Malignancy Flashcards
what is the best way to identify mature non-lymphoid cells?
morphology
malignant haemopoiesis is characterised by what?
inc numbers of abnorml and dysfunctional cells
what malignancy is characterised by proliferation of abnormal proegnitors with block in differentiation/maturation?
acute luekaemia
what malignancy is characterised by proliferation of abnormal progentiors but NO block in differentiation/maturation?
chronic myeloproliferative disorders
what is meant by ‘clone’ with regards to driver mutations?
population of cells dervied from one ‘parent cell’- the daughter cells share genetic marker of parent (they can diversify but will always share genetic backbone)
normal haemopoeisis is poly/monoclonal?
normal- polyclonal
malignant- usually monoclonal
what are the two types of haematological malignancy?
lineage (myeloid or lymphoid)
anatomical site (blood/lymph)
blood involvement is which kind of malignancy?
blood involvment = leukaemia
lymph node involvemnt is which kind of malignancy?
lymphoma
which malignancy involved the marrow?
myeloma → plasma cell malignancy in marrow
which malignancy can involve blood and lymph nodes?
Chronic Lymphocytic Leukaemia (CLL)
if somone presents with lymph nodes what should you always check?
bloods
define acute leukaemia
excess of ‘blasts’ >20% in either the peripheral blood or bone marrow → decrease of normal haemopoetic reserve i.e. low platelts, low neutrophils
who is acute myeloid leukaemia most common in?
more common in elderly
how is AML investigated?
blood count
coag screen
bone marrow aspirate
auer rods are typically seen in which malignancy?
Acute Myeloid Leukaemia (AML)
how is AML treated?
intensive chemo for multiple cycles
during chemo for AML risk of infection increases. if neutropenic fever suspected what should be done?
cover with broad spectrum antibiotic particulary gram -ve
what acute leukaemia tends to occur mainly in children?
acute lymphoblastic leukaemia ALL
ALL is a disease of which cells?
disease of primitive lymphoid cells (lymphoblasts)
ALL has a tendancy to involve which body system?
CNS
(in males can get into testes)
why is bone pain common in ALL?
rapid marrow expansion causes pain
how is ALL treated?
chemotherapy followed by maintainence therapy
what are the problems of marrow suppression?
anaemia
neutropenia
thrombocytopenia
which gram stain is higher risk in neutropenic patients?
gram -ve higher risk (can cause life threatening spesis within hours)
which responds better to treatment- low or high grade Non-hodgkins lymphoma?
high grade
(grows more rapidly but repsonds better to treatment than slow growing low grade tumours)
how is a diagnosis of Non-Hodgkins Lymphoma confirmed?
biopsy
what protein is found on Hodgkins lymphoma?
CD30
what is the treatment for Hodgkins?
brentuximab vedotin
who tends to get hodgkins and who gets NHL?
young- Hodgkins
older- Non-Hodgkins
which is more common Hodgkins or Non-Hodgkins?
Non-Hodgkins
what is the abnormal cell found in Hodgkins Lymphoma?
Reed-sternberg cells (abnormal type of B lymphocytes)
which has a better prognosis, hodgkins or non-hodgkins?
Hodgkins- generally easier to treat
what is a malignancy of plasma cells?
myeloma
what are the direct tumour cell effects of myeloma on the body?
bone lesions
bone pain
inc in calcium
marrwo failure
what are the paraprotein mediated effects of myeloma on the body?
renal failure
immune suppression
amyloid
how is myeloma classified?
type of antibody produced
what is the benign form of myeloma?
MGUS (premalignant)
(monoclonal gammopathy of unknown significance)
which steps in the cell cycle are involved in differentiation and in proliferation?
differentiate- G0 and G1
proliferate- S, G2, M
what the two types of treatments for tumours with regards to the cell cycle?
cell cycle specific (tumour specific)
non-cell cycle specific (non-tumour specific)
duration of dose rather than dose more important in specific or non-specific treamtent?
specific
In non-secific cumalitive dose more importnant rather than duration
tyrosine kinase inhibitors can be used in the treatment of which malignancy?
chronic myeloid leukamia
give an example of a tyrosine kinase inhibitor
imatinib
in what disease is philadelphia chromosome seen?
Chronic myeloid leukaemia
what are the two subtypes of Chronic Myeloproliferative DIsorders?
BRC-ABL1 positive
BRC-ABL1 negative
what is seen in CML?
proliferation if myeloid cells- granulocytes
other than myeloproliferative disorders what else can cause a high Hb?
chronic hypoxia i.e. COPD/sleep apnoea is a common reason for eleavted Hb
bloods can look identical in myeloproliferative disorders vs reactive but what differentiates the two?
eoisinophilia and basophilia in MPD
reactive will have an explanation i.e. empyema
what can be used to treat CML?
tyrosine kinase inhibitors i.e. imatinib
what are the three forms of BRC-ABL1 negative myeloproliferative disorders?
Polycythameia Rubra Vera
Idiopathic myelofibrosis
essential Thrombocytopenia
what are the two forms of Polycythaemia Rubra Vera?
primary or secondary
what are some signs of polycythaemia?
headache, fatigue
itchy- especially when in bath/shower
plethroic (redness)
what mutation is seen in >95% of people with Polycythaemia?
JKA2 mutation
what is the treamtent for polycythaemia rubra vera?
venesect to Hc<0.45
aspirin
cytotoxic oral chemo i.e. hydroxycarbamide
what is essential thrombocytopenia?
uncontrolled production of abnormal platelets?
what does essential thrombocytopenia result in?
thrombosis
at very high levels can cause bleeding >1500 (platelts can bind to VWF so circulatory levels decrease)
why is platelet count following surgery less realible in diagnosing throbocytopenia?
inc in platelets following surgery is normal- way of dealign with massive haemostatic challenge
the JAK 2 gene is found in 50% patients with essential thrombovytopenia. in those that dont what other gene mutation will be present?
CALR- calreticulin
MPL mutation also
how is essential thrombocytopenia treated?
aspirin
cytoreductive therapy to control proliferation i.e. hydroxycarbamide
tear drop shaped RBC’s can be seen in which mhyeloproliferatve disorder?
myelofibrosis
in additon to tear drop RBC’s what else is seen on blood film in myelofibrosis?
leucoerythroblastic
(nucelated red cells and myelocytes)
how is myelofibrosis treated?
supportive: blood transfusion, platelets
allogenic stem cell transplantation in select few
JAK2 inhibitors