Pancytopenia

Question 1

What is pancytopenia?

Answer 1

Deficiency of blood cells of all lineages (generally excludes lymphocytes)

Question 2

Does Pancytopenia always = bone marrow failure?

Answer 2

No

Question 3

What is the general path of neutrophil production?

Answer 3

Promyelocyte
Myelocyte
Blast
Band
Metamyelocyte
Segmented neutrophil

Question 4

What are the 2 branching causes of pancytopenia?

Answer 4

Reduced production

Increased destruction

Question 5

What can cause reduced production?

Answer 5

Bone marrow failure

Inherited or acquired

Question 6

What are the 3 characteristics of inherited marrow failure syndromes?

Answer 6

Cancer predisposition
Impaired haemopoiesis
Congenital anomalies

Question 7

What is an example of inherited marrow failure syndrome?

Answer 7

Fanconi’s anaemia

Question 8

What are the symptoms of Fanconi’s anaemia?

Answer 8

Short stature
Skin pigment
Radial ray 
Hypogenitalia 
Endocrinopathies
GI defects 
Cardiovascular
Renal 
Haematological

Question 9

What is the average age of someone with Fanconi’s Anaemia?

Answer 9

7 Years

Question 10

What is the problem with fanconis anaemia?

Answer 10

Unable to correct inter-strand cross links (DNA damage)

Question 11

What are examples of acquired primary bone marrow failure?

Answer 11

Idiopathic anaplastic anaemia
Myelodysplastic syndromes
Acute leukaemia

Question 12

What happens in anaplastic anaemia?

Answer 12

IFNy and TNFa react to HSC

Question 13

What are 4 types of myelodysplastic syndromes?

Answer 13

Dysplasia
Hypercellular marrow
Increased apoptosis of progenitor and mature cells
Propensity for evolution into AML

Question 14

Why does acute leukaemia cause pancytopenia?

Answer 14

Proliferation of abnormal xcells form leukaemic stem cells
Failure to mature into normal cells
Prevent normal HSC development

Question 15

What can cause secondary bone marrow failure?

Answer 15

Drug induced
B12/Folate deficiency
Infiltrative-non-haemopoietic malignant

Question 16

What can cause increased destruction?

Answer 16

Hypersplenism

Question 17

What is the spleen?

Answer 17

Secondary lymphoid organ

Encapsulated

Question 18

How much does the spleen usually weight?

Answer 18

150–200g

Question 19

What supplies the spleen?

Answer 19

Splenic artery

Question 20

What drains the spleen?

Answer 20

Splenic vein

Question 21

What is included in the parenchyma?

Answer 21

Red pulp and white pulp

Question 22

What does red pulp contain?

Answer 22

Sinusoids and cords

Question 23

What are sinusoids?

Answer 23

Fenestrated
Lined by endothelial cells
Supported by hoops of reticulin

Question 24

What do cords contain?

Answer 24

Macrophages and fibroblasts and cells in transit

Question 25

What is the function of the spleen?

Answer 25

Filter for blood Detects and eliminates unwanted/foreign material Facilitates immune responses to blood borne antigens

Question 26

What happens in hypersplenism?

Answer 26

Increased splenic pool Increased destruction that exceeds bone marrow capacity Enlarged spleen

Question 27

What is the splenic red cell mass of a normal spleen?

Answer 27

5%

Question 28

What is the splenic red cell mass of a hypersplenic spleen?

Answer 28

40%

Question 29

Is red cell transit fast or slow in hypersplenism?

Answer 29

Slow

Question 30

Is the splenic platelet poor higher or lower in hypersplenism?

Answer 30

Higher

Question 31

What can splenomegaly result in?

Answer 31

Hypersplenism

Question 32

What can cause Hypersplenism?

Answer 32

``` Splenic congestion -Portal Hypertension Systemic diseases (RA) Splenic lymphoma ```

Question 33

What are secondary causes of Acquired pancytopenia?

Answer 33

``` Drug induced Vit B12/Folate deficiency Infections Metastatic cancer Storage disorders ```

Question 34

What are the 3 contributing factors to pancytopenia?

Answer 34

Anaemia Neutropenia Thrombocytopenia

Question 35

What are three symptoms of anaemia?

Answer 35

Fatigue SOB CV comprimise

Question 36

What are clinical features of neutropenia?

Answer 36

Infections

Question 37

What are symptoms of thrombocytopenia?

Answer 37

Bleeding Purpura Petechiae Wet bleeds

Question 38

What investigations are done for Pancytopenia?

Answer 38

``` History FBC B12/folate LFTs Virology Autoantibodies Bone marrow ```

Question 39

What is the marrow like in aplastic anaemia?

Answer 39

Hypocellular

Question 40

When is the bone marrow hypercellular?

Answer 40

Myelodysplastic syndromes B12/Folate deficiency Hypersplenism

Question 41

What is the treatment of pancytopenia?

Answer 41

Supportive | Specific (dependant on cause)

Question 42

What is the supportive treatment for pancytopenia?

Answer 42

``` Red cell transfusions Platelet transfusions Neutrophil transfusions Antibiotics prophylaxis/treatment Antibacterial Antifungals ```

Question 43

What is the treatment for congenital bone marrow disorder?

Answer 43

bone marrow transplant

Question 44

What is the treatment for idiopathic anaplastic anaemia?

Answer 44

Immunosuppression

Question 45

What is the treatment for secondary bone marrow disorder?

Answer 45

Drug reaction -STOP Viral - Treat infection Replace B12/folate

Question 46

What is the treatment for hypersplenism?

Answer 46

Treat cause | Consider splenectomy