Essential Thrombocytosis

Question 1

What happens in essential thrombocytosis?

Answer 1

High platelets count + Absence of polycyrhemia and marrow fibrosis

Question 2

What are the other secondary causes of thrombocytosis?

Answer 2

Infection
Inflammation
Iron deficiency

Question 3

What are the three mutations involved in ET?

Answer 3

JAK2
MPL
Calreticulin

Question 4

All mutations increase JAK-STAT signaling as the receptor tyrosine kinase becomes independent of ___________ leading to hyperproliferation

Answer 4

thrombopoietin

Question 5

What are the bone marrow changes in ET?

Answer 5

Mild hypercellularity
No overt fibrosis
Megakaryocytes increased
Delicate reticulin fibers

Question 6

What can be the symptoms of ET?

Answer 6

DVT - leg pain and swelling
MI - chest pain and dyspnea
Portal/Hepatic Vein Thrombosis - RUQ pain, ascites, edema in legs, vomiting blood, black tarry stools

Question 7

What does the peripheral blood film shows?

Answer 7

Large platelets

Mild Leukocytosis

Question 8

What will be the CBC report?

Answer 8

High platelet count

Mild leukocytosis

Question 9

What is the age when patients usually present?

Answer 9

Past 60 years

Question 10

When do patients require treatment for ET?

Answer 10

With a platelet count > 1000 × 10^9/L
With symptoms
With other risk factors for thrombosis such as DM or HTN

Question 11

What is the treatment of ET?

Answer 11

Oral hydroxycarbamide or Anagrelide
IV radioactive phosphorus (old age)
Aspirin

Question 12

Name the megakaryocytes maturation inhibitors?

Answer 12

Hydroxycarbamide

Anagrelide