proliferation of cells

Question 1

myeloproliferative disorder also called ….

Answer 1

myeloproliferative neoplasms

Question 2

what are myeloproliferative neoplasms?

Answer 2

chronic neoplasms derived from a mutated multipotent haemopoietic progenitor cell or pluripotent myeloid stem cell.

Question 3

what are the four important myeloproliferative disorders?

Answer 3

1. chronic myeloid leukaemia (CML)
2. polycythaemia vera (PV)
3. essential thrombocytosis (ET)
4. primary myelofibrosis (PMF)

2, 3 and 4 have the same molecular abnormalities and tend to transform into AML

Question 4

what is polycythaemia?

Answer 4

• increased RBC count
• increase haemoglobin
• increased Hct

Question 5

types of polycythaemia

Answer 5

true polycythaemia
* primary polycythaemia
* actual increase in the production of red blood cells by the bone marrow
* physiological response to hypoxia
* intrinsic bone marrow disorder

pseudopolcythaemia
* not caused by a primary problem in the bone marrow
* acute ( dehydration, burns )
* chronic ( smoking, diuretics )

Question 6

thrombocytosis

Answer 6

increased platelet count

Question 7

types of thrombocytosis

Answer 7

essential thrombocytosis - primary
* bone marrow makes too many platelets
* results from mutation in multipotent haemopoietic progenitor cell

reactive thrombocytosis - secondary
* platelets increase in response to something else
* e.g. infection, inflammation, haemorrhage, IDA

Question 8

clinical features of thrombocytosis

Answer 8

• vascular insufficiency
• thrombus
• pardoxical bleeding due to high platelets casing fall in VWF
• platelet size increase
• may be increase in neutrophils
• RBC, Hb, Hct - normal
• bone marrow extra megakaryocytes that are larger

Question 9

management of thrombocytosis

Answer 9

if under 60 yr, moderate increased numbers, no previous clots then :
* daily aspirin to prevent clots
* stop smoking

for all others :
* stop smoking
* hydroxycarbamide
* daily aspirin to prevent clots

Question 10

primary myelofibrosis

Answer 10

• caused by increased amounts of collagen in marrow
• collagen produces in response to cytokines secreted by neoplastic myeloid cells

Question 11

primary myelofibrosis diagnosis

Answer 11

• hepatosplenomegaly
• fatigue
• weight loss
• fever
• increased sweating
• in late stages - portal hypertension; gout