malignancy Flashcards by Emma Sutcliffe

what is the difference between leukaemia and lymphoma

leukaemia involves marrow and blood - haemopoiesis lost

lymphoma involves nodes - immune function lost

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what cells are involved in acute myeloid leukaemia

common myeloid precursors eg myeloblasts

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what cells are involved in acute lymphoblastic leukaemis

common lymphoid precurosr eg lymphoblasts

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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

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what type o fmutations are responsible for haematological cancers

acquired driver mutations

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what is the difference between normal and malignant haemopoiesis in terms of clonality

norm- polyclonal

malignant - monoclonal

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what is a clone in haematology

population of cells derived from single parent cell

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which leukaemia can also involve nodes

chronic lymphoctic leukaemia

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what do acute leukaemia present with

marrow failure

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who gets AML and ALL respectively

AML- older people

ALL- children

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how would Acute lymphoblastic leukaemia present

anaemia, infection, bleeding, bone pain , CNS /testes involvement

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when are Auer rods seen and what are they

Acute myeloid leukaemia - abnormal granules in some blasts

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what is seen on blood film in AML

decreased Hb, platelets and neutophils and increased wcc

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what test is required for definitive diagnosis of AML

immunophenotyping

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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

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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

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what are A symptoms and B symptoms of lymphoma

A - lymphadenopathy

B -fever , night seats , weight loss

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what are other associated symptoms (esp hodgkins)

itch with out rash , pain with drinking alcohol

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are lymph nodes tender in malignancy

no only in viral or bacterial

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if surface of lymph node is irregular what would you expect

metastesis

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consistency of lump in lymphoma and in metastatic cancer

lymphoma - rubbery

mets - hard

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if inflam seen along side lymph node lump what is suspected

bacterial infection

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when would the lymhpnode be tethered

in mets and sometimes bacterial

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how is chronic lymphocytic leukaemia diagnosed

blood film (lymphocytosis) and immunophenotyping

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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

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