Haematological malignancies Flashcards
How do we classify haematological malignancy?
- Myeloid
- Acute
- AML
- Chronic (Myeloproliferative)
- CML
- Myelofibrosis
- Polycythemia Vera
- Essential thrombocytosis
- Acute
- Lyphoid
- ALL
- CCL
- Lymphoma
- Myeloma
What is the difference between acute myeloid leukaemia and chronic myeloid leukaemia?
Acute myeloid leukaemia affects more immature cells.
Chronic myeloid leukaemia affects more mature cells.
Clinical features of haematological malignancy
- Bone marrow failure
- Anaemia
- Fatigue
- Pallor
- Thrombocytopaenia
- Bleeding: bruising and epistaxis
- Dysfunctional white cells
- Recurrent infections
- Constitutional symptoms
- Weight loss
- Fatigue
- Fever
- Loss of appetite
- Infiltration
- Hepatosplenomegaly
- Lymphadenopathy
- Gum hyperplasia (AML)
General investigations for haematological malignancy
- Bedside
- Full set of observations Bloods
- Bloods
- FBC
- Blood film
- Clotting screen
- Imaging
- CT imaging
- Special tests
- Bone marrow aspirate and biopsy
- Lymph node biopsy
- Immunophenotype
- Genetic testing
Definition of acute myeloid leukaemia
Proliferation of immature myeloid cells
Epidemiology of acute myeloid leakaemia
Most common acute leukaemia in adults
Risk factors for acute myeloid leukaemia
- Age: average age of diagnosis is 68 years old
- Myelodysplasia: precursor lesion and evolves to AML in 30% of cases
- Chemotherapy
- Radiotherapy
Which subtype of acute myeloid leukaemia is associated with t(15;17)?
Acute promyelocytic leukaemia (M3)
Which subtype of AML is most commonly associated with gum infiltration?
Acute monocytic leukaemia (M5)
Which subtype of acute myeloid leukaemia is associated with disseminated intravascular coagulation?
Acute promyelocytic leukaemia
How do you differentiate myelodysplasia (precursor lesion to AML) from AML?
In myelodysplasia blast cells are <20% on bone marrow
Investigations in acute myeloid leukaemia
-
FBC: leukocytosis, thrombocytopenia, anaemia
- Blast cells crowd out bone marrow causing low PLTs and Hb
- Blood film: immature myeloid cells with auer rods
- Clotting screen: deranged in disseminated intravascular coagulation
- Bone marrow biopsy: ≥20% myeloblasts is diagnostic
- Cytogenetic studies: t(15;17)
Management of acute myeloid leukaemia
- Induction
- APML: all-trans retinoic acid
- Consolidation
Which translocation is associated with chronic myeloid leukaemia?
t(9;22)
Investigations for chronic myeloid leukaemia
-
FBC: leucocytosis, thrombocytosis or thrombocytopenia, anaemia
- Raised granulocyte count
- Blood film: increased number of mature granulocytes (<10% blast cells)
-
Bone marrow biopsy: increased number of mature granulocytes
- Myeloblasts not prevalent
- Cytogenic studies:Philidelphia chromosome t(9;22)
Managment of chronic myeloid leukaemia
Tyrosine kinase inhibitor eg imatinib
What is blast transformation?
Poorly controlled chronic myeloid leukaemia can transform or acute leukaemia.
There will be ≥20% blast cells in blood
2/3rds AML; 1/3rd ALL
Definition of myelofibrosis
Myeloproliferative condition. Neoplastic proliferation of mature myeloid cells, particularly megakaryocytes, leading to marrow fibrosis.
Investigations for myelofibrosis
- FBC: anaemia, other cell counts may be low or variable
-
Blood film: leucoerythroblastic smear
- Tear drop RBCs, nuculated RBCs, immature granulocytes
- Bone marrow aspirate:‘dry tap’
- Bone marrow biopsy: marrow fibrosis
- Genetics: JAK2 mutation in 50-60% of patients
Definition of polycythaemia vera
Neoplastic proliferation of mature myeloid cells, particularly RBCs. Can also have thrombocytosis and granulocytosis.
Clinical features of polycythaemia vera
Investigations in polycythaemia Vera
- FBC: raised Hb and haematocrit
- Granulocytes and PLTs may also be raised
- EPO: low in polycythaemia vera
- Raised in secondary polycythaemia eg hypoxia
- Genetics: JAK2 V617F mutation in 95% of patients
Managment of polycythaemia Vera
- Phlebotomy: aim for haematocrit <45%
- Aspirin
- Hydroxyurea: in patients at high risk of thrombosis eg >60 years of age
Define essential thrombocytosis
Neoplastic proliferation of mature myeloid cells, particularly megakarocytes.
Increased risk of bleeding and/or thrombosis.
Investigations for essential thrombocytosis
- FBC: thrombocytosis (PLTs > 600,000)
- Bone marrow biopsy: increased megakaryocytes
- Genetics: JAK2 mutation in 50-60% of patients
Managment of essential thrombocytosis
- Antiplatelet eg aspirin
- Hydroxycarbamide