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3MB Haematology > Bleeding disorders > Flashcards

Bleeding disorders Flashcards

1
Q

What is Henoch-Schonlein Purpura?

A

IgA vasculitis that presents with a purpuric rash affecting the lower limbs and buttocks in children. Inflammation occurs in the affected organs due to IgA deposits in the blood vessels

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2
Q

Henoch-Schonlein Purpura is often triggered by..

A

Upper airway infection or gastroenteritis

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3
Q

Four classic features of HSP are..

A

Purpura (100%)
Joint pain (75%)
Abdominal pain (50%)
Renal involvement (50%)

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4
Q

Abdominal pain in HSP can lead to..

A

Gastrointestinal haemorrhage, intussusception and bowel infarction

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5
Q

HSP affects the kidneys in around 50% of patients, causing..

A

IgA nephritis. Can lead to microscopic or macroscopic haematuria and proteinuria

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6
Q

T or F: If there is more than 2+ of protein on the urine dipstick the child has developed nephrotic syndrome and will have a degree of oedema

A

True

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7
Q

Ix for HSP

A

FBC + blood film
Renal profile

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8
Q

Tx for HSP

A

Analgesia for arthralgia
Nephropathy - supportive

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9
Q

Criteria for diagnosing HSP

A

EULAR/PRINTO/PRES criteria

Palpable purpura (not petichiae) + at least one of:

Diffuse abdominal pain
Arthritis or arthralgia
IgA deposits on histology (biopsy)
Proteinuria or haematuria

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10
Q

It is important that patients with HSP are monitored closely whilst the illness is active. They need close monitoring with repeated..

A

Urine dipstick monitoring for renal involvement
Blood pressure monitoring for hypertension

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11
Q

What is Thrombocytopenia?

A

Low platelet count

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12
Q

Reduced platelet production can occur with..

A

Certain viral infections (e.g., Epstein-Barr virus, cytomegalovirus and HIV)
B12 deficiency
Folic acid deficiency
Liver failure, causing reduced thrombopoietin production by the liver
Leukaemia
Myelodysplastic syndrome
Chemotherapy

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13
Q

Increased platelet destruction can occur with..

A

Sodium valproate and methotrexate
Alcohol
Immune thrombocytopenic purpura
Thrombotic thrombocytopenic purpura
Heparin-induced thrombocytopenia
Haemolytic uraemic syndrome

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14
Q

Platelet counts below 50 x 109/L will result in..

A

Easy bruising and prolonged bleeding times (nosebleeds, bleeding gums, heavy periods etc)

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15
Q

Platelet counts below 10 x 109/L are at high risk for..

A

Spontaneous bleeding:

Intracranial haemorrhage
Gastrointestinal bleeding

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16
Q

The top differentials of abnormal or prolonged bleeding to remember are..

A

Thrombocytopenia
Von Willebrand disease
Haemophilia A and haemophilia B
Disseminated intravascular coagulation (usually secondary to sepsis)

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17
Q

What is ITP?

A

Antibodies are created against glycoprotein IIb/IIIa or Ib-V-IX complex leading to thrombocytopenia

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18
Q

Characteristic of ITP

A

Purpura

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19
Q

Mx of ITP

A

Prednisolone
IV immunoglobulins
Thrombopoietin receptor agonists (e.g., avatrombopag)
Rituximab
Splenectomy

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20
Q

Children with ITP usually have an acute thrombocytopenia that may follow __________ or ____________. In contrast, adults tend to have a more __________ condition

A

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

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21
Q

Epidemiology of ITP in adults

A

More common in older females

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22
Q

Ix for ITP in adults

A

Full blood count: isolated thrombocytopenia
Blood film

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23
Q

Mx for ITP in adults

A

Oral prednisolone
IVIG if active bleeding or an urgent invasive procedure is required

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24
Q

What is Evan’s syndrome?

A

ITP in association with autoimmune haemolytic anaemia (AIHA)

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25
Q

ITP in children is an example of which type of reaction?

A

Type II hypersensitivity reaction

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26
Q

Ix for ITP in children

A

FBC
Blood film

Bone marrow examination if there is lymph node enlargement/ splenomegaly, high/ low white cells failure to resolve/respond to treatment

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27
Q

Mx of ITP in children

A

Resolves within 6 months
Avoid activities that may result in trauma (e.g. team sports)

If platelet is very low then:
Oral/ IV corticosteroid
IV immunoglobulins
Platelet transfusions in an emergency (e.g. active bleeding) but only a temporary measure as they are soon destroyed by the circulating antibodies

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28
Q

Give examples of drugs that cause thrombocytopenia

A

Quinine
Abciximab
NSAIDs
Diuretics: furosemide
Antibiotics: penicillins, sulphonamides, rifampicin
Anticonvulsants: carbamazepine, valproate
heparin

29
Q

If a patient with gestational ITP becomes dangerously thrombocytopenic, she will usually be treated with..

A

Steroids and a diagnosis of ITP assumed

30
Q

Pregnant women found to have low platelets during a booking visit or those with a previous diagnosis of ITP may need to be tested for..

A

Serum antiplatelet antibodies for confirmation

31
Q

Gestational thrombocytopenia does not affect the neonate, but ITP can do if..

A

Maternal antibodies cross the placenta

32
Q

T or F: Depending on the degree of thrombocytopenia in the newborn, platelet transfusions may be indicated. Serial platelet counts can also be performed to see whether there is an inherited thrombocytopenia

A

True

33
Q

What is Thrombotic Thrombocytopenic Purpura?

A

Tiny thrombi develop throughout the small vessels, using up platelets

34
Q

In TTP, as the problem is in the small vessels, it is described as a microangiopathy. It causes..

A

Thrombocytopenia
Purpura
Tissue ischaemia and end-organ damage

35
Q

Thrombi develop due to a problem with a specific protein called ADAMTS13. This protein normally..

A

Inactivates von Willebrand factor
Reduces platelet adhesion to vessel walls
Reduces clot formation

36
Q

Deficiency in the ADAMTS13 protein can be due to..

A

An inherited genetic mutation (hereditary)
Autoimmune disease, where antibodies are created against the protein (acquired)

37
Q

Thrombotic thrombocytopenic purpura overlaps with..

A

Haemolytic uraemic syndrome (HUS)

38
Q

Fluctuating neuro signs (microemboli)
Microangiopathic haemolytic anaemia
Thrombocytopenia
Renal failure

A

Thrombotic thrombocytopenic purpura

39
Q

Causes of TPP

A

Post-infection e.g. urinary, gastrointestinal
Pregnancy
Drugs: ciclosporin, oral contraceptive pill, penicillin, clopidogrel, aciclovir
Tumours
SLE/HIV

40
Q

In Heparin-Induced Thrombocytopenia, heparin targets a protein on platelets called..

A

Platelet factor 4 (PF4)

41
Q

Physiology of HIT

A

HIT antibodies bind to platelets and activate the clotting system, causing a hypercoagulable state and thrombosis (e.g., deep vein thrombosis). They also break down platelets and cause thrombocytopenia

42
Q

Ix of HIT

A

HIT antibodies on a blood sample

43
Q

Tx of HIT

A

Stopping heparin and using an alternative anticoagulant guided by a specialist (e.g., fondaparinux or argatroban)

44
Q

What is DIC?

A

Activation and release of TF which 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

45
Q

Causes of DIC

A

Sepsis and trauma
Obstetric complications e.g. aminiotic fluid embolism, HELLP
Malignancy

46
Q

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

A

DIC

47
Q

Prothrombin time: Prolonged
APTT: Normal
Bleeding time: Normal
Platelet count: Normal

A

Warfarin administration

48
Q

Prothrombin time: Normal
APTT: Normal
Bleeding time: Pronlonged
Platelet count: Normal

A

Aspirin administration

49
Q

Prothrombin time: Often normal (may be prolonged)
APTT: Prolonged
Bleeding time: Normal
Platelet count: Normal

A

Heparin

50
Q

Prothrombin time: Prolonged
APTT: Prolonged
Bleeding time: Prolonged
Platelet count: Low

A

DIC

51
Q

Von Willebrand disease mode of inheritance

A

Autosomal dominant

52
Q

Von Willebrand disease can also occur secondary to..

A

Underlying disease (e.g., leukaemia)

53
Q

Von Willebrand factor is important in..

A

Platelet adhesion and aggregation in damaged vessels

54
Q

What are the three types of VWF?

A

1: partial deficiency of VWF and is the most common and mildest type
2: reduced function of VWF
3: involves a complete deficiency of VWF and is the most rare and severe type

55
Q

History of unusually easy, prolonged or heavy bleeding:

Bleeding gums with brushing
Nosebleeds (epistaxis)
Easy bruising
Heavy menstrual bleeding (menorrhagia)
Heavy bleeding during and after surgical operations

A

VWF

56
Q

Ix for VWF

A

History of abnormal bleeding, family history, bleeding assessment tools and laboratory investigations

57
Q

Mx of VWF

A

In response to significant bleeding:

Desmopressin (stimulates the release of vWF from endothelial cells)
Tranexamic acid
Von Willebrand factor infusion
Factor VIII plus von Willebrand factor infusion

58
Q

Haemophilia mode of inheritance

A

X-linked recessive disorder of coagulation

59
Q

Haemophilia A is due to a deficiency of factor ____ whilst in haemophilia B (Christmas disease) there is a lack of factor ____

A

Haemophilia A is due to a deficiency of factor VIII whilst in haemophilia B (Christmas disease) there is a lack of factor IX

60
Q

Haemoarthroses
Haematomas
Prolonged bleeding after surgery or trauma

A

Haemophilia

61
Q

Ix for Haemophilia

A

Prolonged APTT
Bleeding time, thrombin time, prothrombin time normal

62
Q

Tx for Haemophilia

A

The affected clotting factors (VIII or IX) can be given by intravenous infusion, either regularly or in response to bleeding

63
Q

Up to 10-15% of patients with haemophilia A develop antibodies to..

A

Factor VIII treatment

64
Q

____________ is the commonest cause of primary haemostatic failure

A

Thrombocytopenia

65
Q

Multiple factor deficiencies that affect secondary haemostasis

A

Liver failure
Vitamin K Deficiency/Warfarin therapy
DIC

66
Q

Factors ___________ are carboxylated by vitamin K which is essential for function

A

II, VII, IX & X

67
Q

Where is Vit. K absorbed?

A

Upper intestine
Requires bile salts for absorption

68
Q

What causes Vit. K deficiency?

A

Poor dietary intake
Malabsorption
Obstructive jaundice
Vitamin K antagonists (warfarin)
Haemorrhagic disease of the newborn

3MB Haematology (23 decks)

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