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Flashcards in Pancytopenia Deck (88)
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1

What does 'pan' mean?

Generalised

2

What does 'cyto' mean?

Prefix denoting a cell

3

What does 'penia' mean?

Lack/deficiency (in blood)

4

What is pancytopenia?

A deficiency of blood cells of ALL lineages (but generally excludes lymphocytes).

5

** What is pancytopenia? **

A deficiency of blood cells of ALL lineages (but generally excludes lymphocytes).

6

What does pancytopenia NOT always mean?

Bone marrow failure

7

For steady state, cell production = cell destruction

T

8

In erythropoiesis, what cells are seen in the blood and not the bone marrow?

* Reticulocyte
* Erythrocyte

9

Neutrophils are properly segmented

T

10

What is the immediate precursor to platelets?

Megakaryocyte

11

Megakarocytes are p________

Polypoid

12

In what cells does 'the nucleus divides very quickly without the cytoplasm having divided'

Megakarocyte

13

What is the life span of red cells?

120 days

14

What is the lifespan of platelets?

7-10 days

15

What is the lifespan of neutrophils?

7-8 hours

16

If bone marrow fails, in order of first to drop and last to drop, list the cells.

* Neutrophils
* Platelets
* Red cells

17

What are the 2 main groups of causes of pancytopenia?

1. REDUCED production
2. INCREASED destruction

18

What is the basic cause of reduced production of cells, leading to pancytopenia?

Bone marrow failure

19

Name the 2 main things that lead to bone marrow failure.

1. Inherited syndromes
2. Acquired conditions (where ‘production machinery’ comes to a halt)

20

Name the 2 main things that lead to bone marrow failure.

1. Inherited syndromes
2. Acquired conditions (where ‘production machinery’ comes to a halt) - primary or secondary

21

Describe primary vs secondary acquired bone marrow failure.

Primary

(something has gone wrong in the bone marrow)

Secondary

(something else is going on, and even though bone marrow cells are healthy, they’re not able to produce healthy blood cells)

22

What are the 3 main groups of characteristics of inherited marrow failure syndromes?

1. Cancer predisposition
2. Impaired haempoiesis
3. Congenital anomalies

23

What do these inherited marrow syndromes arise due to?

Defects in DNA repair/ribosomes

24

Inherited bone marrow failure conditions are very common

F - they are very rare

25

What is the most common inherited bone marrow failure syndrome?

Faconi's anaemia

26

What does faconis anaemia lead to clinically?

* Short stature – skeletal abnormalities.
* Skin pigment abnormalities – café au lait spots.
* Radial ray abnormalities
* Hypogenitilia
* Endocrinopathies
* GI defects
* Cardiovascular
* Renal
* Haematological

27

What is the mean age of presentation of inherited bone marrow failure syndromes?

7 years old

28

What do these haematological abnormalities of an inherited bone marrow failure syndrome occur due to?

An inability to correct inter-strand cross-links (DNA damage)

So, there patients are unable to correct their own DNA damage

29

In terms of cell deficiencies, what happens in inherited bone marrow failure syndromes?

Macrocytosis, followed by thrombocytopenia, then neutropenia

30

In patients with haematological abnormalities of inherited bone marrow failure syndromes, what are they unable to do?

Correct their own DNA damage