Myeloproliferative disorder

Question 1

What is Myeloproliferative disorder?

Answer 1

MPDs are blood cancers caused by changes in the stem cells inside bone marrow, the tissue that makes blood cells. These changes cause the body to make too many blood cells. This excess can be any type of blood cell — white, red, or platelets.
Examples:
CML, polycythaemia vera, essential thrombocytosis, primary myelofibrosis

Question 2

What is Essential Thrombocytosis/ thrombocythemia?

Answer 2

• Essential Thrombocytosis (aka Primary Thrombocythemia) → chronic myeloproliferative disorder associated with sustained dysregulated megakaryocyte proliferation in the bone marrow, increasing the number of circulating platelets
• Average age at diagnosis is 50-70 years old
• JAK2 mutation is found in around 50% of patients

Question 3

What is Myelofibrosis?

Answer 3

MYELOFIBROSIS → progressive bone marrow fibrosis (causes bone marrow failure) MPDT:
- Massive splenomegaly
- Pancytopenia
- Dry Tap ⇒ failure of bone marrow aspirate
- Tear Drop poikilocytoses (dacrocytes) on blood film → flat, elongated RBC’s

Question 4

What are the presenting symptoms of myeloproliferative disorder?

Answer 4

“BASE”
- Bleeding
- Arterial and Venous Thrombosis
- Splenomegaly
- Erythromelalgia → burning pain and dusky congestion of the extremities. Pain increases with heat and improves with cold.

Question 5

What investigations are used to diagnose/ monitor myeloproliferative disorder?

Answer 5

1. FBC → platelet count ≥450 x 10⁹/L
2. JAK2 Mutation Testing

Question 6

How is myeloproliferative disorder managed?

Answer 6

1. Hydroxycarbamide → used to reduce platelet count
2. Antiplatelet Therapy → Aspirin (reduce thrombotic risk)

Question 7

What complications may arise from myeloproliferative?

Answer 7

Thrombosis, bleeding, abortion, intrauterine death