Pancytopenia

Question 1

define pancytopenia

Answer 1

deficiency of blood cells of ALL lineages

but generally excludes lymphocytes

Question 2

what is important to remember about pancytopenia

Answer 2

NOT a diagnosis

Question 3

life span of mature RBCs, platelets, neutrophils

Answer 3

RBC- 120 days
Neutrophil- 7-8 hours
Platelet- 7-10 days

Question 4

what are the 2 mechanisms through which pancytopenia can happen

Answer 4

reduced production (marrow failure) 
increased destruction (hypersplenism)

Question 5

causes of reduced production

Answer 5

bone marrow failure

• inherited
• acquired (primary- aplastic, secondary drugs)

Question 6

what is the genetic fault in inherited marrow failure syndromes

Answer 6

defects in DNA repair and ribosomes

Question 7

example of an inherited bone marrow failure

Answer 7

fanconi’s anaemia

Question 8

features of fanconi’s anaemia

Answer 8

short stature
cafe au lait spots
hypogenitalia 
GI, cardio, renal defects
aplastic anaemia 
skeletal abnormalities e.g. missing thumbs

Question 9

haematological abnormalities in fanconi’s anaemia

Answer 9

unable to correct DNA damage

macrocytosis followed by thrombocytopenia, then neutropenia

Can progress to bone marrow failure + leukaemia

Question 10

mean age onset of fanconi’s anaemia

Answer 10

7 years old

Question 11

what is a primary acquired bone marrow failure

e.g. aplastic anaemia

Answer 11

an intrinsic marrow problem

usually a stem cell defect

Question 12

causes of primary bone marrow failure

Answer 12

aplastic anaemia
myelodysplastic syndromes
acute leukaemia

Question 13

what is aplastic anaemia

Answer 13

autoimmune attack against haemopoietic stem cells

Question 14

what is seen in myelodysplastic syndromes

Answer 14

dysplasia
hypercellular marrow – marrow producing more cells but they die more prematurely so lots of immature cells
increased apoptosis of progenitor and mature cells (ineffective haemopoiesis)

Question 15

what can myelodysplastic syndrome evolve into

Answer 15

acute myeloid dysplasia

Question 16

causes of secondary bone marrow failure

Answer 16

drug induced – chemo, chloramphenicol, alcohol
B12/folate deficiency– hypercellular
Lymphoma
HIV

Question 17

causes of increased RBC destruction

Answer 17

hypersplenism

Question 18

what is hypersplenism

Answer 18

increased destruction that exceeds bone marrow capacity generate new cells

Question 19

what leads to hypersplenism

Answer 19

splenomegaly

Question 20

causes of splenomegaly

Answer 20

splenic congestion
- portal hypertension, HF, portal/splenic vein occlusion

systemic disease
- RA

haematological diseases
- splenic lymphoma, haemolytic anaemia,

Question 21

blood findings in pancytopenia

Answer 21

anaemia, neutropenia, thrombocytopenia

Question 22

what is seen in neutropenia

Answer 22

repeated infections

Question 23

what is seen in thrombocytopenia

Answer 23

bleeding

• purpura
• petechia

Question 24

tests to find the cause of pancytopenia

Answer 24

History + family history 
clinical findings
FBC
B12/Folate/LFTs/virology depending on history 
bone marrow examination

Question 25

specialised test for fanconi's anaemia

Answer 25

chromosome fragility

Question 26

what marrow cellularity is seen in aplastic anaemia

Answer 26

hypocellular - marrow failure therefore no cells being produced

Question 27

when is hyper cellular marrow seen

Answer 27

myelodysplastic syndromes B12/folate deficiency hypersplenism bone marrow packed full of immature cells

Question 28

types of treatment for pancytopenia

Answer 28

supportive | specific

Question 29

supportive treatment

Answer 29

red cell transfusion platelet transfusion antibiotic treatment + prophylactic use

Question 30

specific treatment for primary bone marrow failure

Answer 30

malignancy - chemotherapy congenital - bone marrow transplantation aplastic anaemia - immunosuppression

Question 31

specific treatment for secondary bone marrow failure

Answer 31

drug reaction - stop the drug viral - treat cause replace B12/Folate if deficient splenectomy

Question 32

what causes hyper cellular bone marrow

Answer 32

B12/Folate deficiency myeloproliferative disorders hypersplenism

Question 33

infections that can cause pancytopenia

Answer 33

HIV EBV Paravirus Hepatitis

Question 34

drugs that can cause pancytopenia

Answer 34

cytotoxic: azathioprine, methotrexate antibiotics: chloramphenicol diuretics: thiazides hyperthyroid: carbimazole anti-pychotics: clozapine anti-epileptic: phenytoin