Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Pathology (Guide) > Haematology - Haemolytic Anaemias > Flashcards

Haematology - Haemolytic Anaemias Flashcards

1
Q

What is haemolytic anaemia?

A

The breakdown of red blood cells before their normal life span of ~120 days

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What is intravascular haemolytic anaemia and three causes?

A

Where red blood cells are destroyed in circulation (they rupture and release their contents)
- Alloimmune
- Autoimmune
- Hereditary spherocytosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What is extravascular haemolytic anaemia and four causes?

A

Where red blood cells are destroyed in the reticuloendothelial system
- Malaria
- Drugs
- G6PD deficiency
- PNH (Paroxysmal Nocturnal Haemoglobinuria)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What are the biochemical features of all haemolytic anaemias?

A
  • Increased unconjugated bilirubin
  • Increased urobilinogen (in urine)
  • Increased LDH
  • Reticulocytosis (Macro/normocytic + Polychromasia)
  • ?Pigmented gallstones
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What are the biochemical features of intravascular haemolytic anaemia?

A
  • Increased Hb
  • Decreased Haptoglobin (binds free Hb)
  • Haemoglobinuria (dark red urine)
  • Methaemalbuminaemia (Haem + albumin in blood)
  • Increased LDH
  • Decreased haptoglobin
  • Diptick = Blood +
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What are the biochemical features of extravascular haemolytic anaemia?

A
  • Urobilinogen
  • Dipstick = Blood -
  • Spherocytes (on blood smear)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What is a clinical feature of extravascular haemolytic anaemia?

A

Splenomegaly

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What is erythroid hyperplasia?

A

An increased count of erythroid precursor cells as a response to peripheral RBC loss/destruction.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What are erythroid hyperplasia states susceptible to?

A
  • Parvovirus B19 (aplastic crisis)
  • Iron overload
  • Osteoporosis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What are reticulocytes?

A

Immature RBCs halted in the process of maturation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What is a normal reticulocyte count?

A

0.5-2.5%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What happens to the reticulocyte count of a patient who is acutely anaemia and why?

A
  • It increases (high reticulocyte count)
  • Bone marrow is responding and working harder to produce more RBCs
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

When is a reticulocyte count raised?

A

With peripheral causes of anaemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What are the inherited causes of haemolytic anaemia and some examples?

A

Membrane Defect:
- Hereditary spherocytosis
- Hereditary elliptocytosis

Enzyme Defect:
- G6PD Deficiency
- Pyruvate kinase deficiency

Haemaglobinopathies:
- Sickle cell disease
- Thalassaemias

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What are acquired causes of haemolytic anaemia and some examples?

A

Immune:
- Autoimmune (e.g. Warm + Cold)
- Alloimmune (e.g. Haemolytic transfusion reactions + ABO/Rhesus incompatibility)

Non-immune:
- Mechanical (e.g. Metal valves + trauma)
- Paroxysmal Nocturnal Haemaglobinuria
- Microangiopathic Haemolytic Anaemia
- Infections (e.g. Malaria)
- Drugs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What is hereditary spherocytosis?

A

A defect in the vertical interaction of RBC membrane

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What inheritance pattern is hereditary spherocytosis?

A

Autosomal Dominant

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What is hereditary spherocytosis susceptible to?

A

Effects of Parvovirus B19
Often develop gallstones

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Which membrane proteins is hereditary spherocytosis deficient in?

A

Spectrin / Ankyrin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What type of haemolysis does hereditary spherocytosis show and what clinical sign would it present with?

A

Extravascular haemolysis
Splenomegaly

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

What test results would hereditary spherocytosis show?

A
  • Blood Film: Spherocytes + Polychromasia
  • Increased osmotic fragility (lysis in hypotonic solutions)
  • Coombs/DAT negative
  • Flow cytometry/EMA binding test: Positive Eosin-5-maleimide (MOST SENSITIVE)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

What is the treatment for hereditary spherocytosis?

A

Folic acid
Splenectomy in some cases

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

What is hereditary elliptocytosis?

A

A defect in the horizontal transmission of RBCs membrane

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

What type of inheritance pattern does hereditary elliptocytosis have?

A

Autosomal Dominant (mostly)

EXCEPT: Hereditary pyropoikilocytosis (Autosomal Recessive)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

What type of mutation is present in hereditary elliptocytosis?

A

Spectrin mutation (membrane protein)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

What is hereditary pyropoikilocytosis?

A

A type of hereditary elliptocytosis where erythrocytes are abnormal sensitive to heat

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

What symptoms are present with hereditary elliptoocytosis?

A

Most asymptomatic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

What is seen on the blood film of hereditary elliptocytosis

A

Elliptocytes (elliptical RBCs)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

What is South Asian Ovalocytosis and it’s mode of inheritance?

A

An autosomal recessive, heterozygote defect that is similar to hereditary elliptocytosis that presents with ovalocytes on blood film.
Can offer malarial protection

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

What is the commonest RBC enzyme defect?

A

Glucose-6-phosphate dehydrogenase deficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

What is the mode of inheritance of glucose-6-phosphate dehydrogenase deficiency?

A

X-linked

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

Where is glucose-6-phosphate dehydrogenase deficiency prevalent?

A

In areas of malarial endemicity
- Africa
- Mediterranean
- Middle East

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

What are the symptoms of glucose-6-phosphate dehydrogenase deficiency?

A

Rapid anaemia + jaundice

34
Q

What causes rapid anaemia in glucose-6-phosphate dehydrogenase deficiency?

A

Acute haemolysis following an exposure to oxidative stres

35
Q

What is seen on a blood film for glucose-6-phosphate dehydrogenase deficiency?

A
  • Bite cells
  • Heinz bodies (blue deposits, oxidized Hb)
36
Q

What is the action and role of glucose-6-phosphate dehyrogenase?

A

It generates NADPH via the pentose phosphate pathway
It helps RBCs make glutathione which protects them from oxidant damage?

37
Q

What are some precipitants for Glucose-6-phosphate dehydrogenase deficiency?

A
  • Drugs (2-3 days after starting - primaquine, sulfonamides, aspirin)
  • Broad/Fava beans (1 day after eating - favism)
  • Acute stressors
  • Moth Balls
  • Acute Infection
38
Q

What are some clinical findings of glucose-6-phosphate dehydrogenase deficiency?

A

Intravascular haemolysis
- Dark urine
- Haemoglobinuria
- Decreased haptoglobin
- Increased unconjugated bilirubin

39
Q

How is glucose-6-phosphate dehydrogenase deficiency diagnosed?

A

Enzyme assay ~2-3months post-crisis
- Young RBCs may have sufficient enzyme results so may appear normal

40
Q

What is the treatment for Glucose-6-phosphate dehydrogenase deficiency?

A

AVOID PRECIPITANTS
- Transfuse if severe
- Genetic screening (rare subtypes give chronic haemolysis for which splenectomy can be needed)
- Supportive treatment

41
Q

What is the mode of inheritance of pyruvate kinase deficiency?

A

Autosomal Recessive
(AD has been observed with the disorder)

42
Q

What are the clinical features of pyruvate kinase deficiency?

A
  • Severe neonatal jaundice
  • Splenomegaly
  • Haemolytic anaemia
43
Q

What is the treatment of pyruvate kinase deficiency?

A

?Blood transfusion
?Splenectomy

Most don’t require treatment

44
Q

What blood test is used to identify an Autoimmune Acquired Haemolytic Anaemia?

A
  • Direct Antiglobulin Test (DAT) / Coombs Test
  • Positive
45
Q

What are the two types of Autoimmine Acquired Haemolytic Anaemia and which is more common?

A

Warm (WAIHA) - MOST COMMON
Cold agglutinin disease

46
Q

What are some features of WAIHA?

A
  • 37C
  • IgG (Warm in Greece)
  • Positive Coombs Test
47
Q

What type of haemolysis does WAIHA show?

A

Extravascular

48
Q

What is seen on the blood film of WAIHA?

A

Spherocytes

49
Q

What are some features of Cold agglutinin disease?

A
  • <37C
  • IgM (Cold in Moscow)
  • Often with Raynaud’s
50
Q

What type of haemolysis does cold agglutinin disease show?

A

Intravascular

51
Q

What are some causes of WAIHA?

A
  • Idiopathic (main cause)
  • Lymphoma
  • CLL
  • SLE
  • Methyldopa
52
Q

What are some causes of Cold agglutinin disease?

A
  • Idiopathic (main cause)
  • Lymphoma infections: EBV, Mycoplasma, Hepatitis C
53
Q

What is the Management of WAIHA?

A
  • Treat underlying cause
  • Steroids
  • Splenectomy
  • Immunosuppression
54
Q

What is the management of cold agglutini disease?

A
  • Treat underlying conditino
  • Avoid the cold
  • Chemotherapy if lymphoma
  • Steroids
55
Q

What are some symptoms of haemolytic anaemia?

A
  • Pallor
  • Jaundice
  • Splenomegaly
  • Pigmenturia
  • FHx
56
Q

What is the cause of haemoglobinuria in Paroxysmal Cold Haemoglobinuria?

A

Viral infection:
- Measles
- Syphilis
- VZV

57
Q

What type of antibodies are found in Paroxysmal Cold Haemoglobinuria?

A

Donath-Landsteiner antibodies

58
Q

What is the pathophysiology of Paroxysmal Cold Haemoglobinuria?

A

Donath-Lansteiner antibodies stick to RBCs in the cold and cause complement-mediated haemolysis on rewarming.

Self-limiting: IgG dissociates at higher temperatures than IgM

59
Q

What test is used to determine a non-immune acquired haemolytic anaemia?

A

Direct Antiglobulin Test (DAT)/Coombs Test
NEGATIVE

60
Q

What are some general signs of non-immune acquired haemolytic anaemias?

A
  • Mechanical trauma
  • Infections
  • Hypersplenism
61
Q

How is Paroxysmal Nocturnal Haemoglobinuria caused?

A

An acquired loss of protective surface GPI markers on RBCs (platelets + neutrophils) cause complement-mediated lysis leading to chronic intravascular haemolysis, especially at night.

62
Q

What are some signs/symptoms of paroxysmal nocturnal haemoglobinuria?

A
  • Morning haemoglobinuria
  • Thrombosis (+ Budd-chiari syndrome)
63
Q

How is paroxysmal nocturnal haemoglobinuria diagnosed?

A
  • Immunophenotype shows altered GPI
  • HAM’S TEST (in vitro acid-induced lysis)
64
Q

What is the treatment for paroxysmal nocturnal haemoglobinuria?

A
  • Iron/folate supplements
  • Prophylactic vaccines/antibiotics
  • Expensive monoclonal antibodies (ECULIZUMAB) that prevents complement from binding RBCs
65
Q

What is microangiopathic haemolytic anaemia?

A

A non-immune-mediated, small vessel disease

66
Q

What is the pathophysiology of microangiopathic haemolytic anaemia?

A

Damage to endothelial cells causes fibrin deposition and platelet aggregation (TBCs forced through fibrin/platelet mesh) leading to RBC fragmentation

67
Q

What is seen on a blood film/tests for microangiopathic haemolytic anaemia?

A
  • Schistocytes
  • Thrombocytopaenia
68
Q

What are some causes of microangiopathic haemolytic anaemia?

A
  • HUS (Haemolytic Uraemic Syndrome)
  • TTP (Thrombotic Thrombocytopaenic Purpura)
  • Pre-eclampsia
  • Eclampsia
  • Reactions (usually plasma exchange)
69
Q

What is the management of microangiopathic haemolytic anaemia?

A
  • Treat underlying cause
  • Supportive
70
Q

What are the pentad of symptoms of thrombotic thrombocytopaenic purpura?

A
  • MAHA
  • Fever
  • Renal impairment (less pronounced than HUS)
  • Neuro abnormalities
  • Thrombocytopaenia
71
Q

What are the causes of thrombotic thrombocytopaenic purpura?

A
  • Inherited
  • Acquired
72
Q

What is the treatment of thrombotic thrombocytopaenic purpura?

A

Emergency plasma exchange (Haematological emergency)
+ Supportive Management

73
Q

What is the autoimmune pathophysiology of thrombotic thrombocytopaenic purpura?

A

ADAMTS13 defiency causes a breakdown of multimers of vWF leading to long strands of vWF which act like cheese wire in the blood vessels, cutting up RBCs

74
Q

Which protein is associated with TTP?

A

ADAMTS13

75
Q

What are some features of haemolytic uraemic syndrome?

A
  • Common in children + elderly
  • Less severe in children
  • Usually self-limiting
76
Q

What is the primary cause of haemolytic uraemic syndrome?

A

Escherichia Coli 0157:H7 shiga-like-toxin

77
Q

How is haemolytic uraemic syndrome caused?

A

The E. coli (Esherichia coli 0157:H7) shiga-like-toxin damages endothelial cells, forms a fibrin mesh and damages RBCs + impaired renal function + MAHA

78
Q

What are the symptoms of haemolytic uraemic syndrome?

A
  • Diarrhoea
  • Renal failure
  • No neuro problems
79
Q

When do the symptoms appear in haemolytic uraemic syndrome?

A

After diarrhoea-illness
DO NOT GIVE ABX

80
Q

What is the management of haemolytic uraemic syndrome?

A

Supportive

81
Q

What is the triad of haemolytic uraemic syndrome?

A
  • MAHA
  • Acute renal failure
  • Thrombocytopaenia

Pathology (Guide) (84 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions