malignancy Flashcards
what is the difference between leukaemia and lymphoma
leukaemia involves marrow and blood - haemopoiesis lost
lymphoma involves nodes - immune function lost
what cells are involved in acute myeloid leukaemia
common myeloid precursors eg myeloblasts
what cells are involved in acute lymphoblastic leukaemis
common lymphoid precurosr eg lymphoblasts
what is the difference in proliferation of abnormal proginators in acute leukaemia compared to myeloproliferative disorders
acute leukaemia = block in differentiation or maturation
myeloproliferative disorder - No block in differentiation or maturation
what type o fmutations are responsible for haematological cancers
acquired driver mutations
what is the difference between normal and malignant haemopoiesis in terms of clonality
norm- polyclonal
malignant - monoclonal
what is a clone in haematology
population of cells derived from single parent cell
which leukaemia can also involve nodes
chronic lymphoctic leukaemia
what do acute leukaemia present with
marrow failure
who gets AML and ALL respectively
AML- older people
ALL- children
how would Acute lymphoblastic leukaemia present
anaemia, infection, bleeding, bone pain , CNS /testes involvement
when are Auer rods seen and what are they
Acute myeloid leukaemia - abnormal granules in some blasts
what is seen on blood film in AML
decreased Hb, platelets and neutophils and increased wcc
what test is required for definitive diagnosis of AML
immunophenotyping
if someone had leukaemia and is neutropenic with fever what is suspected and done
gram negative bacteria and broad spec antibiotics given after samples obtained
what is some complications of chemotherapy treatment
nausea and vomiting hair loss liver and renal dysfunction tumour lysis syndrom (1st cycle) infection - PCP, bacterial, fungal
later- infertility, cardiomopathy if anthracyclines used
what are A symptoms and B symptoms of lymphoma
A - lymphadenopathy
B -fever , night seats , weight loss
what are other associated symptoms (esp hodgkins)
itch with out rash , pain with drinking alcohol
are lymph nodes tender in malignancy
no only in viral or bacterial
if surface of lymph node is irregular what would you expect
metastesis
consistency of lump in lymphoma and in metastatic cancer
lymphoma - rubbery
mets - hard
if inflam seen along side lymph node lump what is suspected
bacterial infection
when would the lymhpnode be tethered
in mets and sometimes bacterial
how is chronic lymphocytic leukaemia diagnosed
blood film (lymphocytosis) and immunophenotyping
how are lymphomas diagnosed
excision biopsy
what cell is involved in hodgkins lymphoma
B-cell
risk factors for lymphoma
specifically what autoimmune conditions
immunosuppressed autoimmun -sjogrens, coeliac infection - EBV, H.pylori genetic environment
what staging system is used in lymphoma
ANN ARBOUR
what marker is measured for prognosis
serum lactate dehdrogenase
when are reed sternberg cells seen
hodgkins lymphoma
what is a reed sternebrg cell
binucelated inflammatory cell
when does hodgkins lymphoma pek
3rd decade
how is hodgkins lymphoma treated
multi agent chemo +/- radiotherapy
side effect of bleomycin
pneumonitis
what is done in hodgekins if treatment resistant to chemotherapy
immunotherapy/stem cell transplant
treatment of b - cell non hodkeins lymphoma
rituximab
treatment of t cell NHL
brentuximab
what is the fastest growing human tumour
burkitts lymphoma
what cell is involved in burkitts lymphoma
b- cell
what virus’ is burkitts assoc with
EBV and HIV
what are extramedullary sites forb urkitts lymphoma
bone, CNS, testes , kidney
what type of mutation adn on what gene is involved in burkitts
translocation involing c-myc genes
what should be monitored for at the start of treatment esp burkitts lymhoma
tumour lyisis
what is seen in tumour lysis syndrome
hyperkalaemia
hypocalcaemia
hyperphosphateamia
hyperuricaemia- renal falure
what should be given in emergency management of lymphoma
steroids
role of b cell
antigen presenting
antibody producing
what produces antibodies
plasma cells
what comprises and immunoglobulin
2 heavy chain (gives igM, A,D,E, G etc)
2 light chains (kappa and lambda )
what antibodies are monomers
IgD, IgE, IgG
what antibodies is a dimer
IgA
what antibodies is a tetramer
IgM
when b -cells leave the marrow what antibody do they originally express
IgM
clock face nucleus
plasma cell
what test is used to detect immunoglobulins on cells.
serum electrophoresis - identifies paraprotein
what is a paraprotein
a monoclonal immunoglobulin
electrophoresis identifies paraprotein, what test is used to classify what type
serum immunofixation
where are bence jones protein sampled from
urine
what is a bence jones protein
excess light chain production
causes of paraproteinaemia
MGUS Myeloma amyloid waldstrom others eg lymphoma
what is the most common cause of paraproteinaemis
MGUS
what do paraproteins do to the body
renal failure, immune suppression, hyperviscpsity and amyloid
what type of antibodies are produced in myeloma
IgG- most common
IgA
Bence Jones - free light chain
pepper pot skull
myeloms due to lytic bone lesion
how does myeloma result in hyeprcalcaemia and pepper pot skull
produces IL-6 which upregulates osteoclasts and down regulates osteblasts
.osteoclasts encourage myeloma production
therefore pepper pot skull and hypercalcaemia
what does bence jones do to kidneys
excess deposits in glomerulus - make casts
therefore cast nephropathythat block tubule
what medication stops the production of light chains
steroids
normal age of myeloma
65
outline treatment of myeloma
combo therpay including steroids-dexamethasone alkylating agenst - cyclophosphamide monoclonal antibodies - daratumab high dose chemo
how is response to treatemnt measure
paraportein level.
in the symptoms control for bone disease what drugs shoudl NOt be used
NSAIDs - use opiates for pain
survival of myeloma
5-10 yrs - relapse inevitable
what criteria is used to differentiate between MGUS and myeloma
CRAB
what does MGUS stand for
monoclonal gammopathy of undetermines significance
precrsor to myeloma
what paraprotein level is expected in MGUS
<30
difference between myeloma and MGUS
no evidence of myelom end organ damage
what is AMyloid pathophysiolody
build up of insolube b-pleated sheets
congo red stain
amyloid
what birefringement is seen in amyloid
apple green
what test confirms amyloid
biopsy
what paraprotein is involved in Waldsrtom macroglobulinaemia .
IgM paraprotein
treatmnt of waldrostrom
plasmaphoresis - removes paraprotein
what is waldrstom macroglobulinaemis
cross between lymphoma and myeloma
