Pancytopenia and bone marrow failure

Question 1

What is responsible for haematopoiesis?

Answer 1

Bone marrow

Question 2

Describe how some anaemias such as thalassaemia result in Organomegaly

Answer 2

Increased demand induces haematopoiesis outside of the bone marrow (extramedullary) in the liver and spleen

Question 3

What is pancytopenia?

Answer 3

Reduction in all the major cell lines: red cells, white cells, platelets

Question 4

What causes pancytopenia

Answer 4

Decreased marrow production - aplastic anaemia, infiltration, megaloblastic anaemia and myelofibrosis

Increased peripheral destruction - hypersplenism

Question 5

What is agranulocytosis

Answer 5

Impaired production of granulocytes (WBC with neutrophil, basophil and eosinophil granules)

Question 6

What is the major risk of agranulocytosis

Answer 6

Increased infection susceptibility

Question 7

What drugs can cause agranulocytosis

Answer 7

Sulphonamides such as dapsone
Gold 
Clozapine
Carbimazole 
Procainamide

Question 8

What must you tell patients before starting drugs known to cause agranulocytosis?

Answer 8

Warn them to report any fevers

Question 9

What do you do to manage agranulocytosis

Answer 9

Stop causative drug
Commence neutropenic regimen
Consider G-CSF if indicated

Question 10

Considering cell survival, what are the earliest signs of bone marrow failure

Answer 10

Neutropenia and Thrombocytopenia

Question 11

How much should IU of red cells raise the Hb by?

Answer 11

10-15g/L

Question 12

What might happen to the platelets when a transfusion is given?

Answer 12

Platelets will drop

Question 13

When should platelets be given when giving a red cell transfusion?

Answer 13

Before or after

Question 14

What symptoms occur when platelets are below 50X10^9/L

Answer 14

Traumatic bleeds, purpura, easy bruising

Question 15

What symptoms occur when platelets are below 20X10^9/L

Answer 15

Spontaneous bleeding - rarely intracranial haemorrhage

Question 16

At what temperature are platelets stored at?

Answer 16

22 degrees - room temp

Question 17

When might platelets need to be irradiated?

Answer 17

In marrow transplant/severely immunosuppressed - to prevent transfusion associated GVHD

Question 18

When are platelets indicated

Answer 18

Platelets <10X10^9/L
Haemorrhage
Before invasive procedures - biopsy, lumbar puncture - increase count >50X10^9/L - 4U may be needed but check with lab

Question 19

What is the management for neutrophils <0.5X10^9/L

Answer 19

Neutropenic regimen

Question 20

What must platelets be compatible with

Answer 20

ABO

Question 21

Describe a bone marrow biopsy

Answer 21

Gives diagnostic information when there are abnormalities on peripheral blood
Important in staging
Take and aspirate and trephine from posterior iliac crest.
Aspirate provides a film
Trephine is a core of bone with allows assessment of cellularity, bone marrow architecture and the presence of infiltrative disease

Question 22

What must be corrected prior to bone marrow biopsy?

Answer 22

Coagulation

Question 23

What must be done after bone marrow biopsy

Answer 23

Apply pressure, lay on that side for 1-2 hrs if platelets are low

Question 24

What is aplastic anaemia

Answer 24

Rare stem cell disorder where the bone marrow stops making cells - results in a pancytopenia

Question 25

Give the classic presentation of aplastic anaemia and blood results

Answer 25

Decreased WCC - infection
Decreased platelets - bleeding
Decreased RBC - anaemia

Question 26

What is the diagnostic test of aplastic anaemia

Answer 26

Bone marrow biopsy

Question 27

How is aplastic anaemia treated?

Answer 27

Supportive in asymptomatic patients
Initiate neutropenic regimen if neutrophils <0.5X10^9/L
Young people with severe disease - allogenic bone marrow transplantation with a HLA-matched sibling
Immunosuppression with ciclosporin and antithymocyte globulin
No clear role for G-CSF

Question 28

What causes aplastic anaemia

Answer 28

Autoimmune
Drugs
Infection - hepatitis and parvovirus
Irradiation 
Inherited - fanconi anaemia