2: Microangiopathic Haemolytic Anaemias Flashcards

1
Q

What is a microangiopathic haemolytic anaemia

A

RBC damage in circulation causing intravascular haemolysis

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2
Q

What are the 3 types of MAHA

A
  • DIC
  • TTP
  • HUS
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3
Q

Which age group does TTP occur in

A

Middle-age (40 years)

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4
Q

what. gender is TTP more common in

A

women

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5
Q

what ethnicity is TTP more common in

A

afro-carribean

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6
Q

what causes TTP

A

autoantibodies to ADAMST13 a protein that cleaves and inactive VWF

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7
Q

what is the mnemonic to remember symptoms in TTP

A

Nasty Fever Ruined My Tubes

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8
Q

what neurological symptoms are associated with TTP

A
  • Headache
  • Altered mental status
  • Seizures
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9
Q

what is the F in TTP

A

Fever

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10
Q

what are symptoms of renal function impairment in TTP

A
  • Proteinuria

- Haematuria

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11
Q

what are symptoms of MAHA

A
  • Pallour
  • Dyspneoa
  • Jaundice
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12
Q

what are symptoms of thrombocytopenia

A
  • Bleeding gums
  • Petechiae
  • Purpura
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13
Q

explain pathophysiology of MAHA

A
  • Autoantibodies to ADAMST13 means no longer can cleave VWF causing permanent activation
  • Activation results in micro thrombi formation which occlude arterioles and capillaries resulting in fragmentation of RBC
  • Microthrombi occluding arterioles and capillaries result in ischaemia of end organs (kidneys and brain)
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14
Q

how will FBC present in TTP

A
  • Anaemia

- Thrombocytopenia

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15
Q

how will coagulation studies present in TTP

A
  • Increase APTT and PT
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16
Q

how will d-dimer present in TTP and why

A
  • Increase - due to clot degredation
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17
Q

explain coombs test in MAHA

A

Coombs test will be negative

18
Q

what will be seen on peripheral blood smear in TTP

A

Schistocytes

19
Q

what may be seen on U+E and why

A

Increase urea and creatinine due to renal failure

20
Q

what is used to managed TTP

A

Plasma exchange therapy

Glucocorticoids

21
Q

in which age group does haemolytic uraemia syndrome occur

A

Under 5-years

22
Q

what infection causes haemolytic uraemia syndrome

A

E.COLI O157:H7

23
Q

explain manifestation of haemolytic uraemia syndrome

A

Diarrhoea illness then 5-10d later manifests with MAHA, thrombocytopenia and renal failure

24
Q

how does thrombocytopenia present clinically

A

Bleeding
Petechiae
Purpura

25
how does MAHA present clinically
Fatigue Dyspneoa Pallour Jaundice
26
how does impaired renal function present
Oliguria Haematuria Proteinuria
27
how does FBC present in HUS
Anaemia | Thrombocytopenia
28
what is a sign of haemolysis
Low haptoglobin Increase LDH Increase reticulocytes
29
what is haptoglobin
- Protein binds free Hb | - In intravascular Hb it is low
30
how do coagulation studies present in HUS
increased PT and APTT
31
what will be seen on blood film in HUS
schistocytes
32
how is HUS managed
correct abnormalities (eg. electrolytes), dialysis if AKI persists
33
what should not be given in HUS
antibiotics
34
what is DIC
Systemic activation coagulation cascade uses coagulation factors resulting in secondary bleeding
35
what are 5 causes of DIC
- Sepsis - Leukaemia - Obstetric (HELLP) - Acute pancreatitis - Amphetamine OD - Acute haemolytic transfusion reaction
36
what are the thrombotic symptoms of DIC
- Stroke - Purpura fulminans - Jaundice - AKI - VTE - ARDS
37
what skin sign indicates DIC
Purpura fulminans
38
what are the bleeding symptoms of DIC
- Petechiae - Haematuria - Haematemesis
39
what will be seen on FBC in DIC
- Anaemia | - Thrombocytopenia
40
what will be seen on coagulations studies in DIC
- Increase PT and APTT
41
what will be seen on blood film in DIC
Schistocytes
42
how is DIC managed
Treat underling cause