Myeloid Malignancies Flashcards
Types of myeloid malignancy
Acute myeloid leukaemia (AML)
Chronic Myeloid Leukaemia (CML)
Myelodysplastic Syndromes (MDS)
Myeloproliferative Neoplasm (MPN)
Sub groups of acute leukaemia
Acute Myeloblastic Leukaemia
Acute Lymphoblastic Leukaemia
Acute Myeoblastic leukaemia?
Bone marrow failure:
- Anaemia
- Thrombocytopenia bleeding (Purpura and mucosal membrane bleeding)
- infection because of neutropenia (predominately bacterial and fungal)
Essential investigations in AML
Blood count and blood film, Bone marrow aspirate/ trephine, Cytogenetics (karyotypes) from leukaemic blasts, Immunophenotyping of leukaemic blasts, CSF examination if symptoms
Targeted molecular genetics fo associated acquired gene mutations eg FLT3, NPM1, IDH 1 and 2
Treatment of AML (Acute Myeloblastic Leukaemia)
Supportive Anti-leukaemia chemotherapy, Allogeneic stem cell transplantation, All-Trans retinoic acid and arsenic trioxide Targeted treatment
New developments in AML
Targeted antibodies: -Gemtuzumab Ozogamicin Targeted small molecules: -Midostaurin New chemotherapy delivery systems: -CPX-351
Chronic Myeloid leukaemia presentation
Anaemia Splenomegaly Weight loss Hyperleukostasis- fundal haemorrhage, venous congestion, altered consciousness, resp failure Gout
Laboratory features of CML
High WCC
High platelet count
Anaemia
Blood film- shows all stages of white cell differention with increased basophils,
Bone marrow is hypercellular,
Bone marrow and blood cells contain the Philadelphia chromosome- t(9;22)
Treatment of CML
Tyrosine Kinase Inhibitors (Imatinib, dasatinib, nilotinib),
Direct inhibitors of BCR-ABL
Allogenic transplantation
Examples of myeloproliferative Neoplasms
Polycythaemia Vera,
Essential thrombocythaemia,
Idiopathic myelofibrosis
Polycythaemia Vera clinical features
Headaches, Itch, Vascular occlusion, Thrombosis, TIA, stroke, Splenomegaly
Lab features of PV
Raised haemoglobin concentration and haematocrit,
Raised white cell count and platelet count,
Raised uric acid,
True increase in red cell mass when blood volume is measured
Treatment of PRV
Venesection (keep haematocrit correct)
Aspirin,
Hydroxcarbamide/ alpha interferon,
History of PRV
Stroke and other arterial or venous thromboses,
Bone marrow failure (development of secondary myelofibrosis),
Transformation to AML
Essential Thrombocythemia (ET)
Myeloproliferative disease with predominant feature of raised platelet count,
JAK2V617F mutation,
S/S- arterial and venous thromboses, digital ischaemia, gout, headache, splenomegaly
T- aspirin, hydroxycarbamide or anagrelide
Can progress to myelofibrosis or AML
