Myeloid malignancies Flashcards
What are the key features of AML?
Leukaemia cells do not differentiate
Bone marrow failure
What are the clinical features of AML?
Bone marrow failure- anaemia, thrombocytopenia, infection
What causes infection in AML?
Neutropenia
Mainly bacterial and fungal
What investigations are done for AML?
Blood count and film
Bone marrow aspirate
Cytogenics and immunophenotyping of leukaemia blasts
CSF if symptomatic
What is the treatment of AML?
Chemotherapy
stem cell transplant
Targeted teratment
Low risk= All-trans retinoid acid ATRA and arsenic trioxide ATO
What ae the key features of CML?
Leukaemia cells retain ability to differentiate
Proliferative without bone marrow failure
What are the clinical features of CML?
Anaemia Splenomegaly- often massive Weight loss Hyperleukostasis Gout
What are the symptoms of hyperleukostasis in CML?
Fundal haemorrhage and venous congestion
Altered conciousness
Resp failure
What investigations are done for CML?
Blood count and film- high WCC and platelets, anaemia
Bone marrow aspiration
Philadelphia chromosome in blood and bone marrow
What is the treatment of CML?
Tyrosine kinase inhibitors
Allogenic transplant- rare not, if TKIs fail
What are myelodysplastic syndromes?
Acquired clonal disorders of the bone marrow
Pre-leukaemic
What is th epidemiology of myelodysplastic syndromes?
Commonly seen in ol age, rarer in younger
What are the clinical features of myelodysplastic syndromes?
Present as macrocytic anaemia and pancytopenia
What is the treatment of myelodysplastic syndromes?
Supportive
Stem cell transplant in young
What are the complications of myelodysplastic syndromes?
Progression to AML
What are the myeloproliferative neoplasms?
Poycythaemia vera
Essential thrombocythaemia
Idiopathic myelofibrosis
What is polycythaemia vera?
Excess of red cells
What are the clinical features of PV?
Headaches Itch Vascular occlusion Thrombosis TIA, stroke Splenomegaly
What investigations are done for PV?
FBC- raised rec cells
Raised WCC and platelets
Raised uric acid
Increase in red cell mass
What is the treatment of PV?
Standard- Venesection and aspirin
Hydroxycarbamide- prevention of stroke
JAK2 inhibitor- in HC failure with systemic symptoms
What are the complications of PV?
Stroke
Thrombosis
Bone marrow failure
Transformation to AML
What is essential thrombocythaemia ET?
Excess in platelets
What are the clinical features of ET?
Arterial and venous thrombosis Digital ischaemia Gout Headaches Mild splenomegaly
What is the treatment of ET?
Aspirin
Hydroxycarbamide or anagrelide
What are the complications of ET?
Progression to myelofibrosis or AML
