Myeloid malignancy Flashcards
What are essential investigations for acute leukemia?
Blood count and blood film
Bone marrow aspirate - Blasts >20% of marrow cells in acute leukemia
Cytogenetics from leukaemic blasts
Immunophenotyping of leukaemic blasts
CSF examination if symptoms
Targeted molecular genetics for associated acquired gene mutations
What are clinical features of acute myeloid leukaemia?
Bone marrow failure
Anaemia
Thromboycytopenic bleeding
Infection due to neutropenia - bacterial and fungal
How is acute myeloid leukaemia treated?
Supportive care
Anti-leukaemic chemotherapy
Allogeneic stem cell transplantation - consolidate remission/potential cure
All-trans retinoic acid and arsenic trioxide in lowe risk promyelocytic leukaemia
Targeted treatment
What drugs are used in anti-leukaemic chemotherapy?
Daunorubicin and cytosine arabinoside
High dose cytosine arabinoside
Gemtuzumab ozogamicin
CPX-351
What are new developments of chemotherapy?
Targeted antibodies - gemtuzumab ozogamicin: Anti-CD33 with calicheomycin
Targeted small molecules: Midostaurin: tyrosine kinase inhibitor inhibiting FTL3
What is the most effective anti leukaemic strategy?
Allogeneic stem cell transplantation - give patient a new immune system - high risk high reward
What are features of chronic myeloid leukaemia?
Anaemia Splenomegaly Weight loss Hyperleukostasis - fundal haemorrhage and venous congestion, altered consciousness, resp failure Gout
What are lab features of CML?
High white cells
High platelets
Anaemia
Blood film shows all stages of white cell differentiation with increased basophils
Bone marrow is hypercellular
Bone marrow and blood cells contain philadelphia chromosome
How is CML treated?
Tyrosine kinase inhibitors - imatinib, dasatinib, nilotinib
Busitinib
Ponatinib
What are different myeloproliferative neoplasms?
Polycythaemia Vera
Essential Thrombocythaemia
Idiopathic myelofibrosis
What are clinical features of polycythaemia vera?
Headaches Itch Vascular occlusion Thrombosis TIA/stroke Splenomegaly
What are lab features of polycythaemia vera?
Raised haemoglobin concentration and haematocrit
Tendency to have raised white cell and platelet
Raised uric acid
True increase in red cell mass when blood volume is measured
What is haematocrit?
Proportion of blood made up by packed red cells after test - normally 45%
How is Polycythaemia vera treated?
Venesection to keep haematocrit below 0.45 - MAIN TREATMENT
Aspirin
Hydroxcarbamide/alpha interferon
What is natural history of polycythaemia vera?
Stroke and other arterial or venous thromboses if poorly controlled
Bone marrow failure from development of secondary myelofibrosis
Transformation to acute myeloid leukaemia
