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Haematology Flashcards

1
Q

What 2 groups can macrocytic anaemia be split into and what are examples of each?

A
  1. megaloblastic: B12 deficiency (pernicious), folate deficiency, drugs (sulfonamides), gastrectomy, terminal ileum resection
  2. normoblastic: liver disease, alcoholism, hypothyroidism, myelodysplasia, anticonvulsants
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2
Q

What are 5 blood test abnormalities that may be seen in anti-phospholipid syndrome?

A
  1. Anti-cardiolipin antibodies
  2. Lupus anticoagulant
  3. Anti beta2 glycoprotein 1 antibody
  4. Prolonged APTT (paradoxical)
  5. Low platelets
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3
Q

What are the criteria for diagnosing hereditary haemorrhagic telangiectasia?

A

2 criteria - possible diagnosis
3 or more - definite diagnosis
the criteria:
1. epistaxis
2. telangiectases multiple at characteristic sites (lips, oral cavity, fingers, nose)
3. visceral lesions e.g. GI telangiectasia (+/- bleeding), pulmonary AVMs, hepatic AVM, cerebral AVM, spinal AVM
4. family history first-degree relative with HHT

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4
Q

What is the key histological finding in Hodgkin’s lymphoma?

A

Reed-Sternberg cells (cells of B cell origin, unable to express antibodies)

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5
Q

What are the 5 types of Hodkin’s lymphoma and which has a different treatment paradigm?

A
  1. nodular sclerosis
  2. mixed cellularity
  3. lymphocyte-depleted
  4. lymphocyte-rich
  5. nodular lymphocyte predominant Hodgkin disease (different treatment, unique entity)
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6
Q

What is Pel-Ebstein fever in Hodgkin’s lymphoma?

A

high fever for 1-2 weeks followed by afebrile period of 1-2 weeks

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7
Q

What is alcohol induced pain with lymphadenopathy specific for?

A

Hodgkin’s disease

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8
Q

What system is sued to stage Hodgkin’s disease?

A

Ann Arbor system

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9
Q

What is the treatment for Hodgkin’s lymphoma?

A

radiotherapy and/or chemotherapy

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10
Q

What is the commonest inherited theombophilia?

A

Factor V Leiden

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11
Q

What are 5 examples of inherited thrombiphilias?

A
  1. Factor V Leiden
  2. Prothrombin gene mutation
  3. Anthrombin III deficiency
  4. Protein S deficiency
  5. Protein C deficiency
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12
Q

What is an example of an acquired thrombophilia?

A

Antiphospholipid syndrome

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13
Q

What are 4 features of polycythaemia?

A
  1. Pruritus - particularly after warm bath
  2. Ruddy complexion
  3. Gout
  4. Peptic ulcer disease
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14
Q

What is the commonest secondary cause of antiphospholipid syndrome?

A

SLE

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15
Q

Why does antiphospholipid syndrome cause a paradoxical rise in APTT?

A

ex-vivo reaction of lupus anticoagulant antibodies with phospholipids involved in the coagulation cascade

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16
Q

What are 4 diseases associated with anti-phospholipid syndrome?

A
  1. SLE
  2. other autoimmune disorders
  3. lymphoproliferative disorders
  4. phenothiazindes (rare)
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17
Q

What is a key dermatological manifestation of antiphospholipid syndrome?

A

livedo reticularis

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18
Q

What is the primary thromboprophylaxis management for antiphospholipid syndrome?

A

low-dose aspirin

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19
Q

What is the secondary thromboprophylaxis given in antiphospholipid syndrome?

A
  • initial VTE event: lifelong warfarin, target INR 2-3
  • recurrent VTE: lifelong warfarin; if whilst taking warfarin add low dose aspirin, target 3-4
  • arterial thrombosis: lifelong warfarin target INR 2-3
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20
Q

What is immune (idiopathic) thrombocytopenic purpura?

A

immune-mediated reduction in platelet count; antibodies are directed against glycoprotein IIb/IIIa or Ib-V-IX complex

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21
Q

What type of hypersensitivity reaction is immune thrombotuopenic purpura?

A

type II

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22
Q

What are the key features of immune thrombocytopenia in children?

A
  • bruising, petechial or purpuric rash, bleeding less common (if occurs - epistaxis or gingival bleeding)
  • more acute than in adult
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23
Q

What will investigations show in immune thrombocytopenic purpura in children in children?

A

FBC - isolated thrombocytopenia
bone marrow examination - only required if atypical features e.g. LN enlargement/splenomegaly, high/low WCC, failure to resolve/respond to treatment

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24
Q

What is the management of immune thrombocytopenic purpura in chidlren?

A
  • resolves spontaneously in 80% of children within 6 months with or without treatment
  • avoid activties that may result in trauma e.g. team sports
  • if platelet count <10 or significant bleeding, options: oral/IV corticosteroid, IVIG, platelet transfusion in emergency
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25
Q

What should be administered during cardiac arrest if pulmonary embolism is suspected & how does this cahnge the CPR algorithm?

A

thrombolytic drugs e.g. alteplase - if given, CPR should be continued for extended period 60-90 min

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26
Q

What is the classical genetic abnormality in chronic myeloid leukaemia?

A
  • Philadelphia chromosome
  • = translocation between long arm of chromosome 9 and 22
  • results in ABL proto-oncogene being fused with BCR gene - BCR-ABL gene codes for fusion protein with increased tyrosine kinase activity
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27
Q

At what age does chronic myeloid leukaemia present?

A

60-70 years

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28
Q

What are the key presenting clinical features of chronic myeloid leukaemia?

A
  • anaemia: lethargy
  • weight loss and sweating
  • splenomegaly → abdo discomfort
  • 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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29
Q

What is the first-line management of chronic myeloid leukaemia?

A

imatinib - inhibits tyrosine kinase associated with BCR-ABL defect

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30
Q

What are 4 options for treatment of chronic myeloid leukaemia?

A
  • imatinib
  • hydroxyurea
  • interferon-alpha
  • allogenic bone marrow transplant
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31
Q

What are 2 types of acute leukaemia and when do they classically present?

A
  • Acute myeloid leukaemia - adulthood
  • Acute lymphoblastic leukaemia - childhood (2-5 years)
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32
Q

What is the most common type of childhood malignancy?

A

acute lymphoblastic leukaemia (ALL)

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33
Q

What are the clinical features of acute lymphoblastic leukaemia?

A
  • anaemia - lethargy and pallor
  • neutrophenia - infections
  • thrombocytopenia - bruising, petechiae
  • bone pain
  • splenomegaly
  • hepatosplenomegaly
  • fever
  • testicular swelling
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34
Q

What are 3 types of acute lymphoblastic leukaemia?

A
  1. common ALL (75%) - CD10 present, pre-B phenotype
  2. T-cell ALL
  3. B-cell ALL
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35
Q

What are 5 poor prognostic factors for ALL?

A
  1. age < 2 years or >10 years
  2. WBC <20 at diagnosis
  3. T or B cell surface markers
  4. non-Caucasian
  5. male sex
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36
Q

Which patient group does haemolytic uraemic syndrome usually present in?

A

young children

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37
Q

What is the triad of symptoms seen in haemolytic uraemic syndrome?

A
  1. acute kidney injury
  2. microangiopathic haemolytic anaemia
  3. thrombocytopenia
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38
Q

What is the class cause of primary haemolytic uraemic syndrome?

A
  • Shiga toxin-producing E coli (STEC) 0157:H7
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39
Q

In addition to STEC, what are 3 other causes of secondary haemolytic uraemic syndrome?

A
  1. pneumococcal infection
  2. HIV
  3. SLE, drugs, cancer
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40
Q

What causes primary (atypical) HUS?

A

complement dysregulation

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41
Q

What are 3 investigations to perform in haemolytic uraemic syndrome?

A
  1. FBC - anaemia (microangiopathic haemolytic anaemia), thrombocytopenia, fragmented blood film
  2. U+E - AKI
  3. stool culture - PCR for Shiga toxins, looking for STEC
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42
Q

What will blood film show in haemolytic uraemic syndrome?

A

fragmented: schistocytes and helmet cells

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43
Q

What will Coombs test show in haemolytic uraemic syndrome?

A

negative

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44
Q

What is the management of haemolytic uraemic syndrome?

A
  • supportive - fluids, blood transfusion, dialysis if required
  • plasma exchanged in severe cases NOT associated with diarrhoea
  • eculizumab in adult atypical HUS
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45
Q

What are 4 types of crises in sickle cell anaemia?

A
  1. thrombotic a.k.a. ‘vaso-occlusive’ ‘painful crises’
  2. acute chest syndrome
  3. anaemic aplastic or sequestration
  4. infection
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46
Q

What are 3 things that can precipitate a vaso-occlusive / thrombotic sickle cell crisis?

A
  1. infection
  2. dehydration
  3. deoxygenation e.g. high altitude
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47
Q

What are 5 sites where infarction can occur in a sickle cell vaso-occlusive (thrombotic) crisis?

A
  1. avascular necrosis of hip
  2. hand-foot syndrome (children)
  3. lungs
  4. spleen
  5. brain
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48
Q

What is acute chest syndrome in sickle cell disease?

A

vaso-occlusion within the pulmonary microvasculature leading to infarction in the lung parenchuma

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49
Q

What is the management of acute chest syndrome in sickle cell disease?

A
  • pain relief
  • respiratory support e.g. oxygen therapy
  • antibiotics
  • transfusion - improves oxygenation
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50
Q

What causes aplastic crises in sickle cell disease?

A

infection with parvovirus

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51
Q

How will aplastic crises in sickle cell disease manifest on blood tests?

A
  • sudden fall in Hb
  • reduced reticulocyte count (bone marrow suppression)
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52
Q

What is a sequestration crisis in sickle cell disease?

A
  • sickling within organs such as spleen or lungs causing pooling of blood with worsening of the anaemia
  • may present with splenomegaly, abdominal pain, clinical anaemia
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53
Q

How will a sequestration crisis in sickle cell disease manifest on blood tests?

A

worsening of anaemia, INCREASED reticulocyte count

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54
Q

How can aplastic and sequestration crises be differentiated in sickle cell disease, on the basis of blood tests?

A

reticulocyte count increased in sequestration, reduced in aplastic

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55
Q

What are 6 PRC transfusion reactions?

A
  1. Non-haemolytic febrile reaction
  2. Minor allergic reaction
  3. Anaphylaxis
  4. Acute haemolytic reaction
  5. Transfusion-associated circulatory overload (TACO)
  6. Transfusion-related acute lung injury (TRALI)
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56
Q

What are the features of non-haemolytic febrile reaction in response to blood transfusion?

A

fever, chills

10-30% caused by platelet transfusion

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57
Q

What is the management of a non-haemolytic febrile reaction?

A

slow or stop transfusion, paracetamol, monitor

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58
Q

What is thought to cause non-haemolytic febrile transfusion reaction?

A

HLA antibodies reacting with white cell fragments in the blood product and cytokines that have leaked from the blood cell durting storage - often due to sensitisation by previous pregnancies / transfusions

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59
Q

What is thought to cause minor allergic reactions to blood transfusion?

A

foreign plasma proteins

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60
Q

What are the features of a minor allergic reaction to blood transfusion?

A

pruritis, urticaria

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61
Q

What is the management of minor allergic reaction to blood transfusion?

A
  • temporarily stop transfusion
  • antihistamine
  • monitor
  • once symptoms resolve, transfusion may be continued
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62
Q

What can cause anaphylaxis in response to blood transfusion?

A

patients with IgA deficiency who have anti-IgA antibodies

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63
Q

What is the management of anaphylaxis in response to blood transfusion?

A

permanently discontinue - administer IM adrenaline - supportive care; antihistamine, steroids and bronchodilators, IVF

64
Q

What causes an acute haemolytic transfusion reaction?

A

mismatch of blood group (ABO) causng massive intravascular haemolysis - usually result of red blood cell destruction by IgM type antibodies

65
Q

How quickly do acute haemolytic transfusion reaction symptoms develop and what are they?

A
  • within minutes
  • fever, abdominal / chest pain, agitation, hypotension
66
Q

What is the management of acute haemolytic transfusion reaction?

A
  • stop transfusion
  • confirm diagnosis - check patient ID / blood product
  • send blood for direct Coomns test, repeat typing and cross-matching
  • IVF resuscitation
  • inform lab
67
Q

What are 2 possible complications of acute haemolytic transfusion reaction?

A
  • intravascular coagulation
  • renal failure
68
Q

What are 2 things that can pre-dispose to transfusion-associated circulatory overload?

A
  1. excessive rate of transfusion
  2. pre-exisitng heart failure
69
Q

What is a key difference in TACO that is not seen in TRALI?

A

patients may be hypertensive in TACO

70
Q

What is the management of TACO?

A

slow or stop transfusion
consider IV loop diuretic and O2

71
Q

What is transfusion-related acute lung injury (TRALI)?

A
  • non-cardiogenic pulmonary oedema
  • thought secondary to increased vascular permeabiliy caused by host neutrophils
  • activated by substances in donated blood
  • develop hgypoxaemia / acute respiratory distress syndrome
72
Q

How quickly does TRALI develop?

A

within 6 hours of transfusion

73
Q

What are 4 key features of TRALI?

A
  1. hypoxia
  2. pulmonary infiltrates on CXR
  3. fever
  4. hypotension
74
Q

What is the management of TRALI?

A

stop transfusion, oxygen, supportive care

75
Q

What are 4 ways that the risk of vCJD from blood transfusion has been reduced?

A
  1. leucodepletion to reduce vCJD infectivity
  2. plasma derivated have been fractionated from imported plasma (not UK donors)
  3. FFP for children / certain adult groups imported
  4. recipients of blood transfusion excluded from donating blood
76
Q

What are 4 secondary causes of polycythaemia which are due to excessive erythropoietin?

A
  1. cerebellar haemangioma
  2. hypernephroma
  3. hepatoma
  4. uterine fibroids
77
Q

How can you differentiate between true (primary or secondary) polycythaemia and relative polycythaemia (dehydration / stress)?

A

red cell mass studies are sometimes used. In true polycythaemia the total red cell mass in males > 35 ml/kg and in women > 32 ml/kg (not in relative PC)

78
Q

What are 2 relative causes of polycythaemia?

A
  1. dehydration
  2. stress (Gaisbock syndrome)
79
Q

What is primary polycythaemia called?

A

polycythaemia rubra vera

80
Q

What are 4 secondary causes of polycythaemia?

A
  1. COPD
  2. altitude
  3. OSA
  4. excessive EPO
81
Q

What is a cause of episodic RUQ pain in a patient with hereditary spherocytosis?

A

biliary colic - chronic haemolysis -> gallstone formation (differential - splenic rupture)

82
Q

What is the inheritance pattern of hereditary spherocytosis?

A

autosomal dominant

83
Q

What are 7 aspects of the presentation of hereditary spherocytosis?

A
  1. failure to thrive
  2. jaundice
  3. gallstones
  4. splenomegaly
  5. aplastic crisis precipitated by parvovirus infection
  6. degree of haemolysis variable
  7. MCHC elevated
84
Q

When are no additional tests required for a diagnosis of hereditary spherocytosis?

A
  • patients with family history of HS
  • typical clinical features
  • typical laboratory investigations: spherocytes, raised mean corpuscular haemoglobin concentration (MCHC), high reticuloctes
85
Q

If diagnosis is equivocal based on initial presentation and lab tests, what is the diagnostic test for hereditary spherocytosis?

A

EMA binding test and cryohaemolysis test

86
Q

What is the method of choice for diagnosing hereditary spherocytosis with atypical presentations?

A

electrophoresis analysis of erythrocyte membranes

87
Q

What is the management of an acute haemolytic crisis in hereditary spherocytosis?

A

supportive, transfuse if necessary

88
Q

What are 2 aspects of the long term management of hereditary spherocytosis?

A
  1. folate replacement
  2. splenectomy
89
Q

Which region of the world is hereditary spherocytosis more common?

A

North European

90
Q

What are 4 types of Hodgkin’s lymphoma and their relative frequencies?

A
  1. Nodular sclerosing - 70% (commonest)
  2. Mixed cellularity (20%)
  3. Lymphocyte predominant (5%)
  4. Lymphocyte depleted (rare)
91
Q

What is the order of prognosis of the 4 types of Hodgkin’s lymphoma from best to worst?

A
  1. Best: Lymphocyte predominant
  2. Good: nodular sclerosing
  3. Good: mixed cellularity
  4. Worst: lymphocyte depleted
92
Q

Which type of Hodgkin’s lymphoma is associated with lacunar cells?

A

nodular sclerosing

93
Q

Which type of Hodgkin’s lymphoma is associated with a large number of Reed-Sternberg cells?

A

mixed cellularity

94
Q

What symptoms imply a poor prognosis in Hodgkin’s lymphoma?

A

B symptoms - weight loss >10% in last 6 months, fever >38, night sweats

95
Q

What are 7 factors associated with a poor prongosis in Hodgkin’s lymphoma other than b symptoms?

A
  1. age > 45 years
  2. stage IV disease
  3. Hb <10.5
  4. lymphocytes < 600 or 8%
  5. male
  6. albumin < 40
  7. WCC < 15 000
96
Q

Of blood transfusion and exchange transfusion in sickle cell crises, which can rapidly reduce the percentage of Hb S containing cells?

A

exchange transfusion (not blood)

97
Q

When should you admit patients with features of a sickle cell crisis?

A
  • all children with fever - particularly if unexplained
  • low threshold for all children
  • temperature over 38
  • any chest symptoms
98
Q

What are 4 blood test findings in iron deficiency anaemia?

A
  1. low ferritin (can be raised during inflammatory states)
  2. increased total iron binding capacity (TIBC)
  3. low serum iron
  4. reduced transferrin saturation (but increased transferrin)
99
Q

What type of anaemia is IDA?

A

hypochromic microcytic anaemia

100
Q

For how long should patients continue taking iron after iron deficiency has been corrected?

A

3 months

101
Q

How can you differentiate between CLL and CML?

A
  • chronic lymphocytic leukaemia - lymphocytosis with mature lymphocytes and smudge cells on peripheral smear
  • chronic myeloid leukaemia - increased numbers of granulocytes at all stages of development, splenomegaly + high platelets
102
Q

What is the inheritance pattern of beta-thalassaemia?

A

autosomal recessive

103
Q

What are 2 features on blood tests of beta-thalassaemia trait?

A
  • mild hypochromic, microcytic anaemia - microcytosis disproportionate to anaemia
  • HbA2 raised (>3.5%)
104
Q

What is the mechanism of action of dabigatran?

A

direct thrombin inhibitor

105
Q

What is the reversal agent for dabigatran?

A

idarucizumab

106
Q

Which drugs can andexanet alfa be used as the reversal agent?

A

rivaroxaban + apixaban

107
Q

What is the mechanism of action of rivaroxaban / apixaban / edoxaban?

A

direct factor Xa inhibitor

108
Q

What is the excretion of different DOACs?

A
  • dabigatran: renal
  • rivaroxaban: liver
  • apixaban: faecal
  • edoxaban: faecal
109
Q

At what partial pressure of oxygen do patients with HbAS (one normal, one abnormal chain) RBCs sickle?

A

2.5-4 kPa

110
Q

At what partial pressure of oxygen do patients with HbSS (two abnormal chains) RBCs sickle?

A

pO2 5-6 kPa

111
Q

How is a definitive diagnosis of sickle cell disease made?

A

haemoglobin electrophoresis

112
Q

What are 2 causes of benign paraproteinaemia?

A
  1. monoclonal gammopathy of undetermined significance (MGUS)
  2. transient paraproteinaemia (e.g. after infection)
113
Q

What are 4 malignant causes of paraproteinaemia?

A
  1. multiple myeloma
  2. Waldenstrom macroglobulinaemia
  3. primary amyloidosis
  4. B-cell lymphoproliferative disorders e.g. CLL, non-Hodgkin lymphoma
114
Q

What are 5 clinical features of paraproteinaemia?

A
  1. hyperviscosity syndrome
  2. neuropathy e.g. sensory, motor, autonomic
  3. renal dysfunction
  4. haematologic abnormalities e.g. anaemia, thrombocytopenia, leukopenia
  5. bone pain or pathologic fractures
115
Q

What are 3 key aspects of the management of polycythaemia vera?

A
  1. aspirin - reduces risk of thrombotic events
  2. venesection - first line to normalise Hb
  3. chemotherapy - hydroxyurea, phosphorus-32 therapy
116
Q

What are 2 options for chemotherapy for polycythaemia rubra vera?

A
  1. hydroxyurea
  2. phosphorus-32 therapy
117
Q

What are 3 key risks in polycythaemia vera?

A
  1. thrombotic events
  2. progression to myelofibrosis
  3. progression to acute leukaemia (chemotherapy increases risk)
118
Q

What are 5 features of post-thrombotic syndrome after DVT?

A
  1. painful, heavy calves
  2. pruritus
  3. swelling
  4. varicose veins
  5. venous ulceration
119
Q

What is the advice regarding compression stockings and post-thrombotic syndrome?

A

NICE advise do not offer elastic compression stockings to prevent PTS or VTE recurrence - but recommended once PTS has developed

120
Q

What are 5 causes of microcytic anaemia?

A
  1. iron-deficiency anaemia
  2. thalassaemia
  3. congenital sideroblastic anaemia
  4. anaemia of chronic disease (but more commonly normocytic)
  5. lead poisoning
121
Q

What does normal Hb with microcytosis raise the possibility of?

A

polycythaemia rubra vera

122
Q

In which condition can a microcytosis be disproportionate to the anaemia?

A

beta thalassaemia

123
Q

In which condition are ‘tear drop’ poikilocytes generally seen?

A

myelofibrosis (flat, elongated RBCs)

124
Q

In which condition are smear cells generally seen on a blood film?

A

chronic lymphocytic leukaemia (CLL)

125
Q

In what 2 conditions are spherocytes seen on a blood film?

A
  1. hereditary spherocytosis
  2. autoimmune haemolytic anaemia
126
Q

What are 2 conditions in which target cells (RBCs) are seen on blood film?

A
  1. iron-deficiency anaemia
  2. hyposplenism
127
Q

In which condition are Auer rods classically seen?

A

acute promyelocytic leukaemia

128
Q

What is the more common form of acute leukaemia in adults?

A

acute myeloid leukaemia

129
Q

What are 5 features of acute myeloid leukaemia?

A
  1. Anaemia
  2. Neutropenia - may be high WCCs, low functioning neutrophils
  3. thrombocytopenia + bleeding
  4. splenomegaly
  5. bone pain
130
Q

What are 3 poor prognostic features in AML?

A
  1. > 60 years
  2. > 20% blasts after first course of chemo
  3. cytogenetics: deletions of chromosome 5 or 7
131
Q

What causes acute promyelocytic leukaemia M3 genetically?

A

associated with t(15;17), fusion of PML and RAR-alpha genes

132
Q

What is the prognosis of acute promyelocytic leukaemia M3 compared with other AML?

A

good prognosis

133
Q

What age group is commonly affected by acute promeylocytic leukaemia M3?

A

younger than other AML types - avg 25 years

134
Q

What is often seen at presentation of Acute promyelocytic leukaemia M3?

A

DIC or thrombocytopenia

135
Q

What classification is used for AML?

A

French-American-British (FAB) classification, M0-M7

136
Q

When does WHO recommend platelet transfusion is given?

A

platelet count < 30 with clinically significant bleeding (haematemesis, melaena, prolonged epistaxis)

137
Q

When is a platelet threshold > 30 used?

A

maximum <100 for patients with severe bleeding or critical sites e.g. CNS

138
Q

Which type of blood product has the highest risk of bacterial contamination?

A

platelet transfusion

139
Q

What are the platelet thresholds that are aimed for before surgery / invasive procedure?

A
  • most patients: > 50
  • high risk of bleeding: 50-75
  • surgery at critical site: > 100
140
Q

What is the target level of platelets in someone without active bleeding or any planned procedure?

A

threshold of 10 (except if platelet transfusion CI / there are alternative treatments)

141
Q

What are 4 conditions when platelet transfusion should not be performed?

A
  1. chronic bone marrow failure
  2. autoimmune thrombocytopenia
  3. heparin-induced thrombocytopenia
  4. thrombotic thrombocytopenic purpura
142
Q

What is the most common inherited bleeding disorder?

A

von Willebrand’s disease

143
Q

What is the inheritance pattern of von Willebrand’s disease?

A

most commonly autosomal dominant (type 3, total lack of vWF = autosomal recessive)

144
Q

What type of bleeding does von Willebrand’s disease cause?

A

behaves like platelet disorder - epistaxis and menorrhagia common, haemarthroses and muscle haematomas rare

145
Q

What is the role of von Willebrand factor?

A

large glycoprotein which forms massive multimers; promotes platelet adhesion to damaged endothelium. also carrier molecule for factor VIII

146
Q

What are 3 types of von Willebrand’s disease?

A
  1. type 1: partial reduction in vWF (80% of patients)
  2. type 2: abnormal form of vWF
  3. type 3: total lack of vWF
147
Q

What are 4 blood test findings in von Willebrand’s disease?

A
  1. prolonged bleeding time
  2. prolonged APTT
  3. factor VIII levels may be moderately reduced
  4. defective platelet aggregation with ristocetin
148
Q

What are 3 aspects of the treatment of von Willebrand’s disease?

A
  1. TXA - mild bleeding
  2. desmopressin (DDAVP) - raises vWF levels by induced release from Weibel-Palade bodies in endothelial cells
  3. factor VIII ceoncentrate
149
Q

What is seen on a blood film in DIC and why?

A

schistocytes - microangiopathic haemolytic anaemia

150
Q

What are 4 things seen on FBC / clotting profile in DIC?

A
  1. reduced platelets
  2. reduced fibrinogen
  3. raised PT and APTT
  4. raised fibrinogen degradation products
151
Q

How can you distinguish between liver failure and DIC as causes for coagulopathy on blood tests?

A
  • liver failure: all clotting factors low except factor VIII, which is supranormal
  • DIC: all clotting factors depleted
152
Q

What are 3 causes why there is a paradoxical increased risk of venous thromboembolism in liver failure despite prolonged PT and APTT?

A
  1. supranormal levels of factor VIII
  2. reduced synthesis of protein c and protein s (vitamin K dependent)
  3. reduced anti-thrombin synthesis (non-vtamin K dependent)
153
Q

What is the management with antiplatlets/anticoagulation in someone with a history of stable CVD + indication for anticoagulation e.g. AF?

A

anticoagulant monotherapy without antiplatelets

154
Q

What is the antiplatelet/anticoagulant management of a patient post-ACS / PCI?

A
  • initially give 2 antiplatelets + 1 anticoagulant for 4 weeks - 6 months
  • then 1 antiplatelet + 1 anticoagulant for 12 months
155
Q

What is anti-streptolysin O antibody associated with in children?

A

Henoch-Schonlein purpura

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