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Haematology > 2: Microangiopathic Haemolytic Anaemias > Flashcards

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
Q

how does MAHA present clinically

A

Fatigue
Dyspneoa
Pallour
Jaundice

26
Q

how does impaired renal function present

A

Oliguria
Haematuria
Proteinuria

27
Q

how does FBC present in HUS

A

Anaemia

Thrombocytopenia

28
Q

what is a sign of haemolysis

A

Low haptoglobin
Increase LDH
Increase reticulocytes

29
Q

what is haptoglobin

A
  • Protein binds free Hb

- In intravascular Hb it is low

30
Q

how do coagulation studies present in HUS

A

increased PT and APTT

31
Q

what will be seen on blood film in HUS

A

schistocytes

32
Q

how is HUS managed

A

correct abnormalities (eg. electrolytes), dialysis if AKI persists

33
Q

what should not be given in HUS

A

antibiotics

34
Q

what is DIC

A

Systemic activation coagulation cascade uses coagulation factors resulting in secondary bleeding

35
Q

what are 5 causes of DIC

A
  • Sepsis
  • Leukaemia
  • Obstetric (HELLP)
  • Acute pancreatitis
  • Amphetamine OD
  • Acute haemolytic transfusion reaction
36
Q

what are the thrombotic symptoms of DIC

A
  • Stroke
  • Purpura fulminans
  • Jaundice
  • AKI
  • VTE
  • ARDS
37
Q

what skin sign indicates DIC

A

Purpura fulminans

38
Q

what are the bleeding symptoms of DIC

A
  • Petechiae
  • Haematuria
  • Haematemesis
39
Q

what will be seen on FBC in DIC

A
  • Anaemia

- Thrombocytopenia

40
Q

what will be seen on coagulations studies in DIC

A
  • Increase PT and APTT
41
Q

what will be seen on blood film in DIC

A

Schistocytes

42
Q

how is DIC managed

A

Treat underling cause

Haematology (19 decks)

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