Myeloproliferative Disorders Flashcards
What are myeloproliferative disorders?
Uncontrolled proliferation of a single type of stem cell
Considered a form of cancer occurring in the bone marrow, developing and progressing slowly.
What potential transformation can myeloproliferative disorders undergo?
Acute myeloid leukaemia
Name the main types of myeloproliferative disorders.
- Primary myelofibrosis
- Polycythaemia vera
- Essential thrombocytopenia
What are the blood results associated with primary myelofibrosis?
- Low haemoglobin
- High or low WCC
- High or low platelets
What are the blood results associated with polycythaemia vera?
- High haemoglobin
What are the blood results associated with essential thrombocytopenia?
- High platelets
What is a common causative mutation in myeloproliferative disorders?
JAK2
What does myelofibrosis lead to in the bone marrow?
Bone marrow fibrosis
What are the results of bone marrow fibrosis?
- Anaemia (low haemoglobin)
- Leukopenia (low white blood cells)
- Thrombocytopenia (low platelets)
What is extramedullary hematopoiesis?
Production of blood cells in areas outside the bone marrow due to fibrosis
What are the potential effects of extramedullary hematopoiesis in the liver and spleen?
- Hepatomegaly
- Splenomegaly
- Portal hypertension
What are the blood film characteristics in myelofibrosis?
- Teardrop-shaped red blood cells
- Anisocytosis
- Blasts (immature red and white cells)
List some non-specific symptoms of myeloproliferative disorders.
- Fatigue
- Weight loss
- Night sweats
- Fever
What are signs of complications in myeloproliferative disorders?
- Anaemia (tired, SOB, dizzy)
- Splenomegaly (abdo pain)
- Portal hypertension (ascites, varices, abdo pain)
- Low platelets (bleeding and petechiae)
- Raised haemoglobin (itching, headaches, red face)
- Low white cells (infection)
- Gout (complication of polycythaemia)
What are some features of polycythaemia?
- Ruddy complexion (red face)
- Conjunctival plethora
- Splenomegaly
- Hepatomegaly
- Complication → thrombosis
What is the primary method for diagnosing myeloproliferative disorders?
Bone marrow biopsy
What might the aspirate from a bone marrow biopsy show in myelofibrosis?
Dry aspirate due to scar tissue
What testing is done for diagnosing JAK2 mutations?
Testing for JAK2 genes
What are the management strategies for primary myelofibrosis?
- Supportive care
- Chemotherapy (e.g., hydroxycarbamide)
- Targeted therapies (e.g., JAK2 inhibitors - ruxolitinib)
- Allogenic stem cell transplant
What management strategies are used for polycythaemia vera?
- Venesection
- Aspirin
- Chemotherapy (e.g., hydroxycarbamide)
What are the management strategies for essential thrombocytopenia?
- Aspirin
- Chemotherapy (e.g., hydroxycarbamide)
- Anagrelide
How to differentiate myeloproliferative disorders with myelodsplatic syndromes?
Myeloproliferative - MPD (P - proliferate, too many MATURE cells)
Myelodysplastic - MDS (D - dysfunctional, too many IMMATURE or faulty cells)
Both are myeloid affecting.
Myelodysplastic has > blasts so more chance developing into AML.
