(Malignancy 1) Acute Leukaemias, Pre-leukaemia & Chronic Leukaemias Flashcards
Myelodysplastic syndrome = failure of effective ________ (results in low blood counts)
‘Low risk’ vs ‘high risk’ disease can be established by proportion of _____ ____ in marrow as well as the _______ _____ (aka chromosomal profile).
Leukaemia is ____ ___ over __%, myelodysplasia is
____ ____ under __% in relation to bone marrow.
% determines severity.
Incurable other than ___ ___ ______.
Myelodysplastic syndrome = failure of effective haematopoiesis (results in low blood counts)
‘Low risk’ vs ‘high risk’ disease can be established by proportion of blast cells in marrow as well as the cytogenetic profile (aka chromosomal profile).
Leukaemia is blast cells over 20%, myelodysplasia is blast cells under 20% in relation to bone marrow.
% determines severity.
Incurable other than stem cell transplant.
What are myeloproliferative disorders (MPDs)?
- they are _____ blood disorders
- ____ mutation is common
Basically, haematopoiesis is ‘too effective’:
- Essential thrombocytopenia: ___ ____ ______
- Polycythaemia vera / primary polycythaemia: ___ ____ ___
- myelofibrosis: __ ___ ____ _____
What are myeloproliferative disorders (MPDs)?
- they are clonal blood disorders
- JAK2 mutation is common
Basically, haematopoiesis is ‘too effective’:
- Essential thrombocytopenia: too many platelets
- Polycythaemia vera / primary polycythaemia: too many RBCs
- myelofibrosis: too much fibrous tissue
Think of it as MPDs as being overly effective haematopoiesis and MDS as being ineffective.
Outcomes for myeloproliferative disorders (MPDs):
ET and PRV:
- ___ outcome
- _____ to reduce risk of vascular events
- _________ for cytoreduction (_____ ____ ___)
- 10% risk of AML, 10% risk of myelofibrosis
Myelofibrosis:
- ___ outcome
- large ____
- only treatment is ___ ___ ______
Outcomes for myeloproliferative disorders (MPDS):
ET and PRV:
- good outcome
- aspirin to reduce risk of vascular events
- hydroxycarbamide for cytoreduction (reduces tumour size)
- 10% risk of AML, 10% risk of myelofibrosis
Myelofibrosis:
- bad outcome
- large spleen
- only treatment is stem cell transplant
Actue Leukaemia (AL):
- clonal disorder
- ____ ___ proliferation in bone marrow (‘maturation arrest’)
- _____ onset
- normal blood count ____
- death within ___ or ____ if untreated
Actue Leukaemia (AL):
- clonal disorder
- blast cell proliferation in bone marrow (‘maturation arrest’)
- rapid onset
- normal blood count falls
- death within days or weeks if untreated
Good pics for types of leukaemia in folder.
Incidence of acute leukaemias:
- Acute lymphoid leukaemia (ALL) = common in _____
- Acute myeloid leukaemia (AML) = common in _____
Incidence of acute leukaemias:
- Acute lymphoid leukaemia (ALL) = common in young
- Acute myeloid leukaemia (AML) = common in elderly
Good graph pic for this in folder.
Extra - AML cytogenetics (chromosome profile) essential to determine prognosis and treatment choice.
Acute leukaemia causes:
- chemicals
- chemotherapy
- radiotherapy
- genetic e.g. ____, Fanconi syndrome.
- Pre-leukaemia disorders = ___ and ___
- viruses
Acute leukaemia causes:
- chemicals
- chemotherapy
- radiotherapy
- genetic e.g. Down’s, Fanconi syndrome.
(NB - Fanconi syndrome is NOT the same as Fanconi anaemia) - Pre-leukaemia disorders = MPDs and MDS
- viruses
Diagnosis / presenting features on history + examination:
- ____ onset
- lethargy
- infection
- b_____ and b______
- b___ pain
- gum _______
- lymphadenopathy
- skin ___
Diagnosis / presenting features on history + examination:
- rapid onset
- lethargy
- infection
- bleeding and bruising
- bone pain
- gum swelling
- lymphadenopathy
- skin rash
diagnosis blood morphology pics in folder.
Management of AML, 3 choices:
- ______ chemo and ___ ___ ______ (<60-65, survival 5yr is 50%)
- ___ ___ chemo (>60-65, survival 5yr is <10%)
- ________ ___ ___ (much older patients, survival 3-6 months)
Management of AML, 3 choices:
- intensive chemo and stem cell transplant (<60-65, survival 5yr is 50%)
- low dose chemo (>60-65, survival 5yr is <10%)
- supportive care only (much older patients, survival 3-6 months)
NB - AML relapse common, 50%
Acute Lymphoid Leukaemia (ALL) common presentation:
- limping ____
- ______ rash
- ____ pains
Acute Lymphoid Leukaemia (ALL) common presentation:
- limping child
- purpuric rash
- bone pains
NB - check for ‘lumps’ vs ‘liquid’ (i.e. lymphoblastic lymphoma vs true ALL)
Remember lymphoma is lumpy and leukaemia is liquid.
ALL Management:
- __________ i.e. __________ can be used
- ___ directed treatment essential
- initially ________ treatment then ___ maintenance for years
- specialist ______
ALL Management:
- chemotherapy i.e. prednisalone can be used
- CNS directed treatment essential
- initially aggressive treatment then oral maintenance for years
- specialist nursing
Supportive care for acute leukaemia:
- ____ _______ for symptomatic patients
- ____ _____ ____ for coagulopathy / DIC
- ______ ______ for _____, bleeding, DIC (cos they get used up)
- ______
- Growth factors e.g. _-___
- ________ for refractory infections
Supportive care for acute leukaemia:
- blood transfusion for symptomatic patients
- fresh frozen plasma for coagulopathy / DIC
- platelet transfusion for purpura, bleeding, DIC (cos they get used up)
- antibiotics
- Growth factors e.g. G-CSF
- Granulocytes for refractory infections
Pic in folders
Allogeneic transplant for acute leukaemia:
- ___ matched stem cells
- ______ or ______ donor
- ____ vs ____ disease
- ____ vs _______
- higher earlier mortality
- [[less or more]] risk of relapse
Allogeneic transplant for acute leukaemia:
- HLA matched stem cells
- siblings or unrelated donor
- graft vs host disease
- graft vs leukaemia
- higher earlier mortality
- less risk of relapse (cos donor’s immune blood cells attacks patients leukaemia cells)
Chronic Leukaemias:
___ and ___ (good pic showing which is which in folder).
CML and CLL
CLL:
- commonest leukaemia
- more [[male or female]]
- may have no presenting features
- otherwise may have l____,
n___ s____, w___ l__, a____, l________, i_____. - FBC shows _______ (high lymphocytes)
CLL quick patho - reduction in functioning B-cells causes immunosuppression as the leukaemia B-cells outcompete the normal
CLL:
- commonest leukaemia
- more [[male or female]]
- may have no presenting features
- otherwise may have lethargy,
night sweats, weight loss, anaemia, lymphadenopathy, infections. - FBC shows lymphcytosis (high lymphocytes)
CLL quick patho - reduction in functioning B-cells causes immunosuppression as the leukaemia B-cells outcompete the normal
Diagnosis of CLL:
- clonal population of _ ________ (>5 x10^9)
- presence of CD _, __, __, __ (CDs are surface antigens)
- weak surface Ig (B lymphocytes have Ig/immunoglobulins/antobodies on surface)
- unique immunophenotype
Also, CD _ is normally expressed on T-cells so if it is being expressed by B-cells then indicates something must be wrong
Diagnosis of CLL:
- clonal population of B lymphocytes (>5 x10^9)
- presence of CD 5, 19, 20, 23 (CDs are surface antigens)
- weak surface Ig (B lymphocytes have Ig/immunoglobulins/antobodies on surface)
- unique immunophenotype
Also, CD 5 is normally expressed on T-cells so if it is being expressed by B-cells then indicates something must be wrong
