Haem - Myeloproliferative Disorders Flashcards
What are myeloproliferative disorders?
Uncontrolled proliferation of a single type of stem cell
Develop and progress slowly
Potential to transform into acute myeloid leukaemia
What are the 3 major myeloproliferative disorders?
Primary myelofibrosis
Polycythaemia vera
Essential thrombocythaemia
Complete the table
What genes are myeloproliferative diseases associated with?
JAK2
MPL
CALR
What is myelofibrosis?
Proliferation of a single cell line leads to bone marrow fibrosis
Bone marrow replaced by scar tissue in response to cytokines released from proliferating cells
What particular cytokine is released in myelofibrosis?
Fibroblast growth factor
What does bone marrow fibrosis lead to?
Affected production of :
- Blood cells
- White blood cells
- Platelets
What happens to haematopoiesis in myelofibrosis?
Extramedullary haematopoiesis
Production of blood cells in the liver and spleen
This leads to hepatomegaly, splenomegaly and portal hypertension
May occur around spine leading to compression
What can myelofibrosis result from?
Primary myelofibrosis
Polycythaemia vera
Essential thrombocythaemia
What can a blood film in myelofibrosis show?
Teardrop-shaped red blood cells
Anisocytosis (varying sizes of RBCs)
Blasts (immature red and white cells)
How do myeloproliferative disorders present?
Non-specific symptoms
- Fatigue
- Weight loss
- Night sweats
- Fever
May be signs and symptoms of underlying complications
- Anaemia
- Splenomegaly
- Portal hypertension
- Low platelets
- Raised Hb (itching, headaches and red face)
- Low white blood cells
- Gout
What is a common complication of polycythaemia and thrombocythaemia?
Thrombosis
Leading to MI, stroke or VTE
What are the clinical signs of polycythaemia?
Ruddy complexion (red face)
Conjunctival plethora
Splenomegaly
Hypertension
How are myeloproliferative disorders diagnosed?
Bone marrow biopsy required to confirm diagnosis
Aspiration can be dry with myelofibrosis as bone marrow turned to scar tissue
What can be done in addition to bone marrow biopsy for diagnosis of myeloproliferative conditions?
Genetic testing for :
JAK2
MPL
CALR
