Myeloproliferative Disorders Flashcards
All myeloproliferative disorders effect what stage of haematopoiesis?
Clonal stem cells
Is the marrow hypercellular, normocellular or hypocellular in myeloproliferative disorders?
Hypercellular
______ is preserved in myeloproliferative disorders
Maturation
Myeloproliferative disorders are a type of reactive change. T or F
False - myeloproliferative disorders are never reactive
What are the potential complications of all myeloproliferative disorders?
AML
Secondary myelofibrosis
How are myeloproliferative disorders classified?
BCR:ABL +ve
BCR:ABL -ve
What are the causes of BCR:ABL positive myeloproliferative disorders?
CML
What are the causes of BCR:ABL negative myeloproliferative disorders?
Polycythaemia rubra vera
Essential thrombocytheamia
Primary myelofibrosis
What lineages are effected in myeloproliferative disorders?
Myeloid
Hence may see raised granulocytes,RBCs, PLTs or Hb
What is the common presentation of all myeloproliferative disorders?
Asymptomatic or Splenomegaly Weight loss Night sweats Fatigue Gout Unusual thrombosis
What causes gout, fatigue, night sweats and weight loss in myeloproliferative disorders?
Increased cell turnover
What effect does polycythaemia rubra vera have on:
- RBC production
- Hb
- RBC numbers
- Total red cell mass
- HCT
All raised
Can also cause increased production of another lineage
What are the additional symptoms of polycythaemia rubra vera as well as those common to all myeloproliferative disorders?
Plethora
Aquagenic pruritus (itch worse in warm water)
HA
Fatigue
What causes the HA and fatigue in polycythaemia rubra vera?
Increased blood viscosity
What test is diagnostic of polycythaemia rubra vera and is useful in distinguishing it from secondary polycythaemia?
JAK2 mutation test
What is the management of polycythaemia rubra vera?
Venesect to HCT less than 0.45
Plus aspirin
What causes secondary polycythaemia?
Chronic hypoxia eg COPD
Both reactive
What causes pseudopolycythaemia?
Dehydration
Diuretics
Obesity
(All reactive)
What is the pathology of essential polycythaemia?
Uncontrolled proliferation of abnormal platelets
What is the additional complication in essential thrombocytheamia compared to other myeloproliferative disorders? How does this frequently present?
Thrombosis
Digit ischaemia
50% of patients with essential thrombocythaemia have what mutation? A smaller percentage have what other mutation?
50% JAK2 positive
Also CALR mutation
When investigating essential thrmbocythaemia, if a patient has no linked mutations what test should you do next?
Marrow biopsy
What is the main management of essential thrombocythaemia?
Aspirin
How does splenomegaly present?
Early satiety
LUQ pain / dragging sensation
Portal HTN
What is the cause of splenomegaly in myeloproliferative disorders?
Extramedullary hematopoiesis
What is the cause of primary myelofibrosis?
Idiopathic
What are the S+S in primary myelofibrosis due to hypercatabolism?
Itch
Night sweats
Weight loss
What is seen on blood film in primary myelofibrosis?
Teardrop RBCs (poikilocytes) Leukoerythroblastic
Which myeloproliferative disorder requires a biopsy to diagnose?
Primary myelofibrosis
What is the management of primary myelofibrosis?
Transfuse RBCs / PLTs
ABx
What is the cause of secondary myelofibrosis?
After years of essential thrombocythaemia or polycythaemia rubra vera
What is the commonest cause of thrombocytopenia?
Reactive - not MPD
