Haematology 5 - Haemolytic anaemias Flashcards Preview

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Flashcards in Haematology 5 - Haemolytic anaemias Deck (25)
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1

Recall some causes of intravascular vs extravascular haemolytic anaemias

Intravascular:
- Malaria (black water fever)
- G6PDD
- MAHA (due to HUS/TTP/PNH)
Extravascular:
- Autoimmune
- hereditary spherocytosis

2

Which infection are patients with haemolytic anaemias more susceptibile to?

Parvovirus B19

3

What is the expected LDH level in haemolytic anaemia?

High

4

What is the inheritance pattern of hereditary spherocytosis?

Autosomal dominant

5

How can hereditary spherocytosis be diagnosed?

Examination of mean cell fluorescence

Osmotic fragility test

6

What is the difference in clinical features of hereditary elliptocytosis in a heterozygote vs a homozygote?

Heterozygote: no polychromasia
Homozygote: Severe haemolytic anaemia (pyropoikilocytosis)

7

In which parts of the world is G6PDD most common?

Where malaria is endemic

8

What is the inheritance pattern of G6PDD?

X linked recessive

9

What is the normal physiological role of G6PD?

NADPH generation

10

What is the main symptom of G6PDD in neonates?

Jaundice

11

Recall 3 triggers for a crisis in G6PDD

Moth balls
Fava beans
Anti-malarials

12

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

Kinocytes ('spiky')

13

What is the first investigation to do in haemolytic anaemia?

DAT/ Coombs test
Need to exclude autoimmune haemolysis

14

What does a positive urinary haemosiderin test show?

Intravascular haemolysis

15

What is homozygous hereditary elliptocytosis also known as?

Pyropoikilocytosis

16

Differentiate the class of immunoglobin involved in warm vs cold AIHA

Warm: IgG
Cold: IgM

17

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

Spherocytes

18

Recall and compare the management of warm vs cold AIHA

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

19

What is paroxysmal cold haemoglobinuria?

Hb in the urine, usually caused by a viral infection

20

Which antibodies are the cause of paroxysmal cold haemoglobinuria?

Donath-Landsteiner antibodies

21

What is paroxysmal nocturnal haemoglobinuria?

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

22

Recall 2 symptoms of paroxysmal nocturnal haemoglobinuria

Morning haemoglobinuria
Throbosis

23

Recall 2 ways that paroxysmal nocturnal haemoglobinuria can be diagnosed?

1. Altered GPI (surface proteins on RBC)
2. Ham's test

24

What is the pathophysiology of autoimmune TTP?

Antibodies against ADAMST13 --> long strands of VWF

These VWF strands act like cheese wire in blood vessels

25

What is the classic pentad of symptoms in TTP?

MAHA
Fever
Renal impairment
Neurological abnormalities
Thrombocytopaenia

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