Brainscape's Knowledge GenomeTM

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


See full index

Hematology > Disorders Of Red Cell Membrane > Flashcards

Disorders Of Red Cell Membrane Flashcards

1
Q

Weight percentage of red cell membrane

A

1%

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

Role of red cell membrane

A

Maintain RBC integrity, flexibility, durability, tensile strength ,

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

Red cell membrane components and function

A

Lipid bilayer (phospholipids, cholesterol -> permeability

Integral proteins

Membrane skeleton -> structural integrity

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

Major component of RCM

A

Lipids - 50-60%

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

Types of lipids in RCM

A 
Phosphatidylcholine 
Phosphatidylethanolamine 
Phosphatidylserine
Phosphatidylinositol 
Sphingomyelin 
Cholesterol
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Cholesterol function in RCM

A

Control membrane fluidity

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

Integral membrane proteins

A

Glycophorins

Rh proteins

Kidd glycoproteins

Duffy glycoproteins

Lutheran glycoproteins

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

Peripheral membrane proteins

A 
Spectrin
Actin
Ankaryn
Protein 4.1
Protein 4.2
Protein p55, adducin, dermatitis, tropomyosin
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Spectrin, actin and ankaryn functionsp

A

Maintain cellular shape
regulates natural mobility of integral membrane proteins
structural support for the lipid bilayer

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

Abnormality of this protein is the most common cause of typical hereditary spherocytosis

A

Ankaryn

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

Protein 4.1 role

A

Link Spectrin actin membrane to lipid bilayer

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

Protein 4.2 role

A

Stabilize Spectrin actin Ankaryn association with band 3

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

This type of proteins is responsible for maintenance of the structural integrity of the cell as well as the high tensile strength of the RCM

A

Horizontal proteins

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

What is the function of vertical Interactions of proteins

A

Stabilizes lipid bilayer

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

Most common disorders in the red cell membrane Hereditary anemia

A

Hereditary spherocytosis
Hereditary Elliptocytosis
Hereditary pyropoikilocytosis

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

What is hereditary spherocytosis

A

Disorders characterized by spherical red blood cell with increased osmotic fragility

17
Q

Most common hereditary anemia in people of northern European descent

A

Hereditary spherocytosis

18
Q

Main causes of hereditary spherocytosis ytosis

A

Deficiency of ankaryn spectrin and proteins 4.2

Which are involved in vertical interactions between membrane skeleton and lipid bilayer

19
Q

Sodium potassium and water content in hereditary spherocytosis

A

Potassium and water are reduced

sodium is increased

20
Q

What happens to read cell in the spring when there is hereditary spherocytosis

A

Red cells cannot deformed themselves to go through the splenic microcirculation so destruction of RBC sites in the spleen especially in acidic pH and low glucose levels

21
Q

Clinical features of hereditary spherocytosis

A 
Slight Anemia
Hb does not go below 7.5 g/dl
Jaundice 
splenomegaly
Visible in multiple members of one family 
gallstone 
leg ulcers
 aplastic crisis especially in parvovirus
22
Q

Laboratory findings of hereditary Spherocytosis

A 
Anemia 
bloods film 
Multiple Spherocytes
no central pallor
 reduced diameter 
MCHC increased 
Osmotic fragility increased 
Mean cell fragility over  0.45 
reticulocyte count 5 to 20% 
negative coomb tests( so not inflammatory in origin )
23
Q

Treatment of Hereditary spherocytosis

A

Folic acid

splenectomy if severe

24
Q

What is Hereditary elliotocytosis

A

Similar to HS but elliptocytes instead

25
Q

Who is first patient of HE

A

Medical students in dresbach

26
Q

Incidence of HE

A

1 in 2000 or 4000 individuals

27
Q

Maine population affected by hE

A

African and Mediterranean

28
Q

Is there resistance to malaria in HE

A

Yes to some extent

29
Q

Are most people are symptomatic in HE

A

Yes

30
Q

Is HE autosomal dominant or recessive

A

Dominance in most patients

31
Q

Is HE milder than HS

A

Yes requires the next to me on the rarely

32
Q

Is HE due to vertical or horizontal to interactions defect

A

Horizontal

33
Q

Why is HE mostly diagnosed incidentally

A

Because it’s mostly asymptomatic

34
Q

What are other examples of hereditary red cell membrane disorders

A

Hereditary stomatacytosis
Hereditary Pyropoilikocytosis
South east Asian ovalocytosis

Hematology (24 decks)

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