Myeloproliferative Disorders

Question 1

What are myeloproliferative disorders?

Answer 1

Diseases of the bone marrow and blood

Question 2

What is chronic myeloid leukaemia?

Answer 2

Too many myeloid cells

Philadelphia chromosome positive

Question 3

What is Polycythaemia Rubra Vera/Polycythaemia

Vera (PV)? And some signs?

Answer 3

Too many RBCs. JAK2 mutation common

Tendency to thrombosis and bleeding, plethora, acquagenic pruritis

Question 4

What is Essential Thrombocythaemia (ET)?

Answer 4

Too many platelets. Half have JAK2 mutation.

Mostly asymptomatic at diagnosis.

Question 5

What is Myelofibrosis (MF)?

Answer 5

Excessive fibrosis in the marrow secondary to a probable ET/PRV. Half have JAK2 mutation

Asymptomatic, spleen pain, fevers, sweats, back
pain, infections. Cytopenias.

Poor prognosis.

Tear drop cells

Question 6

What is the Philadelphia

chromosome?

Answer 6

Bcr-Abl protein

Chromosome 9 and 22

Particularly related to chronic myeloid leukaemia

Question 7

What is myelodysplasia?

Answer 7

Cancers in which immature blood cells in the bone marrow do not mature and therefore do not become healthy blood cells.

Early on, typically asymptomatic. Later symptoms may include feeling tired, shortness of breath, easy bleeding, or frequent infections.

1 or more of anaemia, neutropenia, and thrombocytopenia

Question 8

What is faconi’s anaemia?

Answer 8

A rare disease familial disorder that mainly affects the bone marrow.

It results in decreased production of all types of blood cells.

The most common inherited form of aplastic anemia.