Myeloproliferative Disorders

Question 1

Essential ?
PCV = ? ? ?
Primary ?

Clones of hematopoietic stem cells proliferate in the ?, yet retain the ability to ?.

Answer 1

thrombocytosis
polycythemia rubra vera
myelofibrosis

marrow
differentiate

Question 2

Essential Thrombocytosis

Clonal proliferation of megakaryocytes leading to persistently ? ?, which is often asymptomatic.
The platelets have abnormal function, thus the most common presentation is with ? ?.
Other symptoms may be related to ? or arterial / venous ?

Answer 2

raised platelets
microvascular occlusion
bleeding
thrombosis

Question 3

Polycythaemia rubra vera (PCV);

Malignant proliferation of a clone derived from one pluripotent marrow cell,
with the erythroid progenitor offspring not requiring EPO to avoid apoptosis.
Excess production of ?, ? and ? lead to serum ? and ? complications.

Answer 3

rbc, wbc, platelets
hyperviscosity
thrombotic

Question 4

PCV

It is diagnosed by increased red cell ?, or investigation for a ? mutation .
It is often asymptomatic, or presents in patients >? with arterial or venous
?.

Answer 4

mass
JAK2
60
thrombosis

Question 5

PCV

Other rarer presentations;

Vague hyperviscosity symptoms: ?ache, dizziness, ?, ? plethora, erythromelalgia (? sensation in the fingers/ toes).
?.
?: due to increased cell turnover.

Answer 5

head
tinnitus
facial
burning
splenomegaly
gout

Question 6

PCV

Treatment;
Repeated ? and low dose ?.

? and ? disease are the key differentials to rule out in primary PCV, but in
these secondary polycythaemia syndromes only the ? ? ? is raised.

Answer 6

venesection
aspirin
hypoxia
renal
red cell count

Question 7

Primary myelofibrosis;

Hyperplasia of megakaryocytes, which produce excess platelet-derived
? ?, leading to marrow fibrosis and ?.
There is secondary ? in the liver/ spleen, leading to ? hepatosplenomegaly (most common presentation) .

Answer 7

growth factor
metaplasia
haematopoiesis
massive

Question 8

Primary myelofibrosis;

Symptoms are ? symptoms, ? discomfort and of bone marrow ?
Essential thrombocytosis and PCV both may progress to ? or ?, yet the risk of this is relatively rare.

Answer 8

B
abdo
failure
AML
myelofibrosis