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Year 5 path > haematology - bleeding disorders > Flashcards

haematology - bleeding disorders Flashcards

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1
Q

approaches to bleeding disorders

A
  1. bleeding patient
    - can have normal initial labs
  2. abnormal bleeding tests
    - little clinical sig without Hx of bleeding
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2
Q

broad classification of bleeding disorders

A

congenital
acquired

vascular defects
platelet disorders
coagulation disorders

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3
Q

features of vascular defects

A

superficial bleeding into skin and mucosal membranes

bleeding immediately after injury

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4
Q

features of platelet disorders

A

superficial bleeding into skin and mucosal membranes

bleeding immediately after injury

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5
Q

features of coagulation disorders

A

bleeding into deep tissues, muscles, and joints
delayed but severe bleeding after injury
prolonged bleeding

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6
Q

examples of congenital vascular defects

A

Osler-Weber-Rendu syndrome

connective tissue disease, eg. EDS

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7
Q

examples of acquired vascular defects

A

senile purpura
steroids
scurvy (perifollicular haemorrhages)
infection (eg. meningococcal measles, dengue)

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8
Q

platelet disorder classification

A

decreased function

  • acquired
  • congenital

decreased number

  • decreased production
  • increased destruction
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9
Q

causes of acquired decreased platelet function

A

aspirin
cardiopulmonary bypass
uraemia

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10
Q

causes of congenital decreased platelet function

A

storage pool disease

thrombasthenia (glycoprotein deficiency)

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11
Q

causes of thrombocytopenic platelet disorders with decreased production

A

bone marrow failure

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12
Q

causes of thrombocytopenic platelet disorders with increased destruction

A 
autoimmune thrombocytopenia purpura (AITP)
drugs, eg. heparin
DIC
HUS
TTP
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13
Q

peak age of acute ITP vs chronic ITP

A

acute: 2-6 years
chronic: adults

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14
Q

F:M of acute ITP vs chronic ITP

A

acute: 1:1
chronic: 3:1

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15
Q

preceding infection in acute ITP vs chronic ITP

A

acute: common
chronic: rare

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16
Q

onset of symptoms of acute ITP vs chronic ITP

A

acute: abrupt
chronic: abrupt - indolent

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17
Q

platelet count at presentation in acute ITP vs chronic ITP

A

acute: <20,000
chronic: <50,000

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18
Q

duration of acute ITP vs chronic ITP

A

acute: 2-6 weeks
chronic: long term (associated with autoimmune disease, CLL, HIV)

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19
Q

spontaneous remission of acute ITP vs chronic ITP

A

acute: common, self limiting
chronic: uncommon

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20
Q

treatment of chronic ITP

A

IVIg
steroids
splenectomy

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21
Q

examples of inherited coagulation disorders

A

haemophilia A
haemophilia B
Von Willebrand’s Disease

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22
Q

haemophilia A

A

factor VIII deficiency

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23
Q

inheritance pattern of haemophilia A

A

X linked recessive

rarely acquired

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24
Q

presentation of haemophilia A

A

often early in life or prolonged bleeding after surgery/trauma

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25
dx of haemophilia A
↑ APTT normal PT ↓ factor VIII assay
26
severity of haemophilia A depends on
factor level <1%: severe 1-5%: moderate 5-25%: mild
27
management of haemophilia A
avoid NSAIDS and IM injections desmopressin factor VIII concentrates replacement
28
desmopressin in treatment of haemophilia A
↑ VWF release which is factor VIII carrier
29
haemophilia B
factor IX deficiency
30
inheritance pattern of haemophilia B
X linked recessive
31
clinical presentation of haemophilia B
similar to that of haemophilia A
32
management of haemophilia B
factor IX concentrates
33
Von Willebrand's Disease
factor VIII deficiency and ↓ platelet function
34
types of VWD
quantitative qualitative ``` variable phenotype (from complete to asymptomatic/mild deficiency) type 1: low levels of VWF type 2: deficiency in VWF function type 3: absent VWF ```
35
inheritance pattern of VWD
autosomal dominant
36
presentation of VWD
mucocutaneous bleeding | bleeding indicative of coagulation disorders
37
dx of VWD
``` (↑) APTT normal PT ↓ factor VIII (↓) vWF antigen normal platelet count ```
38
management of VWD
prophylaxis indicated in some patients treatment of bleeds - desmopressin - VWF concentrate - factor VIII concentrate
39
examples of coagulation disorders
DIC liver disease vit K deficiency
40
DIC
widespread activation of coagulation | ↓ clotting factors and platelets
41
causes of DIC
``` malignancy sepsis trauma obstetric complications toxins ```
42
dx of DIC
↓ platelets ↓ fibrinogen ↑ D dimer ↑ PT
43
treatment of DIC
``` treat cause transfusions FFP platelets cryoprecipitate ```
44
liver disease coagulation disorder
↓ synthesis of II, V, VII, IX, V, VI and fibrinogen ↓ absorption of vit K abnormal platelet function
45
vit K deficiency coagulation disorder
vit K needed for factors II, VII, IX, X and protein C/S
46
warfarin may have what effect initially
pro-coagulant because vit needed for protein C/S
47
causes of vit K deficiency coagulation disorder
warfarin vit K malabsorption/malnutrition Abx therapy biliary obstruction
48
treatment of vit K deficiency coagulation disorder
IV vitamin K | FFP for acute haemorrhage
49
↑/= APTT = PT = bleeding time = fibrinogen = D dimer = platelet count
vit K deficiency | warfarin
50
= APTT = PT ↑ bleeding time = fibrinogen = D dimer = platelet count
aspirin | thienopyridines
51
= APTT ↑ PT = bleeding time = fibrinogen = D dimer = platelet count
early stage liver failure
52
↑ APTT ↑ PT ↑ bleeding time ↓ fibrinogen ↑ D dimer ↓ platelet count
end stage liver failure
53
= APTT = PT ↑ bleeding time = fibrinogen = D dimer = platelet count
uraemia
54
↑ APTT ↑ PT ↑ bleeding time ↓ fibrinogen ↑ D dimer ↓ platelet count
DIC
55
= APTT = PT ↑ bleeding time = fibrinogen = D dimer ↓↓ platelet count
TTP
56
↑ APTT ↑ PT (↑) bleeding time ↓ fibrinogen ↑↑ D dimer = platelet count
hyperfibrinolysis
57
vit K deficiency warfarin clotting screen
↑/= APTT = PT = bleeding time = fibrinogen = D dimer = platelet count
58
aspirin thienopyridines clotting screen
= APTT = PT ↑ bleeding time = fibrinogen = D dimer = platelet count
59
early stage liver failure clotting screen
= APTT ↑ PT = bleeding time = fibrinogen = D dimer = platelet count
60
end stage liver failure clotting screen
↑ APTT ↑ PT ↑ bleeding time ↓ fibrinogen ↑ D dimer ↓ platelet count
61
uraemia clotting screen
= APTT = PT ↑ bleeding time = fibrinogen = D dimer = platelet count
62
DIC clotting screen
↑ APTT ↑ PT ↑ bleeding time ↓ fibrinogen ↑ D dimer ↓ platelet count
63
TTP clotting screen
= APTT = PT ↑ bleeding time = fibrinogen = D dimer ↓↓ platelet count
64
hyperfibrinolysis clotting screen
↑ APTT ↑ PT (↑) bleeding time ↓ fibrinogen ↑↑ D dimer = platelet count

Year 5 path (16 decks)

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