Myeloid Malignancy Flashcards
4 types of myeloid malignancy
- Acute myeloid leukaemia (AML)
- Chronic myeloid leukaema (CML)
- Myelodysplastic syndromes (MDS)
- Myeloproliferative diseases (MPD)
Describe acute myeloid leukaemia
- Leukaemic cells don’t differentiate
- Bone marraw failure
- Rapidly fatal if untreated
- Potentially curable
Describe Chronic myeloid leukaemia
- Leukaemic cells able to differentiate
- Proliferative without bone marrow failure
- Survival for a few years
- Long term survival/possible cures with modern therapy
Sub groups of acute leukaemia
- Acute myeloblastic leukaemia (AML)
- Acute lymphoblastic leukaemia (ALL)
Clinical features of AML
- Bone marrow failure
- Anaemia
- Thrombocytopenic bleeding (purpura + mucosal bleeding)
- Infection due to neutropenia (mainly bacterial + fungal)
Rx of AML
-Support care
-Anti-leukaemic chemotherapy
-Stem cell transplantation (allogenic)
-Targeted antibodies (midostaurin + Myelotarg anti-CD3 with Calicheomycin)
(Tretinoin in APL (acute promyelocytic leukemia) )
Presentation of chronic myeloid leukaemia
- Anaemia
- Splenomegaly (often massive)
- Weight loss
- Gout
- Hyperleukostasis (fundal haemorrhage + venous congestion, altered consciousness + resp. failure)
Labratory features of CML
- High WCC
- High platelet count
- Anaemia
- Bone marrow is hypercellular
- Blood film = all stages of WC differentiation with increased basophils
- Bone marrow + blood cells contatin the Philadelphia chromosome
Rx of chronic myeloid leukaemia
Direct inhibitors of BCR-ABL first line in all patients nowadays
Tyrosine kinase inhibitors
- Imatinib
- Dasatinib
- Nilotinib
- Busitinib
- Ponatinib
Allogenic transplant (few now) (only in TKI failures)
What is pancytopenia
Deficiency of all three cellular components of the blood (red cells, white cells, and platelets).
How do myelodysplastic syndromes present
- Macrocytic anaemia + pancytopenia
- Commonly seen in old age
What are myelodysplastic syndromes
- Acquired clonal disorders of the bone marrow
- They’re pre-leukaemic
Prognosis of myelodysplastic syndromes
They’re fatal as a result of progression to bone marrow failur or AML
Rx of myelodysplastic syndromes
- Supportive
- Stem cell transplantation for the few young people
3 myeloproliferative diseases
- Polycythaemia vera
- Essential thrombocythaemia
- Idiopathic myelofibrosis
Polycythaemia vera clinical features
- Headaches
- Itch
- Vascular occlusion
- Thrombosis
- TIA/Stroke
- Splenomegaly
Laboratory features of polycythaemia vera
- Raised Hb conc. + haematocrit
- May have raised WC + platelet count
- Raised uric acid
- Increase in RBC mass when the volume is measured
Rx for polycythaemia vera
- Venesection to keep haematocrit below 0.45 in men + 0.43 in women
- Aspirin
- Hydroxcarbamide
Natural Hx of polycythaemia vera
- Stroke + other arterial/venous thromboses if poorly controlled
- Bone marrow failure from the development of secondary myelofibrosis
- Transformation to AML
What is essential thrombocythaemia
- Myeloproliferative disease, predominant feature is raised platelets
- 5% +ve for JAK2V617F mutation
Symptoms of essential thrombocythaemia
- Arterial + venous thromboses
- Digital ischaemia
- Gout
- Mild splenomegaly
Rx for essential thrombocythaemia
- Aspirin
- Hydroxyurea
What can essential thrombocythaemia progress to
- Myelofibrosis
- AML
