Thrombocytopaenia/thrombocytosis

Question 1

Why would someone with splenomegaly likely have thrombocytopaenia?

Answer 1

storage of platelets in spleen causing reduction in platelets in peripheral circulation

Question 2

Which hormone stimulates production of plts and where is it produced?

Answer 2

TPO= thrombopoetin, produced in liver

Question 3

Someone has an isolated platelet count of 100. Are you worried?

Answer 3

Not necessarily, clinical picture is important. Plt <50 is very concerning, speak to haematology team immediately

Question 4

A patient has a history of epistaxis and fatigue. Their platelet count is normal. Could there still be a platelet issue?

Answer 4

Yes, it could be a qualitative issue e.g. non functioning platelets due to mutations in receptors etc

Question 5

What are the clinical signs of thrombocytopaenia?

Answer 5

skin signs: non-blanching rash (purpura, petechiae)

Mucosal bleeding: epistaxis, gum bleeding, menorrhagia

Question 6

How can your differential diagnosis be divided for thrombocytopaenia?

Answer 6

productive (BM issue) versus consumptive (peripheral destruction)

Question 7

What are examples of underproduction causes of thrombocytopaenia?

Answer 7

B12, folate, drug-induced, aplastic anaemia, fanconi syndrome, sepsis, leukaemia, liver failure (thrombopoietin produced in the liver!!!!)

Question 8

Examples of overdestruction causes of thrombocytopaenia?

Answer 8

DIC, ITP, TTP, haemolytic uraemic syndrome. alcohol, drugs

Question 9

A patient has pancytopaenia. Is the cause of thrombocytopaenia likely to be productive or consumptive in origin?

Answer 9

productive- BM

Question 10

What are causes of DIC?

Answer 10

sepsis, cancer, trauma, OBG

Question 11

Is the risk of clotting or bleeding higher in DIC?

Answer 11

imbalance between both, therefore risk of both

Question 12

What is TTP?

Answer 12

thrombotic thrombocytic purpura, results in blood clots forming throughout the body. This is a medical emergency

Question 13

What is the pathophysiology of TTP?

Answer 13

autoantibody targets ADAMTS-13 which is an enzyme that breaks down clots

Question 14

Which feature would see you on blood film in TTP?

Answer 14

schistocytes due to fragmentation of RBCs through narrowed vessels due to blockage with clots

Question 15

Should you give platelets in TTP?

Answer 15

No!!

Question 16

What is the main difference between in DIC and TTP that will become apparent during your investigations

Answer 16

TTP has normal coag screen (PT/PTT, fibrinogen) while DIC shows increased PT/PTT and decreased fibrinogen. D-dimer is normal in TTP and raised in DIC

Question 17

Which hematological condition can arise from heparin usage?

Answer 17

HIT= heparin induced thrombocytopaenia

Question 18

What is the management of HIT?

Answer 18

switch anticoag therapy

Question 19

Which scoring is available for HIT?

Answer 19

4T’s- medcalc (stratifies risk of patient having HIT)

Question 20

Which principles should you follow before speaking to haematologist?

Answer 20

1. Acute/chronic
2. Evidence of bleeding, pregnant, clot
3. Med hx- alcohol, liver disease
4. DH- heparin, anticoag?
5. Tests: FBC, LFTs, U+E, CRP, coag
6. Blood film results

Question 21

What is the management of DIC?

Answer 21

treat underlying cause

Question 22

What is the treatment of ITP?

Answer 22

steroids, TPO agonists, IV immunoglobulins

Question 23

What is the treatment of TTP?

Answer 23

plasma exchange

Question 24

How can differential for thrombocytosis be divided?

Answer 24

reactive vs BM

Question 25

What are reactive causes of thrombocytosis?

Answer 25

infection, inflammation, malignancy, iron deficiency

Question 26

What is a BM cause of thrombocytosis?

Answer 26

myeloproliferative neoplasm

Question 27

What is the pentad of TTP?

Answer 27

renal failure, neuro symptoms, microangiopathic haemolytic anaemia, thrombocytopenia, fever

Question 28

Two differentials for abnormal or prolonged bleeding?

Answer 28

Thrombocytopenia- ITP, TTP Haemophilia A and B Von Willebrand disease DIC

Question 29

Deficiency in factor VIII is associated with which condition?

Answer 29

Haemophilia A

Question 30

Haemophilia B is associated with a deficiency in which factor?

Answer 30

factor IX

Question 31

Which sex is affected in haemophilia?

Answer 31

men- x-linked recessive disorder

Question 32

Name two features of haemophilia

Answer 32

sponatneous bleeding into joints and muscles abnormal bleeding in gums, GI tract, urinary tract, following procedures...anywhere in the body, spontaneous haemorrhage without trauma

Question 33

How is diagnosis made for von willebrand disease and haemophilia?

Answer 33

family history genetic testing coag factor assays bleeding scores

Question 34

Why is desmopressin given to manage von willebrand disease and haemophilia?

Answer 34

stimulates release of von willebrand factor