Haematology

Question 1

What immune thrombocytopenia?

Answer 1

Anti-platelet ab that cause destruction of platelets that causes a isolated thrombocytopenia

Question 2

What are the basic mechanisms that can cause reduced platelets?

Answer 2

Increased platelet destruction/Sequestration (majority)

Reduced production (minority)

Question 3

What causes/triggers ITP?

Answer 3

Medications - Classically Quinine

Viral infection - EBV, HIV, CMV

Lymphoproliferative disorders (B cell eg NHLymphoma or CLL)

Connective tissue disorders eg Lupus

Idiopathic (the majority)

Question 4

How do you Ix ITP?

Answer 4

FBE and blood film

Viral screening

Screen for lymphoma

Bone marrow if older patient, other symptoms eg lymphadenopathy

Question 5

How is ITP Mx?

Answer 5

Remove trigger if possible

1mg/kg daily oral Pred

IVIg

Anti-CD20 (Rituximab)

Splenectomy

Thrombopoietin receptor agonist - Increase platelet production

Question 6

What must you consider when using steroids for ITP?

Answer 6

It can take up to a week for it to work

Consider IVIg if not working fast enough for the patient

Question 7

What is the role of platelet transfusion in ITP?

Answer 7

Only lasts 5 minutes therefore only used in very particular circumstances eg splenectomy surgery

Question 8

Who gets ITP?

Answer 8

Children - Typically virally induced

Adults - Typically drug induced or idiopathic

Question 9

How does Mx change in children and adults?

Answer 9

Chilren Observe as usually viral cause

Adults - treat

Question 10

What is Thrombotic thrombocytopenic purpura?

Answer 10

A microangiopathic haemolytic anaemia

Question 11

What are the MAHAs

Answer 11

TTP

HUS

Haemolytic anaemia and elevated liver enzymes

Ecclampsia

Question 12

What is the pentad of TTP?

Answer 12

Thrombocytopenia

MAHA - ie schistocytosis on blood film

CNS abnormalities

Renal failure

Fever

Question 13

What are some triggers of TTP

Answer 13

Idiopathic

Pregnancy

E coli

Malignancy

Shigellosis

Question 14

What is the pathophysiology of TTP?

Answer 14

ADAMTS 13 (that usually cleaves VWF to stop coagulations) is either deficient or opsonised by an ab to cause over excess platelet aggregation and thrombus formation in different tissues of the body

Thrombocytopenia is due to platelets comsumption

Question 15

How is TTP treated?

Answer 15

Aggressive plasma exchange against FFP with replacement of ADAMTS 13

Aspirin and dipyrimadole

Splenectomy

Steroids

Question 16

What is the role of LDH in TTP?

Answer 16

Marker of the success of plasma exchange (as it is a marker of haemolysis)

Question 17

Why do you get haemolysis in TTP?

Answer 17

Abnormal VWF binds to GAF proteins on RBC surface + upregulation of complement

Question 18

What is myelodysplasia

Answer 18

Inefficiency disorders of the blood marrow - less effective that manifest with anaemia, neutropenia, and thrombocytopenia. Also the cells that are produced are less effective

Question 19

Who gets myelodysplasia?

Answer 19

Older people - Quite common - suspect if persistent neutropenia or thrombocytopenia

Question 20

What do you find in film in myelodysplasia?

Answer 20

Abnormal morphology of neutrophils - reduced lobulation and granules

Question 21

How is the diagnosis of myelodysplasia made?

Answer 21

On bone marrow biopsy

and karyotyping

Question 22

What are potential complications of myelodysplasia?

Answer 22

Transformation into leukaemia

Question 23

How is the prognosis determined in myelodysplasia?

Answer 23

The blast progression

Question 24

How is myelodysplasia treated?

Answer 24

Supportive

Bone marrow transplant