Haem - useful passmed facts

Question 1

Management of Hereditary angioedema?

Answer 1

IV C1-inhibitor concentrate

Question 2

Sequestration crisis vs aplastic crisis - reticulocytes?

Answer 2

Aplastic crisis has reduced reticulocytes

Whereas sequestration crisis has increased reticulocytes

sequestration crisis is when blood pools in organs due to sickling occurring, and this can cause worsening of anaemia due to loss of blood in the vasculature, as well as an increase in reticulocytes as the body tries to compensate for the loss in blood. There is no occlusion of any vessels, and therefore this is different from a vaso-occlusive crisis. Therefore, due to the blood results, this is the most likely diagnosis in this patient.

An aplastic crisis is when there is bone marrow suppression due to parvovirus infection. There would be a rise in white blood cells, as well as a decrease in reticulocytes as red blood cell production is lessened. Therefore, since this doesn’t match with the laboratory picture, it is not the correct answer.

Question 3

What is the typical thing you would see on a blood film for IDA?

Answer 3

• target cells
• ‘pencil’ poikilocytes

Question 4

What is the typical thing you would see on a blood film for Megaloblastic anaemia?

Answer 4

hypersegmented neutrophils

Question 5

What is the Haemoglobin transfusion threshold in patients with and without ACS

Answer 5

Without ACS - 70g/L

With ACS - 80g/L

These thresholds should not be used in patients with ongoing major haemorrhage or patients who require regular blood transfusions for chronic anaemia

Question 6

When would you refer patients aged 0-24 years for immediate specialist assessment?

Answer 6

Children and young people (0-24yrs): Refer for immediate specialist assessment for leukaemia if: unexplained petechiae or hepatosplenomegaly

Question 7

What condition would you see ‘rouleaux formation’ on the blood film?

Answer 7

Myeloma

It is the stacking of red blood cells

Question 8

Howell-Jolly Bodies and Target Cells associated with what underlying pathology?

Answer 8

Hyposplenism

Associated with Coeliac Disease

Question 9

What do you see on blood film for myelofibrosis?

Answer 9

‘Tear drop’ poikilocytes

Question 10

What is an antidote for apixaban and rivaroxaban?

Answer 10

Andexanet alfa is a recombinant modified Factor Xa protein used as an antidote of apixaban and rivaroxaban

Question 11

Causes of neutropaenia?

Answer 11

• viral
  
  • HIV
  • Epstein-Barr virus
  • hepatitis
• drugs
  
  • cytotoxics
  • carbimazole
  • clozapine
• benign ethnic neutropaenia
  
  • common in people of black African and Afro-Caribbean ethnicity
  • requires no treatment
• haematological malignancy
  
  • myelodysplastic malignancies
  • aplastic anemia
• rheumatological conditions
• SLE: mechanisms include circulating antineutrophil antibodies
• rheumatoid arthritis: e.g. hypersplenism as in Felty’s syndrome
• severe sepsis
• haemodialysis

Question 12

How can you differentiate between ITP and vWD?

Answer 12

In ITP, there is autoimmune destruction of platelets - so only the platelet count drops (PT and APTT stay the same)

In VWD, platelets are working fine, they just can’t bind to the site of endothelial damage

So in VWD, bleeding time and ATT are prolonged as it takes longer to form a plug to stop the bleeding, but there is a normal platelet level

Question 13

In Transfusion Associated Circulatory Overload (TACO), and Transfusion Related Acute Lung Injury (TRALI), what ar ethe signs that you get

Answer 13

Transfusion Associated Circularory Overload: Hypertension, raised jugular venous pulse, afebrile, S3 present

Transfusion Related Acute Lung Injury: Hypotension, pyrexia, normal/unchanged JVP

Opposite of one another

Question 14

Summary of ITP, TTP, HUS, HSP, HHT

Answer 14

Idiopathic thrombocytopaenic purpura (ITP) - Isolated thrombocytopaenia in a relatively well person

Thrombotoic thrombocytopaenic purpura (TTP) - Thrombocytopaenia in a very unwell person

Haemolytic uraemic syndrome (HUS) - Thrombocytopaenia, schistocytes, renal failure, post-dysentry

Henoch Schonlein Purpura (HSP) - Abdo pain, joint pain, haematuria, purpura. Kids

Haemorrhagic Haemolytic Telangiectasis (HHT) - Epistaxis, GI bleeds, telangiectasia

Question 15

What blood group is the universal blood donor, and what blood group is the universal plasma donor?

Answer 15

Group O is a universal blood donor and group AB is a universal plasma donor

Question 16

What is myelofibrosis?

Answer 16

Myelofibrosis is a myeloproliferative neoplasm resulting in fibrosis of the bone marrow, which leads to a lack of production of normal blood cells