Myeloproliferative disorders Flashcards
Uncontrolled […] of a single type of […] cell
uncontrolled proliferation of a single type of stem cell
3 types
Primary […]
[…] […]
Essential […]
Primary myelofibrosis
Polycythaemia vera
Essential thrombocythaemia
Primary myelofibrosis is the result of proliferation of the […] […] […]
hematopoietic stem cells.
Polycythaemia vera is the result of proliferation of the […] cell line
erythroid
Essential thrombocythaemia is the result of proliferation of the […] cell line
megakaryocytic
Which gene is associated with an increased risk of myeloproliferative disorders progressing into acute myeloid leukaemia
JAK2
Myelofibrosis
Fibrosis of bone marrow –> replaced by scar tissue
When the bone marrow is replaced with scar tissue the production of blood cells ([…]) starts to happen in other areas such as the […] and […]. This is known as […] […] and can lead to […] and […].
This can lead to portal hypertension.
If it occurs around the spine it can lead to spinal cord compression.
haematopoiesis
liver, spleen
extramedullary haematopoiesis
hepatomegaly
splenomegaly
Polycythaemia vera features
- […] plethora (excessive redness to the conjunctiva in the eyes)
- A “[…]” complexion
- […]
- Raised […]
- Low EPO
Conjunctival
ruddy
Splenomegaly
Hb
Diagnosis
1. Bone marrow aspiration is usually “[…]” as the bone marrow has turned to scar tissue.
Testing for the […], MPL and CALR genes can help guide management.
dry
JAK2
Management of Primary Myelofibrosis
- Allogenic stem cell transplant
- Chemo
- RADIO
Supportive management of the anaemia, splenomegaly and portal hypertension.
Management of Polycythaemia Vera
Venesection + aspirin
Management of Essential Thrombocythaemia
Aspirin + Chemo
