Haematological Malignancies Flashcards
Most common childhood cancer
Acute lymphoblastic leukaemia
Epidemiology of acute myeloid leukaemia
more common in elderly
Difference between “acute” and “chronic” leukaemiad
both are proliferation of abnormal blood cell progenitors
in acute types there is also a block to maturation/differentiation
This does not occur in the chronic types.
characteristics of a ‘blast’
high nuclear to cytoplasmic ration
Auer rod
seen in acute myeloid leukaemia
how are leukaemias investigated
Blood count and film Coag screen Marrow aspirate Immunophenotype - definitive diagnosis Cytogenetics - prognostic significance Trephine biopsy (piece of bone) better assessment and helpful is aspirate sub optimal
what is immunophenotyping
uses flow cytometry to look at what proteins (antigens) are present on the cell surface using antibodies
Describe the curative treatment of ALL
chemotherapy lasting up to 2-3 years
induction, consolidation, intensification and maintenance
Decribe the curative treatment of AML
intensive
2-4 cycles of chemo (5-10 days of chemo followed by t-4 weeks recoveraly)
prolonged hospitalisation
how is chemo administered
via a hickman line (central venous catheter)
what type of bacteria usually causes neutropenic sepsis
gram negatives
name the complications associated with marrow supression in leukaemias
anaemia
neutropenia
thrombocytopenia - bleeding, purpura, petechia
complications of chemotherapy
nausea and vomiting hair loss liver, renal dysfunction tumour lysis syndrome loss of fertility cardiomyopathy with antracyclines
what is tumour lysis syndrome
a group of metabolic abnormalities that can occur as a complication during the treatment of cancer,[1] where large amounts of tumor cells are killed off (lysed) at the same time by the treatment, releasing their contents into the bloodstream
what is APML
acute promyelocytic leukaemia- associated with specific translocation and DIC
how does chemo/radiothearpy work
damages cells as they are dividing
cell recognises damaged beyond repair so undergoes apoptosis
which genetic conditions have an increased risk of haematoligcal malignancies (leukaemia)
downs
klinefleters
what type of cells does chronic lymphocytic leukaemia usually affect
b cells
what is seen on blood film of CLL and is pathoneumonic
smear cells
treatment of CLL
watchful waiting
supportive
chemo
rituximab
what is ricter syndrome
transformation of CLL to high grade B cell lymphoma
name the myeloproliferative disorders
polycythaemia rubra vera
Essential thrombocythemia
Myelofibrosis
CML
symptoms of polycythaemia
headaches, visual distrubances, vascular events, erythromylagia, gout, itching classically after shower or bath - due to increased histamine release
treatment of polycythaemia
venesection
aspirin
hydroxyurea
what is polycythaemia
myeloproliferative disorder in which there is increase roduction of red blood cells
what is ET
increased megakaryocytes - increased platelets
how is ET treated
antiplatelets
hydroxyurea
what is myelofibrosis
also increased megakaryocity production but leads to bone marrow fibrosis and inefective erythropoesis
no increased platelets like in ET
“tear drop poikilocytes”
myelofirosis
treatment of myelofibrossi
bone marrow transplant
philidelphia chromosome
CML
translocation of BCR - ABL chromosomes 22 and 9.
specific treatment of CML
tyrosine kinase inhibitor - imatinib
most common type of lymphoma
non hodgkins (80 percent) - usually b cell
name types of t cell lymphoma
cutaneous (mycosis fungoides)
enteropathy (in coeliac)
reed sternberg cells
hodgkins lymphoma
most common type of hodgkins
nodular sclerosing
staging of lymphoma
1 - one site of lymphadenopaty e.g. neck
2 - two sites on same side of diapgragm e.g. neck and axilla
3. - two or more sites on different sides of diaphragm e.g. neck and groin
4. - widespread extra nodal involvement multiple sites
what is myeloma
cancer affecting plasma cells - disease characterised by abnormal monoclonal antibody formation (para proteins)
what type of antibodies affected in myeloma
IgG
bence jones proteins
myeloma
myeloma symptoms
lytic bone lesions due to increased osteoclast activity
hypercalcaemia
renal failure
bone marrow faillure - anaemia and neutropenia
blood film in myeloma
rouleux
raised ESR + osteoporosis
myeloma until proven otherwise
treatment of myeloma
supportive - hydrate, bisphosphates, local radiotherrapy, EPO and transfusions
life prolonging - prednisilone and bone marrow transplant
typical sites of bone lesions in myeloma
vertebrae
skull
ribs
investigation of leukaemia
FBC and blood film
marrow aspirate/biopsy
immunophenotyping
cytogenetics - prognosis
investigation of lymphoma
FBC and blood film
ultrasound and excisional biopsy
immunohistochemistry - to subclassify e.g. CD 20 positice in NHL and CD30 positive reed sternberg cells in hodgkins
immunophenotyping
what is seen on electrophoresis of serum protein in myeloma
a large narrow spinke in gamma region - - due to increased monoclonal antibodies - IgG
investigation of myeloma
serum protein electrophoresis serum light chain assay bone marrow examination urin protein eletrophoresis xrays of the bones
describe the process of b cell maturation
b cell identifies antigen in circulation and moves to lymph node (naive b cell)
enters mantle zone (mantle cell)
enters germinal centre (centroblast –> centrocyte)
enters marginal zone (marginal)
enters circulation (plasma cell)
give the types of lymphoma arising from each stage of b cell
naive - small lymphcytic mantle - mantle centroblast - diffuse large b cell centrocyte - follicular marginal - marginal
give the high grade lymphomas
burkitts
diffuse large b cell
mantle
more curable
give the low grade lymphomas
small lymphocytlc
follicular
marginal
less curable
which lymphoma is associated with EBV
burkitts
type types of non hodgkins
classical
nodular sclerosing
most common type of NHL
nodular sclerosing
