Brainscape's Knowledge GenomeTM

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


See full index

Haematology > Hereditary Spherocytosis > Flashcards

Hereditary Spherocytosis Flashcards

1
Q

What is hereditary spherocytosis?

A

A condition where the red blood cells are sphere shaped, rather than the biconcave disc shape

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

How does the red blood cells being spherical rather than biconcave affect them?

A

It makes them fragile and easily destroyed when passing through the spleen.

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

What is hereditary spherocytosis the most common type of?

A

Hereditary haemolytic anaemia

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

What type of inheritance does hereditary spherocytosis have?

A

Autosomal dominant

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

How does hereditary spherocytosis present?

A
  • Failure to thrive
  • Jaundice
  • Anaemia
  • Gallstones
  • Splenomegaly
  • Aplastic crisis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What might an aplastic crisis be precipitated by?

A

Parvovirus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What might be elevated in hereditary spherocytosis?

A

MCHC (Mean Corpuscle Haemoglobin Concentration)
Raised Reticulocytes

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

What id MCHC a measure of?

A

Measurement of how much haemoglobin is inside one red blood cell

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

What are episodes of haemolytic crisis triggered by?

A

Infection

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

How does a haemolytic crisis present?

A

symptoms are more significant:
Haemolysis
Anaemia
Jaundice

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

How is hereditary spherocytosis diagnosed?

A
  • Spherocytes on the blood film.
  • Raised mean corpuscular haemoglobin concentration (MCHC)
  • Raised Reticulocytes
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What is the diagnostic test for hereditary spherocytosis?

A

EMA binding

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What is important to ask about when diagnosing hereditary spherocytosis?

A

Family history

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

What is the long term treatment of hereditary spherocytosis?

A
  • Folate supplementation

* Splenectomy

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

What is the acute treatment of hereditary spherocytosis?

A
  • Supportive treatment

* Transfusions may be required during acute crises.

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

What is a haemolytic crisis?

A

Large numbers of red blood cells are destroyed over a short time

17
Q

What is an aplastic crisis?

A

Rapid fall in haemoglobin levels associated with few or no reticulocytes
Resulting in Reticocytopenia
This indicates a failure of the bone marrow to respond to increased cell turnover

18
Q

What is a typical history for someone with hereditary spherocytosis?

A
  • Neonatal jaundice
  • Chronic symptoms although haemolytic crises may be precipitated by infection
  • Gallstones
  • Splenomegaly is common
19
Q

What results would suggest an aplastic crisis?

A

Severely low haemoglobin (anaemia)

Decreased reticulocyte count

20
Q

What are the biggest signs to look for in someone with hereditary spherocytosis?

A

Splenomegaly- pain in right upper quadrant
family history (of hereditary spherocytosis or splenectomy)
severely low haemoglobin

Haematology (36 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions