Myeloproliferative Disorders Flashcards
1
Q
- What are myeloproliferative disorders?
2. Name the 4 myeloproliferative disorders
A
- clonal stem cell dusorders characterised by uncontrolled proliferation of one or more of the myeloid cell lines within the bone marrow
- polycythaemia vera
essential thrombocythaemia
myelofibrosis
CML
2
Q
What can myeloproliferative disorders transform into (2)
A
- can transition from one disorder to another
2. AML
3
Q
Define the following:
- -cytosis
- -cythaemia
- -penia
- leukocytosis
- leukopenia
- neutropenia
- lymphocytosis
- thrombocytosis
- thrombocythaemia
- thrombocytopenia
- polycythaemia
- pancytopenia
A
- benign increase in a cell line
- malignant increase in a cell line
- decrease in a cell line
- benign or malignant increase in leucocytes
- reduction in total WCC
- reduction in neutrophils
- benign or malignant increase in lymphocytes
- benign increase in platelets
- malignant increase in platelets
- increase in red cells or all 2 myeloid cell lineages
- reduction in all 3 myeloid cell lines (associated with bone marrow failure)
4
Q
- What is polycythaemia?
- Which mutations are associated with primary polycythaemia?
- What is secondary polycythaemia caused by?
A
- increase in Hb, packed cell volume and red cell count
- Jak2
- hypoxic increase in EPO (associated with hypoxic states) or inappropriate increase in EPO (paraneoplastic syndromes)
5
Q
Describe clinical features of polycythaemia
A
- tiredness
- itching
- vertigo
- headache
- visual disturbance
- gout
- haemorrhage or thrombpsis
- hepatosplenomegaly
6
Q
How is polycythaemia managed?
A
- venesection
- chemotherapy
- low dose aspirin
- anagrelide
7
Q
- What is essential thrombocytopenia?
- How do patients typically present?
- Which mutations are associated with thrombocytopenia?
- How is it manahed?
A
- sustained platelet count ≥450 in the absence of a possible reactive cause for thrombocytosis
- thromboembolic event or incidental diagnosis
- JAK2 or CALR
- hydroxycarbamide (antimetabolite)
anagrelide (inhibits megakaryocytic differentiation)
busulfan (alkylating agent)
8
Q
What is myelofibrosis?
A
chronic myeloproliferative neoplasm involving clonal proliferation of stem cells and abnormal myeloid cells
hyperplasia of abnormal megakaryocytes causes them to produce fibroblast stimulating factors, resulting in fibrosis of the bone marrow
9
Q
Describe clinical features of myelofibrosis
A
- lethargy
- weakness
- weight loss
- fullness in the upper abdomen due to splenomegaly
- bone pain
- gout
- bruising and bleeding
10
Q
- How is myelofibrosis managed?
2. Describe the prognosis of myelofibrosis
A
- supportive measures - transfusion, analgesia, allopurinol
allogenic stem cell transplantation
JAK inhibitors - death may occur due to transformation to AML; CV disease and infection
