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Year 5 path > haematology - bleeding disorders > Flashcards

haematology - bleeding disorders Flashcards

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
Q

dx of haemophilia A

A

↑ APTT
normal PT
↓ factor VIII assay

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26
Q

severity of haemophilia A depends on

A

factor level
<1%: severe
1-5%: moderate
5-25%: mild

27
Q

management of haemophilia A

A

avoid NSAIDS and IM injections
desmopressin
factor VIII concentrates replacement

28
Q

desmopressin in treatment of haemophilia A

A

↑ VWF release which is factor VIII carrier

29
Q

haemophilia B

A

factor IX deficiency

30
Q

inheritance pattern of haemophilia B

A

X linked recessive

31
Q

clinical presentation of haemophilia B

A

similar to that of haemophilia A

32
Q

management of haemophilia B

A

factor IX concentrates

33
Q

Von Willebrand’s Disease

A

factor VIII deficiency and ↓ platelet function

34
Q

types of VWD

A

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
Q

inheritance pattern of VWD

A

autosomal dominant

36
Q

presentation of VWD

A

mucocutaneous bleeding

bleeding indicative of coagulation disorders

37
Q

dx of VWD

A 
(↑) APTT 
normal PT
↓ factor VIII
(↓) vWF antigen
normal platelet count
38
Q

management of VWD

A

prophylaxis indicated in some patients

treatment of bleeds

  • desmopressin
  • VWF concentrate
  • factor VIII concentrate
39
Q

examples of coagulation disorders

A

DIC
liver disease
vit K deficiency

40
Q

DIC

A

widespread activation of coagulation

↓ clotting factors and platelets

41
Q

causes of DIC

A 
malignancy
sepsis
trauma
obstetric complications
toxins
42
Q

dx of DIC

A

↓ platelets
↓ fibrinogen
↑ D dimer
↑ PT

43
Q

treatment of DIC

A 
treat cause
transfusions
FFP
platelets
cryoprecipitate
44
Q

liver disease coagulation disorder

A

↓ synthesis of II, V, VII, IX, V, VI and fibrinogen
↓ absorption of vit K
abnormal platelet function

45
Q

vit K deficiency coagulation disorder

A

vit K needed for factors II, VII, IX, X and protein C/S

46
Q

warfarin may have what effect initially

A

pro-coagulant because vit needed for protein C/S

47
Q

causes of vit K deficiency coagulation disorder

A

warfarin
vit K malabsorption/malnutrition
Abx therapy
biliary obstruction

48
Q

treatment of vit K deficiency coagulation disorder

A

IV vitamin K

FFP for acute haemorrhage

49
Q

↑/= APTT
= PT
= bleeding time

= fibrinogen
= D dimer
= platelet count

A

vit K deficiency

warfarin

50
Q

= APTT
= PT
↑ bleeding time

= fibrinogen
= D dimer
= platelet count

A

aspirin

thienopyridines

51
Q

= APTT
↑ PT
= bleeding time

= fibrinogen
= D dimer
= platelet count

A

early stage liver failure

52
Q

↑ APTT
↑ PT
↑ bleeding time

↓ fibrinogen
↑ D dimer
↓ platelet count

A

end stage liver failure

53
Q

= APTT
= PT
↑ bleeding time

= fibrinogen
= D dimer
= platelet count

A

uraemia

54
Q

↑ APTT
↑ PT
↑ bleeding time

↓ fibrinogen
↑ D dimer
↓ platelet count

A

DIC

55
Q

= APTT
= PT
↑ bleeding time

= fibrinogen
= D dimer
↓↓ platelet count

A

TTP

56
Q

↑ APTT
↑ PT
(↑) bleeding time

↓ fibrinogen
↑↑ D dimer
= platelet count

A

hyperfibrinolysis

57
Q

vit K deficiency
warfarin

clotting screen

A

↑/= APTT
= PT
= bleeding time

= fibrinogen
= D dimer
= platelet count

58
Q

aspirin
thienopyridines

clotting screen

A

= APTT
= PT
↑ bleeding time

= fibrinogen
= D dimer
= platelet count

59
Q

early stage liver failure

clotting screen

A

= APTT
↑ PT
= bleeding time

= fibrinogen
= D dimer
= platelet count

60
Q

end stage liver failure

clotting screen

A

↑ APTT
↑ PT
↑ bleeding time

↓ fibrinogen
↑ D dimer
↓ platelet count

61
Q

uraemia

clotting screen

A

= APTT
= PT
↑ bleeding time

= fibrinogen
= D dimer
= platelet count

62
Q

DIC

clotting screen

A

↑ APTT
↑ PT
↑ bleeding time

↓ fibrinogen
↑ D dimer
↓ platelet count

63
Q

TTP

clotting screen

A

= APTT
= PT
↑ bleeding time

= fibrinogen
= D dimer
↓↓ platelet count

64
Q

hyperfibrinolysis

clotting screen

A

↑ APTT
↑ PT
(↑) bleeding time

↓ fibrinogen
↑↑ D dimer
= platelet count

Year 5 path (16 decks)

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