytic anameia
blood film - basophilic strippling and clover leaf
raised serum and urine levels of delta aminolaevulinic acid
urine coproporphyrin increased

263
Q

mx lead poisoning

A

chelating agents - dimercaptosuccinic acid (DMSA)
D-penicillamine
EDTA
dimercaprol

264
Q

infective causes of generalised lymphadenopathy

A

infectious mononucleosis
HIV, including seroconversion illness
eczema with secondary infection
rubella
toxoplasmosis
CMV
tuberculosis
roseola infantum

265
Q

neoplastic causes of generalised lymphadenopathy

A

leukaemia
lymphoma

266
Q

other causes of generalised lymphadenopathy

A

autoimmune conditions: SLE, rheumatoid arthritis
graft versus host disease
sarcoidosis
drugs: phenytoin and to a lesser extent allopurinol, isoniazid

267
Q

lymphatic drainage of ovaries

A

para-aortic lymphatics

268
Q

uterus lymphatic drainage

A

within broad ligament to iliac LN’s

269
Q

cervic ln drainage

A

laterally through the broad ligament to the external iliac nodes, along the lymphatics of the uterosacral fold to the presacral nodes and posterolaterally along lymphatics lying alongside the uterine vessels to the internal iliac nodes.

270
Q

megaloblastic causes of macrocytic anaemia

A

vitamin B12 deficiency
folate deficiency
e.g. secondary to methotrexate

271
Q

normoblastic causes of macrocytic anaemia

A

alcohol
liver disease
hypothyroidism
pregnancy
reticulocytosis
myelodysplasia
drugs: cytotoxics

272
Q

aged 0-24 yrs symptoms urgent referral

A

within 48 hours to investigate for leukaemia -
Pallor
Persistent fatigue
Unexplained fever
Unexplained persistent infections
Generalised lymphadenopathy
Persistent or unexplained bone pain
Unexplained bruising
Unexplained bleeding

273
Q

methaemoglobinaemia patho

A

haemoglobin which has been oxidised from Fe2+ to Fe3+. This is normally regulated by NADH methaemoglobin reductase, which transfers electrons from NADH to methaemoglobin resulting in the reduction of methaemoglobin to haemoglobin. There is tissue hypoxia as Fe3+ cannot bind oxygen, and hence the oxidation dissociation curve is moved to the left

274
Q

congenital causes methaemoglobinaemia

A

haemoglobin chain variants: HbM, HbH
NADH methaemoglobin reductase deficiency

275
Q

acquired causes methaemoglobinaemia

A

drugs: sulphonamides, nitrates (including recreational nitrates e.g. amyl nitrite ‘poppers’), dapsone, sodium nitroprusside, primaquine
chemicals: aniline dyes

276
Q

clinical fx methoglobinaemia

A

‘chocolate’ cyanosis
dyspnoea, anxiety, headache
severe: acidosis, arrhythmias, seizures, coma
normal pO2 but decreased oxygen saturation

277
Q

mx methoglobinaemia

A

NADH methaemoglobinaemia reductase deficiency: ascorbic acid
IV methylthioninium chloride (methylene blue) if acquired

278
Q

MGUS causes

A

paraproteinaemia

279
Q

MGUS mistaken for

A

myeloma

280
Q

MGUS risk of

A

eventually develop myeloma

281
Q

clinical fx MGUS

A

usually asymptomatic
no bone pain or increased risk of infections
around 10-30% of patients have a demyelinating neuropathy

282
Q

myeloma V MGUS

A

normal immune function
normal beta-2 microglobulin levels
lower level of paraproteinaemia than myeloma (e.g. < 30g/l IgG, or < 20g/l IgA)
stable level of paraproteinaemia
no clinical features of myeloma (e.g. lytic lesions on x-rays or renal disease)

283
Q

causes of microcytic anaemia

A

iron-deficiency anaemia
thalassaemia*
congenital sideroblastic anaemia
anaemia of chronic disease (more commonly a normocytic, normochromic picture)
lead poisoning

284
Q

normal Hb, microcytic, no risk of thalassaeia

A

polycythiamia rubra vera which may cause iron def secondary to bleeding

285
Q

microcytic anaemia urgent referral

A

if new onset in elderly pts

286
Q

myelodysplastic syndrome definition

A

encompass a heterogeneous group of clonal hematopoietic stem cell disorders characterized by ineffective hematopoiesis, peripheral blood cytopenias, and a risk of progression to acute myeloid leukaemia (AML).

287
Q

median age of diagnosis myelodysplastic syndrom

A

70-75

288
Q

primary or secondary myelodysplastic syndrome

A

Around 90% of cases are primary with the remaining 10% secondary to causes such as chemotherapy and radiotherapy. Secondary MDS typically develops around 5 years post-treatment.

289
Q

patho MDS

A

ineffective hematopoiesis leading to peripheral cytopenias despite a typically hypercellular bone marro

290
Q

common presentations MDS

A

fatigue, weakness, and pallor due to anaemia; recurrent infections due to neutropenia; and easy bruising or bleeding due to thrombocytopenia. Some patients may be asymptomatic and are diagnosed incidentally on routine blood counts.

291
Q

diagnosis MDS

A

peripheral blood counts, bone marrow examination, and cytogenetic analysis. Bone marrow biopsy typically shows dysplastic changes in hematopoietic cells and a varying degree of blasts. Cytogenetic analysis can identify specific chromosomal abnormalities that may have prognostic implications.

292
Q

tx MDS

A

supportive care (e.g., blood transfusions, growth factors), disease-modifying therapy (e.g., hypomethylating agents, lenalidomide), immunosuppressive therapy, and hematopoietic stem cell transplantation

293
Q

myelofibrosis definition

A

a type of myeloproliferative disorder caused by hyperplasia of abnormal megakaryocytes…release of platelet derived growth factor…stimulate fibroblasts
Haematopoeisis develops in liver and spleen

294
Q

clinical fx myelofibrosis

A

e.g. elderly person with symptoms of anaemia e.g. fatigue (the most common presenting symptom)
massive splenomegaly
hypermetabolic symptoms: weight loss, night sweats etc

295
Q

lab findings myelofibrosis

A

anaemia
high WBC and platelet count early in the disease
‘tear-drop’ poikilocytes on blood film
unobtainable bone marrow biopsy - ‘dry tap’ therefore trephine biopsy needed
high urate and LDH (reflect increased cell turnover)

296
Q

multiple myeloma definition

A

a haematological malignancy characterised by plasma cell proliferation. It arises due to genetic mutations which occur as B-lymphocytes differentiate into mature plasma cells

297
Q

myeloma age

A

70 yrs old

298
Q

myeloma clinical fx

A

CRABBI
Calcium
Renal
Anaemia
Bleeding
Bones
Infection

299
Q

CRABBI explained

A

Calcium
hypercalcaemia
primary factor: due primarily to increased osteoclastic bone resorption caused by local cytokines (e.g. IL-1, tumour necrosis factor) released by the myeloma cells
much less common contributing factors: impaired renal function, increased renal tubular calcium reabsorption and elevated PTH-rP levels
this leads to constipation, nausea, anorexia and confusion
Renal
monoclonal production of immunoglobulins results in light chain deposition within the renal tubules
this causes renal damage which presents as dehydration and increasing thirst
other causes of renal impairment in myeloma include amyloidosis, nephrocalcinosis, nephrolithiasis
Anaemia
bone marrow crowding suppresses erythropoiesis leading to anaemia
this causes fatigue and pallor
Bleeding
bone marrow crowding also results in thrombocytopenia which puts patients at increased risk of bleeding and bruising
Bones
bone marrow infiltration by plasma cells and cytokine-mediated osteoclast overactivity creates lytic bone lesions
this may present as pain (especially in the back) and increases the risk of pathological fractures
Infection
a reduction in the production of normal immunoglobulins results in increased susceptibility to infection

300
Q

investigations myeloma

A

bloods
protein electrophoresis
bone marrow aspiration
imagin

301
Q

myeloma blood

A

full blood count: anaemia
peripheral blood film: rouleaux formation
urea and electrolytes: renal failure
bone profile: hypercalcaemia

302
Q

myeloma protein electrophoriesis

A

raised concentrations of monoclonal IgA/IgG proteins will be present in the serum
in the urine, they are known as Bence Jones proteins

303
Q

myeloma bone marrow aspiration

A

plasma cells is significantly raised

304
Q

myeloma imagin

A

whole body MRI
X-rays: ‘rain-drop skull’ (likened to the pattern rain forms after hitting a surface and splashing, where it leaves a random pattern of dark spots). Note that a very similar, but subtly different finding is found in primary hyperparathyroidism - ‘pepperpot skull’

305
Q

major criteria myeloma

A

Plasmacytoma (as demonstrated on evaluation of biopsy specimen)
30% plasma cells in a bone marrow sample
Elevated levels of M protein in the blood or urine

306
Q

minor criteria myeloma

A

10% to 30% plasma cells in a bone marrow sample.
Minor elevations in the level of M protein in the blood or urine.
Osteolytic lesions (as demonstrated on imaging studies).
Low levels of antibodies (not produced by the cancer cells) in the blood.

307
Q

neutropenia count

A

<1.5 x 10^9

308
Q

neutrophil count severity

A

Mild 1.0 - 1.5 * 109
Moderate 0.5 - 1.0 * 109
Severe < 0.5 * 109

309
Q

causes of neutropenia

A

viral
HIV
Epstein-Barr virus
hepatitis
drugs
cytotoxics
carbimazole
clozapine
benign ethnic neutropaenia
common in people of black African and Afro-Caribbean ethnicity
requires no treatment
haematological malignancy
myelodysplastic malignancies
aplastic anemia
rheumatological conditions
systemic lupus erythematosus: mechanisms include circulating antineutrophil antibodies
rheumatoid arthritis: e.g. hypersplenism as in Felty’s syndrome
severe sepsis
haemodialysis

310
Q

neutropenic sepsis definition

A

defined as a neutrophil count of < 0.5 * 109 in a patient who is having anticancer treatment and has one of the following:
a temperature higher than 38ºC or
other signs or symptoms consistent with clinically significant sepsis

311
Q

causes neutropenic sepsis

A

coagulase-negative, Gram-positive bacteria are the most common cause, particularly Staphylococcus epidermidis

312
Q

prophylaxis neutropenic sepsis

A

fluroquinolone

313
Q

neutropenic sepsis mx

A

abx started immediately, not wait for WBC - Tazocin
assessed by a specialist
if patients are still febrile and unwell after 48 hours an alternative antibiotic such as meropenem is often prescribed +/- vancomycin
if patients are not responding after 4-6 days the Christie guidelines suggest ordering investigations for fungal infections (e.g. HRCT), rather than just starting therapy antifungal therapy blindly
there may be a role for G-CSF in selected patients

314
Q

lymphoma definition

A

the malignant proliferation of lymphocytes which accumulate in lymph nodes or other organs. Lymphoma may be classified as either Hodgkin’s lymphoma (a specific type of lymphoma characterized by the presence of Reed-Sternberg cells) or non-Hodgkin’s lymphoma (every other type of lymphoma that is not Hodgkin’s lymphoma).

315
Q

non hodgkins lymphoma subgroup

A

either B or T cells
further classified into high or low grade

316
Q

which type lymphoma more common

A

non hodgkin’s

317
Q

non hodgkins age

A

over 75 yrs usually
can be any age

318
Q

risk fx non hodgkins

A

Elderly
Caucasians
History of viral infection (specifically Epstein-Barr virus)
Family history
Certain chemical agents (pesticides, solvents)
History of chemotherapy or radiotherapy
Immunodeficiency (transplant, HIV, diabetes mellitus)
Autoimmune disease (SLE, Sjogren’s, coeliac disease)

319
Q

non hodgkins sx

A

Painless lymphadenopathy (non-tender, rubbery, asymmetrical)
Constitutional/B symptoms (fever, weight loss, night sweats, lethargy)
Extranodal Disease - gastric (dyspepsia, dysphagia, weight loss, abdominal pain), bone marrow (pancytopenia, bone pain), lungs, skin, central nervous system (nerve palsies

320
Q

how different non hodgkins v hodgkins

A

biopsy how differentiate
Lymphadenopathy in Hodgkin’s lymphoma can experience alcohol-induced pain in the node
‘B’ symptoms typically occur earlier in Hodgkin’s lymphoma and later in non-Hodgkin’s lymphoma
Extra-nodal disease is much more common in non-Hodgkin’s lymphoma than in Hodgkin’s lymphoma

321
Q

clinical signs non hodgkins

A

Signs of weight loss
Lymphadenopathy (typically in the cervical, axillary or inguinal region)
Palpable abdominal mass - hepatomegaly, splenomegaly, lymph nodes
Testicular mass
Fever

322
Q

non hodgkins inv

A

diagnostic - excisional node biopsy
CT TAP
HIV - risk factor
FBC and blood film - normocytic anaemia to rule out leukaemia
ESR - prognostic fx
LDH - prognostic fx

323
Q

non hodgkins staging

A

Ann arbor
Stage 1 - One node affected
Stage 2 - More than one node affected on the same side of the diaphragm
Stage 3 - Nodes affected on both sides of the diaphragm
Stage 4 - Extra-nodal involvement e.g. Spleen, bone marrow or CNS
with A or B to indicated B symptoms present or not

324
Q

non hodgkins mx

A

dependent on the specific sub-type of non-Hodgkin’s lymphoma and will typically take the form of watchful waiting, chemotherapy or radiotherapy.
Rituximab is used in combination with conventional chemotherapy regimes (e.g. CHOP) for a variety of types of NHL
All patients will receive flu/pneumococcal vaccines
Patients with neutropenia may require antibiotic prophylaxis

325
Q

complications non hodgkins

A

Bone marrow infiltration causing anaemia, neutropenia or thrombocytopenia
Superior vena cava obstruction
Metastasis
Spinal cord compression
Complications related to treatment e.g. Side effects of chemotherapy

326
Q

prognosis low grade non hodgkins

A

better

327
Q

prognosis high grade non hodgkins

A

worse but higher cure rate

328
Q

causes of normocytic anaemia

A

anaemia of chronic disease
chronic kidney disease
aplastic anaemia
haemolytic anaemia
acute blood loss

329
Q

paroxysmal nocturnal haemoglobinuria definition

A

an acquired disorder leading to haemolysis (mainly intravascular) of haematological cells. It is thought to be caused by increased sensitivity of cell membranes to complement (see below) due to a lack of glycoprotein glycosyl-phosphatidylinositol (GPI)

330
Q

PNH more prone to

A

venous thrombosis

331
Q

PNH patho

A

GPI can be thought of as an anchor which attaches surface proteins to the cell membrane
complement-regulating surface proteins, e.g. decay-accelerating factor (DAF), are not properly bound to the cell membrane due a lack of GPI
thrombosis is thought to be caused by a lack of CD59 on platelet membranes predisposing to platelet aggregation

332
Q

clinical fx HPN

A

haemolytic anaemia
red blood cells, white blood cells, platelets or stem cells may be affected therefore pancytopaenia may be present
haemoglobinuria: classically dark-coloured urine in the morning (although has been shown to occur throughout the day)
thrombosis e.g. Budd-Chiari syndrome
aplastic anaemia may develop in some patients

333
Q

diagnosis non hodgkins

A

flow cytometry of blood to detect low levels of CD59 and CD55 has now replaced Ham’s test as the gold standard investigation in PNH

334
Q

mx HPN

A

blood product replacement
anticoagulation
eculizumab, a monoclonal antibody directed against terminal protein C5, is currently being trialled and is showing promise in reducing intravascular haemolysis
stem cell transplantation

335
Q

offer platelet transfusion at what count

A

<30x10^9 with clinically significant bleeding
if severe bleeding or bleeding at critical sites such as CNS - <100 x 10^9 before transfusing

336
Q

risk of platelet transfusion

A

bacterila contamination

337
Q

platelet transfusion for thrombocytopenia before surgery aim

A

> 50×109/L for most patients
50-75×109/L if high risk of bleeding
100×109/L if surgery at critical site

338
Q

if no active bleeding platelet threshold

A

10x10^9

339
Q

not perform platelet transfusion for following

A

Chronic bone marrow failure
Autoimmune thrombocytopenia
Heparin-induced thrombocytopenia, or
Thrombotic thrombocytopenic purpura.

340
Q

relative causes of polycythaemia

A

dehydration
stress: Gaisbock syndrome

341
Q

primary cause of polycythaemia

A

polycythaemia rubra vera

342
Q

secondary causes of polycythaemia

A

COPD
altitude
obstructive sleep apnoea
excessive erythropoietin: cerebellar haemangioma, hypernephroma, hepatoma, uterine fibroids*

343
Q

polycythaemia vera /rubra vera definition

A

a myeloproliferative disorder caused by clonal proliferation of a marrow stem cell leading to an increase in red cell volume, often accompanied by overproduction of neutrophils and platelets

344
Q

polycythaemia vera genetics

A

a mutation in JAK2 is present in approximately 95% of patients with polycythaemia vera

345
Q

clinical fx polycythaemia vera

A

pruritus, typically after a hot bath
splenomegaly
hypertension
hyperviscosity
arterial thrombosis
venous thrombosis
haemorrhage (secondary to abnormal platelet function)
low ESR

346
Q

initial tests polycythaemia vera

A

full blood count/film (raised haematocrit; neutrophils, basophils, platelets raised in half of patients)
JAK2 mutation
serum ferritin
renal and liver function tests

347
Q

if JAK2 mutation -ve other investigations polycythamia evra

A

red cell mass
arterial oxygen saturation
abdominal ultrasound
serum erythropoietin level
bone marrow aspirate and trephine
cytogenetic analysis
erythroid burst-forming unit (BFU-E) culture

348
Q

JAK2 positive polycythameia vera diagnosis

A

both criteria -
A1 High haematocrit (>0.52 in men, >0.48 in women) OR raised red cell mass (>25% above predicted)
A2 Mutation in JAK2

349
Q

JAK2 negative polycythaemia vera diagnosis

A

A1 + S2 _ A3 + either A or B criteria
A1 Raised red cell mass (>25% above predicted) OR haematocrit >0.60 in men, >0.56 in women
A2 Absence of mutation in JAK2
A3 No cause of secondary erythrocytosis
A4 Palpable splenomegaly
A5 Presence of an acquired genetic abnormality (excluding BCR-ABL) in the haematopoietic cells
B1 Thrombocytosis (platelet count >450 * 109/l)
B2 Neutrophil leucocytosis (neutrophil count > 10 * 109/l in non-smokers; > 12.5*109/l in smokers)
B3 Radiological evidence of splenomegaly
B4 Endogenous erythroid colonies or low serum erythropoietin

350
Q

polycythaemia vera mx

A

aspirin
reduces the risk of thrombotic events
venesection
first-line treatment to keep the haemoglobin in the normal range
chemotherapy
hydroxyurea - slight increased risk of secondary leukaemia
phosphorus-32 therapy

351
Q

polycythaemia vera prognosis

A

thrombotic events are a significant cause of morbidity and mortality
5-15% of patients progress to myelofibrosis
5-15% of patients progress to acute leukaemia (risk increased with chemotherapy treatment)

352
Q

post thrombotic syndrome definition

A

complications following a DVT. Venous outflow obstruction and venous insufficiency result in chronic venous hypertension. The resulting clinical syndrome is known as post-thrombotic syndrome.

353
Q

clinical fx post thrombotic syndrome

A

painful, heavy calves
pruritus
swelling
varicose veins
venous ulceration

354
Q

reduce risk of post thrombotic syndrome

A

compressions stocking on pts with DVT

355
Q

mx of post thrombotic syndromes

A

compression stockings
leg elevation

356
Q

when pregnancy dvt/pe

A

last trimester

357
Q

pathophysiology pregnancy dvt development

A

increase in factors VII VIII, X, and fibrinogen
decrease in protein S
uterus also presses on IVC…venous stasis in legs

358
Q

pregnancy dvt/pe mx

A

S/C LMWH
(preferred over IV as less bleeding and thrombocytopenia)

359
Q

chronic granulomatous defect underlying defect

A

lack of NADPH oxidase…reduced ability of phagocytes to produce reactive oxygen species

360
Q

which organisms more likely affect chronic granulomatous disease

A

staph aureus and aspergillus
…causes recurrent pneumonias and abscesses

361
Q

chediak-higashi syndrome underlying defect

A

microtubule polymerisation defect…decrease in phagocytosis

362
Q

chediak-higashi syndrome clinical fx

A

peripheral neuropathy
partial albinism
recurrent bacterial infections

363
Q

chronic granulomatous disease inv

A

abnormal dihydrorhodamine flow cytometry test

364
Q

chediak-higashi syndrome blood film

A

giant granules in neutrophils and platelets

365
Q

leukocyte adhesion deficiency underlying pathology

A

defect of CD18 protein on neutrophils

366
Q

leukocyte adhesion deficiency clinical fx

A

recurrent bacterial infections
delay in umbilical cord sloughing
absence of pus at sites of infection

367
Q

neutrophil disorders

A

chronic granulomatous disorder
chediak higashi
leukocyte adhesion def

368
Q

B cell disorders

A

common variable immunodeficiency
bruton’s
selective IgA def

369
Q

common variable immunodeficiency immunoglobulin levels

A

low antibodies - IgG, IgM, IgG

370
Q

common variable immunodefiency increased risk

A

recurrent chest infections
autoimmune disorders
lymphoma

371
Q

bruton’s underlying patho

A

defect in BTK gene…severe block in B cell development

372
Q

bruton’s inheritance

A

x linked recessive

373
Q

bruton’s clinical fx

A

recurrent bacterial inf

374
Q

bruton’s tests

A

absence of B cells
reduced immunoglobulins of all classes

375
Q

selective IgA def underlying patho

A

maturation defect in B cells

376
Q

selective IgA def how common

A

most common primary antibody def

377
Q

selective IgA def associated

A

coeliac disease and may cause false negative coeliac antibody screen

378
Q

selective IgA def and blood transfusions

A

Severe reactions to blood transfusions may occur (anti-IgA antibodies → analphylaxis)

379
Q

T cell disorder

A

DiGeorge Syndrome

380
Q

di george syndrome underlying patho

A

chromosome deletion…failure to develop 3rd and 4th pharngeal pouches

381
Q

clinical fx di george syndrome

A

congenital heart disease (e.g. tetralogy of Fallot), learning difficulties, hypocalcaemia, recurrent viral/fungal diseases, cleft palate

382
Q

combined b and t cell defect examples

A

Severe combined immunodef
ataxic telangiectasia
wiskott-aldrich syndrome
hyper-IgM syndromes

383
Q

SCID underlying defect

A

many
defect in common gamma chain…used to form receptors for IL2 nad other interleukins

384
Q

SCID clinical fx

A

recurrent infections

385
Q

SCID possible tx

A

stem cell transplant

386
Q

ataxic telangiectasia defect

A

defect in DNA repair enzymes

387
Q

ataxic telangiectasia inheritence

A

aut rec

388
Q

clinical fx ataxic telangiectasia

A

cerebellar ataxia, telangiectasia (spider angiomas), recurrent chest infection

389
Q

increased risk of.. in ataxic telangiectasia

A

lymphoma or leukaemia

390
Q

wiskott-aldrich syndrome defect

A

defect in WASP gene

391
Q

wiskott-aldrich syndrome inheritance

A

x linked rec

392
Q

clinical fx wiskott-aldrich

A

recurrent bacterial infections, eczema, thrombocytopaenia.

393
Q

wiskott aldrich antibody levels

A

low IgM

394
Q

increased risk of..wiskott-aldrich syndrome

A

autoimmune and malignancy

395
Q

hyper IgM syndromes defect

A

mutations in CD40 gene

396
Q

hyper IgM syndromes clinical fx

A

Infection/Pneumocystis pneumonia, hepatitis, diarrhoea

397
Q

sickle cell inheritance

A

aut recessive

398
Q

sickle cell pathophysiology

A

synthesis of abnormal Hb chain = HbS
if homozygous = HbSS
if hetero = HbAS
if inherited one HbS+abnormal Hb = HbSC

glutmate substituted for valine in each of the two beta chains…decreased water solubility of deoxy-Hb.
In dexoygenated state - HbS polymerases and causes red cells to sickle (sickle at a higher oxygen level)
these cells are fragile and haemolyse..block vessels and cause infarction

399
Q

sickle cell epidemiology

A

african descent = heterozygous…protection from malaria

400
Q

when in life develop sickle cell sx

A

when homozygous…4-6mths in when abnormal HbSS takes over from fetal Hb

401
Q

definitive diagnosis sickle cell

A

haemoglobin electrophoresis

402
Q

sickle cell crisis mx

A

analgesia e.g. opiates
rehydrate
oxygen
consider antibiotics if evidence of infection
blood transfusion
exchange transfusion: e.g. if neurological complications

403
Q

long term mx of sickle cell

A

hydroxyurea - increases HbF levels…prophylactically prevent painful episodes
pneumococcal vaccine

404
Q

type of crisis in sickle cell

A

thrombotic, ‘vaso-occlusive’, ‘painful crises’
acute chest syndrome
anaemic
aplastic
sequestration
infection

405
Q

thrombotic crises precipitated

A

infection, dehydration, deoxygenation (e.g. high altitude)

406
Q

examples clinical presentations thrombotic crisis

A

avascular necrosis of hip
hand foot sundrome
infarcts in lung, spleen, brain

407
Q

acute chest syndrome patho

A

vaso occlusion within pulmonary microvasculature…infarction

408
Q

clinical fx acute chest syndrome

A

dyspnoea, chest pain, low o2
inv - CXR - pulmonary infiltrates

409
Q

acute chest syndrome mx

A

pain relief
respiratory support e.g. oxygen therapy
antibiotics: infection may precipitate acute chest syndrome and the clinical findings (respiratory symptoms with pulmonary infiltrates) can be difficult to distinguish from pneumonia
transfusion: improves oxygenation

410
Q

aplastic crisis caused by

A

infection with parvovirus

411
Q

aplastic crises investigations

A

sudden drop in Hb
reduced reticulocyte count

412
Q

sequestration crises patho

A

sickling within organs such as lungs or spleen…pooling of blood and worsening of anaemia

413
Q

sequestration crises blood results

A

increased reticulocyte

414
Q

sickle cell crisis general mx

A

analgesia e.g. opiates
rehydrate
oxygen
consider antibiotics if evidence of infection
blood transfusion
indications include: severe or symptomatic anaemia, pregnancy, pre-operative
do not rapidly reduce the percentage of Hb S containing cells
exchange transfusion
indications include: acute vaso-occlusive crisis (stroke, acute chest syndrome, multiorgan failure, splenic sequestration crisis
rapidly reduce the percentage of Hb S containing cells

415
Q

sideroblastic anaemia patho

A

red cells fail to form haem…leads to deposits of iron in mitochondria = forms a ring sideroblast around nucleus

416
Q

causes of sideroblastic anaemia

A

congenital causes - delta-aminolevulinate synthase-2 deficiency
acquired - myelodysplasia, alcohol, lead, anti-TB meds

417
Q

sideroblastic anaemia inv

A

hypochromic microcytic anaemia
iron studies - high ferritin, high iron, high transferrin sat
basophilic strippling of red cell
bone marrow staining - sideroblasts

418
Q

sideroblastic anaemia mx

A

supportive
treat any underlying cause
pyridoxine may help

419
Q

massive splenomegaly possible causes

A

myelofibrosis
chronic myeloid leukaemia
visceral leishmaniasis (kala-azar)
malaria
Gaucher’s syndrome

420
Q

other causes of splenomegaly

A

portal hypertension e.g. secondary to cirrhosis
lymphoproliferative disease e.g. CLL, Hodgkin’s
haemolytic anaemia
infection: hepatitis, glandular fever
infective endocarditis, thalassaemia
rheumatoid arthritis (Felty’s syndrome)

421
Q

sickle cell large or small spleen?

A

majority of adults will have atrophied spleen due to repeated infarctions

422
Q

causes of severe thrombocytopenia

A

ITP
DIC
TTP
haem malignancy

423
Q

causes of moderate thrombocytopenia

A

heparin induced thrombocytopenia (HIT)
drug-induced (e.g. quinine, diuretics, sulphonamides, aspirin, thiazides)
alcohol
liver disease
hypersplenism
viral infection (EBV, HIV, hepatitis)
pregnancy
SLE/antiphospholipid syndrome
vitamin B12 deficiency

424
Q

pseudothrombocytopenia association

A

use of EDTA as an anticoagulant

425
Q

platelet count thrombocytosis

A

high
>400 x 10^9/l

426
Q

possible causes of thrombocytosis

A

reactive - platelets act as an acute phase reactant…respond to stress/infection/iron def anaemia
malignancy
essential thrombocytosis
hyposplenism

427
Q

essential thrombocytosis definition

A

one of the myeloproliferative disorders which overlaps with chronic myeloid leukaemia, polycythaemia rubra vera and myelofibrosis

428
Q

essential thrombocytosis patho

A

megakaryocyte prolferation..overproduction of platelets

429
Q

fx of essential thrombocytosis to diagnose

A

platelet >600
both thrombosis and haemorrhage
burning sensation in hands
JAK2 mutation in 50% of pts

430
Q

essential thrombocytosis mx

A

hydroxyurea - reduce platelet count
low dose aspirin -

431
Q

causes of inherited thrombophilias

A

factor V Leiden (activated protein C resistance)
prothrombin gene mutation

deficiencies -
antithrombin III deficiency
protein C or S deficiency

432
Q

most common cause of thrombophilia

A

factor V Leiden (activated protein C resistance)

433
Q

acquired cause of thrombophilia

A

antiphospholipid syndrome
COCP

434
Q

patho of thrombotic thrombocytopenic purpura

A

defiency of enzymes which breaksdown von willebrand factor. so these factors cause platelets to clump within vessels

435
Q

TTP overlaps with

A

HUS

436
Q

clinical fx TTP

A

rare, typically adult females
fever
fluctuating neuro signs (microemboli)
microangiopathic haemolytic anaemia
thrombocytopenia
renal failure

437
Q

causes of TTP

A

post-infection e.g. urinary, gastrointestinal
pregnancy
drugs: ciclosporin, oral contraceptive pill, penicillin, clopidogrel, aciclovir
tumours
SLE
HIV

438
Q

thymoma
- how common
- tumour of

A

most common tumour of anterior mediastinum

439
Q

thymoma when detected

A

60-70 years old

440
Q

thymoma associations

A

myasthenia gravis (30-40% of patients with thymoma)
red cell aplasia
dermatomyositis
also : SLE, SIADH

441
Q

causes of death from thymoma

A

airway compression
cardiac tamponade

442
Q

MoA of tranexamic acid

A

reversibly binds to lysine receptor sites on plasminogen or plasmin…prevents plasmin from degrading fibrin

443
Q

indications tranxemaic acid

A

menorrhagia
bleeding trauma if given in 1st 3 hours (IV bolus following by infusion)

444
Q

when tumour lysis syndrome tx what

A

high grade lymphomas or leukaemias
usually triggered by combo chemo
or with steroid tx alone

445
Q

TLS patho

A

he breakdown of the tumour cells and the subsequent release of chemicals from the cell

446
Q

TLS electrolytes

A

2 high one low
high potassium and phosphate, low calcium

447
Q

when suspect TLS

A

AKI
high phosphate
high uric acid

448
Q

prevention TLS

A

IV fluids
allopurinol or rasburicase

449
Q

which prophylactic tx for high risk pts of TLS and how work

A

rasburicase - a recombinant version of urate oxidase, an enzyme that metabolizes uric acid to allantoin. Allantoin is much more water-soluble than uric acid and is, therefore, more easily excreted by the kidneys

450
Q

TLS grading

A

Cairo-bishop scoring system

451
Q

clinical tumour lysis syndrome diagnosis

A

laboratory tumour lysis syndrome plus one or more of the following:
increased serum creatinine (1.5 times upper limit of normal)
cardiac arrhythmia or sudden death
seizure

452
Q

general risks of VTE

A

increased risk with advancing age
obesity
family history of VTE
pregnancy (especially puerperium)
immobility
hospitalisation
anaesthesia
central venous catheter: femoral&raquo_space; subclavian

453
Q

underlying conditions causing increased risk of VTE

A

malignancy
thrombophilia: e.g. Activated protein C resistance, protein C and S deficiency
heart failure
antiphospholipid syndrome
Behcet’s
polycythaemia
nephrotic syndrome
sickle cell disease
paroxysmal nocturnal haemoglobinuria
hyperviscosity syndrome
homocystinuria

454
Q

medications causing higher risk of VTE

A

COCP
HRT (more if combined)
raloxifene and tamoxifen
antipsychotics esp olanzapine

455
Q

how vit b12 absorbed

A

It is absorbed after binding to intrinsic factor (secreted from parietal cells in the stomach) and is actively absorbed in the terminal ileum. A small amount of vitamin B12 is passively absorbed without being bound to intrinsic factor.

456
Q

causes of vit b12 def

A

pernicious anaemia: most common cause
post gastrectomy
vegan diet or a poor diet
disorders/surgery of terminal ileum (site of absorption)
Crohn’s: either diease activity or following ileocaecal resection
metformin (rare)

457
Q

clinical fx of vit b12 def

A

macrocytic anaemia
sore tongue and mouth
neurological symptoms
the dorsal column is usually affected first (joint position, vibration) prior to distal paraesthesia
neuropsychiatric symptoms: e.g. mood disturbances

458
Q

vit b12 def mx

A

if no neuro - IM hydroxocobalamin 3x/wk for 2 wks then 1/3mths
if def in folic acid - treat b12 first

459
Q

most common inheritable bleeding disorder

A

von willebrand disease

460
Q

von willebrand’s inheritance

A

aut dom USUALLY

461
Q

clinical fx von willebrand

A

epistaxis
menorrhagia
haemoarthroses
muscle haematomas

462
Q

von willebrand’s patho

A

promotes platelet adhesion to damaged endothelium
also carrier molecule for factor VIII

463
Q

what are 3 types of von willebrands

A

type 1: partial reduction in vWF (80% of patients)
type 2*: abnormal form of vWF
type 3**: total lack of vWF (autosomal recessive)

464
Q

von willebrands inv

A

prolonged bleeding time
APTT may be prolonged
factor VIII levels may be moderately reduced
defective platelet aggregation with ristocetin

465
Q

von willebrands mx

A

mild bleeding - tranxemic acid
desmopressin - raises level of vWF
factor VIII concentrate

466
Q

waldenstrom’s macroglobulinaemia age and sex

A

older men

467
Q

patho waldenstrom’s macroglobulinaemia

A

It is a lymphoplasmacytoid malignancy characterised by the secretion of a monoclonal IgM paraprotein

468
Q

clinical fx waldenstrom’s macroglobulinaemia

A

systemic upset: weight loss, lethargy
hyperviscosity syndrome e.g. visual disturbance
the pentameric configuration of IgM increases serum viscosity
hepatosplenomegaly
lymphadenopathy
cryoglobulinaemia e.g. Raynaud’s

469
Q

waldenstroms macroglobulinaemia inv

A

monoclonal IgM paraproteinaemia
bone marrow biopsy is diagnostic
infiltration of the bone marrow with lymphoplasmacytoid lymphoma cells

470
Q

waldenstrom’s macroglobulinaemia mx

A

rituximab-based combo chemo

471
Q
A

Cancer Care (2 decks)

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