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Path Y > Haematology - Haemolytic Anaemias > Flashcards

Haematology - Haemolytic Anaemias Flashcards

1
Q

Recall some causes of intravascular vs extravascular haemolytic anaemias

A 
EXTRAvascular = hereditary spherocytosis 
INTRAvascualr = everything else (malaria, G6PDD, MAHA, PNH ETC)
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2
Q

Which infection are patients with haemolytic anaemias more susceptibile to?

A

Parvovirus B19
often develop gallstones

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3
Q

What is the expected LDH level in haemolytic anaemia?

A

High

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4
Q

What is the inheritance pattern of hereditary spherocytosis?

A

Autosomal dominant

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5
Q

How can hereditary spherocytosis be diagnosed (2)?

A

eosin-5 -maleimide (EMA) binding test

osmotic fragility test = increased susceptibility to lysis in hypotonic saline

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6
Q

In which parts of the world is G6PDD most common?

A

Where malaria is endemic

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7
Q

What is the inheritance pattern of G6PDD?

A

X linked recessive

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8
Q

What is the normal physiological role of G6PD?

A

NADPH generation

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9
Q

What is the main symptom of G6PDD in neonates?

A

Jaundice

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10
Q

Recall 4 triggers for a crisis in G6PDD

A

Moth balls
Fava beans
Anti-malarials
Antibiotics (sulphonamides, cipro

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11
Q

What is the typical appearance of erythrocytes in Pyruvate Kinase Deficiency?

A

echinocytes ‘hedgehog’ appearnce

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12
Q

What is the first investigation to do in haemolytic anaemia?

A

DAT/ Coombs test
Need to exclude autoimmune haemolysis

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13
Q

What does a positive urinary haemosiderin test show?

A

Intravascular haemolysis

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14
Q

What is homozygous hereditary elliptocytosis also known as?

A

Pyropoikilocytosis

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15
Q

Differentiate the class of immunoglobin involved in warm vs cold AIHA

A

Warm: IgG = EXTRAvascular haemolsysis - most common
Cold: IgM = INTRAvascular haemolysis

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16
Q

What abnormal shape of RBC may be seen on blood film in warm AIHA?

A

Spherocytes

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17
Q

Recall and compare the management of warm vs cold AIHA

A

Warm: steroids, splenectomy, immunosuppression
Cold: treat underlying condition (lymphoma/EBV), avoid the cold, chlorambucil

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18
Q

What is paroxysmal cold haemoglobinuria?

A

Hb in the urine, usually preceded by exposure to cold temperature and viral- infection

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19
Q

Which antibodies are the cause of paroxysmal cold haemoglobinuria?

A

Donath-Landsteiner antibodies

20
Q

What is paroxysmal nocturnal haemoglobinuria?

A

Acquired loss of protective surface markers on RBCs leading to complement-mediated lysis and nocturnal intravascular haemolysis

21
Q

Recall 2 symptoms of paroxysmal nocturnal haemoglobinuria

A

Morning haemoglobinuria
Throbosis

22
Q

Recall 2 ways that paroxysmal nocturnal haemoglobinuria can be diagnosed?

A
  1. Altered GPI (surface proteins on RBC)
  2. Ham’s test
23
Q

What is the pathophysiology of autoimmune Thrombotic thrombocytopenia purpura

A

mutation in ADAMST13 –> long strands of VWF (deficienct in VWF cleaving proteaste)

These VWF strands act like cheese wire in blood vessels

24
Q

What is the classic pentad of symptoms in TTP?

A

MAHA
Fever
Renal impairment
Neurological abnormalities
Thrombocytopaenia

25
Q

what’s a finding of extravascular HA

A

splenomegaly

26
Q

state 2 inherited conditions w membrane defects

A

hereditary spherocytosis
hereditary eliptocytosis

27
Q

state 2 inherited enzyme defects

A

G6PDD
pyruvate kinase def

28
Q

state 2 inherited haemoglobiopathies

A

SCD
thalassemia

29
Q

what is the membrane abnormality in hereditary spherocytosis

A

spectrin or ankyrin deficiency (membrane proteins)

30
Q

2 histological findings of G6PDD

A 
bite cells 
Heinz bodies (blue deposits, oxide Hb)
31
Q

echinocytes and spherocytes seen in what condition

A

pyruvate kinase deficiency
echinocytes = hedgehog appearance

32
Q

what stain for G6PDD

A

heinz body stains (methyl violet)

33
Q

what causes HUS

A

e coli

34
Q

what is the inheritance of hereditary eliptocytosis? what protein is affected

A

AD
spectrin

35
Q

4 consequences of haemolysis broadly

A

anaemia
erythroid hyperplasia (increased RBC production and reticulocyte circulation)
increased folate demand
susceptibility to parvovirus B19, galstones etc

36
Q

what does a positive DAT test indicate

A

autoimmune haemoytic anaemia

37
Q

what cell do you see in MAHA

A

schistocytes

(fragmented RBC)

38
Q

5 causes of MAHA

A

HUS
TTP
DIC
Pre-eclampsia

ADENOCARCINOMA

39
Q

define MAHA

A

mechanical RBC destruction (as forced through fibrin/platelet mesh in damaged cels)

40
Q

treatment of paroxsymal nocurnal heamoglobinuria

A

Eculizumab is a recombinant monoclonal antibody that inhibits terminal complement activation at the C5 protein and thereby reduces complement-mediated cell lysis

41
Q

treatment of TTP

A

PLASMA EXCHANGE

42
Q

triad of HUS

A

MAHA, thrombovytopaenia, AKI

43
Q

5 bllod findings common to HUS and TTP

A

Hb down

Platelets down (bleeding, petechiae, haematemeis, melena)

DAT/Coombs -ve (not autoimmune)

schistocytes on blood smear

signs of haemlosys = bilirubin up, reticulocytes up, LDH up

44
Q

thrombi are found where in HUS vs TTP

A

HUS - thrombi confined to kidney

TTP - cicrulation inclduing CNS

45
Q

does HUS of TTP usually affect children

A

HUS - children mre (e coli from kids touchign animlas etc)

TTP - more adults (ADAMTS13 mutation)

46
Q

does HUS typically have neuro sequelae

A

NO

HUS = renal failure

TTP= neuro (headache, sezire, redcued GCS, coma)

47
Q
A

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