Pancytopenia

Question 1

what is the definition of pancytopenia

Answer 1

a deficiency of blood cells of all lineage

BUT generally excludes lymphocytes

Question 2

what is the life span for RBC, neutrophils and platelets

Answer 2

RBC - 120 days

Neutrophil - 7-8 hours

Platelets - 7-10 days

Question 3

what are the two mechanisms through which pancytopenia can happen

Answer 3

reduced production

increased destruction

Question 4

what are cause of reduced production

Answer 4

bone marrow failure

• inherited
• acquire = primary or secondary

Question 5

what causes inherited marrow failure syndromes

Answer 5

defects in DNA repair and ribosomes

Question 6

what is an example of an inherited bone marrow failure

Answer 6

Fanconi’s anaemia

Question 7

what are features of Fanconi’s anaemia

Answer 7

Short stature
Skin pigment abnormalities e.g. cafe au lait spots
Hypogenitilia
Endocrinopathies
Haematological abnormalities (aplastic anaemia)
Skeletal abnormalities (can be missing their thumbs)

Question 8

what are the haematological abnormalities in Fanconi’s anaemia

Answer 8

Unable to correct DNA damage

Macrocytosis followed by thrombocytopenia, then neutropenia

Can progress to bone marrow failure and leukaemia

Question 9

at what age do the haematological abnormalities in FA present

Answer 9

mean onset 7 years old

Question 10

what is meant by primary bone marrow failure

Answer 10

no obvious cause

- usually stem cell defect

Question 11

what are causes of primary bone marrow failure

Answer 11

Aplastic anaemia

Myelodysplastic syndromes (MDS)

Acute leukaemia

Question 12

what is aplastic anaemia

Answer 12

autoimmune attack against haemopoietic stem cells

Question 13

what is seen in MDS

Answer 13

Dysplasia

Hypercellular marrow (marrow producing more cells but they die more prematurely)

Increased apopotosis of progenitor and mature cells (inefffective haemopoiesis)

Question 14

what can MDS evolve into

Answer 14

Acute myeloid leukaemia

Question 15

what are causes of secondary bone marrow failure

Answer 15

drug induced e.g. chemo,
- causes aplasia

B12/folate deficiency
- hypercellular

Lymphoma

HIV

Question 16

what are causes of increased destruction of RBC

Answer 16

hypersplenism

Question 17

what leads to hypersplenisms

Answer 17

splenomegaly

Question 18

what are causes of hypersplenism

Answer 18

Portal Hypertension
Congestive cardiac failure
RA
Splenic lymphoma

Question 19

what are the blood findings in pancytopenia

Answer 19

anaemia
neutropenia
thrombocytopenia

Question 20

what is seen in neutropenia

Answer 20

repeated infections

Question 21

what is seen in thrombocytopenia

Answer 21

bleeding

• purpura
• petechia

Question 22

what specialised test is done for Fanconi’s syndrome

Answer 22

chromosome fragility

Question 23

what cellularity is seen in aplastic anaemia

Answer 23

hypocellular

Question 24

when is the bone marrow hyper cellular

Answer 24

Myelodysplastic syndromes
B12/folate deficiency
Hypersplenism

Question 25

what is Tx for pancytopenia (supportive)

Answer 25

supportive

• red cell transfusion
• platelet transfusion
• Antibiotics treatment and prophylactic use

Question 26

what is Tx for pancytopenia (specific) - treating the cause for primary bone marrow failure

Answer 26

Malignancy – consider chemotherapy

Congenital – consider bone marrow transplantation

Idiopathic Aplastic Anaemia – Immunosuppression

Question 27

what is Tx for pancytopenia (specific) - treating the cause for secondary bone marrow failure

Answer 27

Drug reaction – STOP

Viral – eg treat HIV

Replace B12/folate

Splenectomy