1.2 Acute Leukaemia Flashcards
define leukaemia:
A spectrum of diseases resulting from a malignant neoplastic proliferations of haemopoietic or lymphoid cells. It results from an acquired mutation in a single stem cell, the progeny of which form a clone of leukaemic cells
define acute leukaemia
Malignant transformation in the HSC or early progenitor which causes accumulation of blast cells (early HSC) in the bone marrow.
What is the dominant feature of acute leukaemia?
Bone marrow failure
What is cytopenia?
reduction in the number of blood cells
Define chronic leukaemia
A slowly progressive proliferation of haemopoietic or lymphoid cells. The leukaemic clone often retains some functional capabilities and the clinical disease is indolent. Over time, the disease process becomes more aggressive
Who normally gets AML?
adults >60 (most common leukaemia in adults)
M:f 5:3
Who normally gets ALL?
Highest incidence in childhood with = M:F
What are the features of acute leukeamia?
Bone marrow failure
What are the features of bone marrow failure?
Bone marrow failure
Leukopenia (low WCC - fever, infection, mouth ulcers)
Thrombocytopenia (low platelets): bleeding, ecchymoses, petechiae
Anaemia (low RCC): fatigue, SOB on exertion, exacerbation of CCF/IHD
What are the features of bone marrow failure?
Leukopenia (low WCC - fever, infection, mouth ulcers)
Thrombocytopenia (low platelets): bleeding, ecchymoses, petechiae
Anaemia (low RCC): fatigue, SOB on exertion, exacerbation of CCF/IHD
What are the extramedullary infiltration features of acute leukaemia?
Gingival hyperplasia Hepatosplenomegaly and lymphadenopathy Spinal cord compression and paraparesis Headache and seizures Nerve palsies Skin rashe/infiltration
What tests can be used to classify leukaemia?
Morphology of cells Immunophenotyping pattern Cytogenetics Molecular testing Disease behaviour and treatment response
What are the AML subtypes with recurrent genetic abnormalaties?
AML with t(8;21) RUNX1-RUNX1T1
AML with inv(16) CBFβ-MYH11
AML with t(15;17) PML- RAR α
What are the AML subtypes with normal cytogenetics?
AML with mutated NPM1
AML with FLT–3ITD
AML with mutated CEBPA
What are the three main classifications of AML?
AML with recurrent genetic abnormalities
AML with normal cytogenetics
Therpay related AML
What are the 3 main classifications of ALL?
B lymphoblastic leukaemia/lymphoma NOS
B lymphoblastic leukaemia/lymphoma with recurrent genetic abnormalities
T Lymphoblastic leukaemia/lymphoma
What is the usual FBC results of someone with leukaemia?
Low Hb, Low platelets, High WCC
What will you see in a bone marrow biopsy of leukaemia?
Loss of normal architecture
Loss of fat cells
Deep infiltrate of leukaemic blast cells
Deep blue, basophilic neuclei
What will you see in immunophenotyping of AML?
CD33+ CD13+ CD14+ myeloperoxidase + CD7+/- HLA-DR+
What will you see in immunophenotyping of ALL?
B cell lineage (90%) CD10+ CD 19+ TdT+ surface or cytoplasmic Ig T cell lineage (10%) CD2,3,4,8+/-
What are the common genetic mutations of AML?
FLT-3, NPM1, CEBPA
What is graft vs. leukaemia
Donor T cells eliminate host malignant T cells
What is graft vs, host disease and what are the complications
Occurs when the donor immune cells recognise host cells as foreign and begins to attack them. This can lead to skin rash, diarrhoea, thickening of skin and liver abnormalities
What is graft vs, host disease and what are the complications
Occurs when the donor immune cells recognise host cells as foreign and begins to attack them. This can lead to skin rash, diarrhoea, thickening of skin and liver abnormalities
What is graft vs. leukaemia
Donor T cells eliminate host malignant T cells (good)
What is graft vs, host disease and what are the complications
Occurs when the donor immune cells recognise host cells as foreign and begins to attack them. This can lead to skin rash, diarrhoea, thickening of skin and liver abnormalities
What are the general treatment principles in acute leukaemia?
Chemotherapy
Supportive care
Bone marrow transplant
Palliation
Good prognosis AML and ALL have reasonable cure rate with chemo alone
Poor prognosis need bone marrow transplant as well
What are the general treatment principles in acute leukaemia?
Chemotherapy
Supportive care
Bone marrow transplant
Palliation
Good prognosis AML and ALL have reasonable cure rate with chemo alone
Poor prognosis need bone marrow transplant as well
What are the types of B lymphoblastic leukaemia with recurrent genetic abnormalities?
t(9;22) BCR-ABL
t(12;21) TEL-AML-1
Hyperploidy
Hypoploidy
