302 2. Haematology (the case of the woman who wouldn't stop bleeding)

Question 1

What is acute promyelocytic leukaemia?

Answer 1

Malignant proliferation of promyelocytes

It’s very acute and needs to be treated rapidly. It’s very rare

It’s a type of acute myeloid leukaemia (AML)

Question 2

What are promyelocytes?

Answer 2

A type of cell that develops into a granulocyte like Eosinophils, neutrophils, and basophils

Question 3

What is ARTA treatment?

Answer 3

All trans retinoic acid (acid form of vitamin A)
-Used to induce terminal differentiations of promyelocytes by binding to retinoic acid receptor alpha (RARA)

Its used to treat acute promyelocytic leukaemia

Question 4

What is ARTA syndrome/differentiation syndrome?

Answer 4

Occurs in 15% of patient given ARTA treatment

Causes dyspnoea, fever, weight gain, oedema

Treated by stopping ARTA and dexamethasone

Question 5

How does arsenic trioxide treat acute promyelocytic leukaemia?

Answer 5

It induces apoptosis of promyelocytes

More effective than chemotherapy but causes QTc prolongation so requires monitoring

It’s the standard treatment in low-risk disease

Question 6

What are the clinical presentations of acute promyelocytic leukaemia?

Answer 6

Fevers
fatigue
loss of appetite
frequent infections
Bleeding: retinal haemorrhages, nose bleeds, menorrhagia, gum bleeding

Question 7

How is APML diagnosed?

Answer 7

Genetic testing like FISH

Question 8

Why is APML complicated by DIC?

Answer 8

It can appear like the patient has DIC when they don’t