leukaemia + myeloma Flashcards
leukaemia
cancer of particular line of stem cells in bone marrow, causing unregulated excessive production of a specific blood cell
- can suppress other cell lines -> pancytopenia
acute/chronic
myeloid/lymphoid
leukaemia presentation
fatigue, fever
pallor due to anaemia
petechiae or bruising due to thrombocytopenia
abnormal bleeding
lymphadenopathy
hepatosplenomegaly
failure to thrive (kids)
definitive diagnosis of leukaemia
bone marrow biopsy
- taken from iliac crest
–>aspiration or trephine - trephine better
acute lymphoblastic leukaemia
acute proliferation of lymphocytes, usually B-lymphocytes
–> causing pancytopenia
kids <5
more common in downs syndrome
assoc with philadelphia chromosome
chronic lymphocytic leukaemia
slow proliferation of lymphocytes, usually B-lymphocytes
adults >60yrs
often asymptomatic - infection, anaemia, bleeding, weightloss
may cause warm haemolytic anaemia
smear/smudge cells present on blood film (ruptured WBCs)
Richters transformation!!
Richters tranformation
refers to rare transformation of CLL –> high grade B cell lymphoma
leukaemia cells enter lymph node + change
- patients often become unwell very suddenly
chronic myeloid leukaemia
assoc with philadelphia chromosome 95% patients
phases
1. chronic - asymptomatic, raised WCC, can last years
- accelerated - most symptomatic, anaemia + thrombocytopenia
- blast phase - high proportion of blast cells in blood, pancytopenia, fatal
Philadelphia chromosome
abnormal chromosome 22 caused by reciprocal translocation (swap) of genetic material between chromosome 9 + 22
-> this creates an abnormal gene = BCR-ABL1 which codes for an abnormal tyrosine kinase enzyme that drives proliferation of the abnormal cells
assoc with CML
acute myeloid leukaemia
can be secondary transformation of a myeloproliferative disorder
Auer rods in cytoplasm of blast cells
rapidly progresses
chronic lymphocytic leukaemia complications
- anaemia
- hypogammaglobulinaemia leading to recurrent infections
- warm autoimmue haemolytic anaemia
- transformation to high grade lymphoma (Richters)
general management of leukaemia
MDT, chemo + targeted therapies
others - bone marrow transplant
targeted therapies (mainly in CLL)
- tyrosine kinase inhibitors - ibrutinib
- monoclonal antibodies - rituximab (targets B-cells)
electrolyte abnormalities in tumour lysis syndrome
high uric acid
high potassium
high phophate
low calcium
(AKI from uric acid forming crystals)
myelodysplastic syndrome
arises from mutations in haemoapoietic stem cells
- ineffective haematopoiesis
- pancytopenia
- risk of progression to AML
affect older adults -70-75s
how does seconday myelodysplastic currently occur
caused by chemo/radiotherapy
–> typically develops 5 years post treatment
investigations of myelodysplastic syndrome
blood count, blasts on blood film
diagnosis = bone marorw biopsy
- dysplastic changes in haematopoietic cells
- varying degree of blasts
management of myelodysplastic syndrome
supportive - transfusions
erythropoietin
targeted therapies = lenalidomide
only potential curative = haematopoietic (allogenic) stem cell transplant
multiple myeloma
haematological malignancy characterised by plasma cell proliferation
- arises due to genetic mutation which occur as B lymphocytes differentiate into mature plasma cells
myeloma features
mean age = 70yrs
CRABBI
- hypercalcaemia
- renal - light chain deposition in tubules
- anaemia
- bone - pathological fractures
- infection susceptibility
in myeloma, what can be seen on peripheral blood film?
rouleaux formation
myeloma investigations
FBC - anaemia, leukopenia
calcium riased
ESR + plasma viscosity riased
U&E - renal impairment
- Serum protein electrophoresis -> paraproteinaemia IgA/IgG
- Serum-free light-chain assay -> abundant light chains
- Urine protein electrophoresis -> Bence-Jones protein
definitive diagnosis = bone marrow biopsy
imaging in myeloma
1st = whole body MRI
xray = “rain drop skull”
why is hyperviscosity present in myeloma
plasma viscosity increase when more proteins are in blood, such as paraproteins found in myeloma
can cause;
- bleeding
- visual/eye sx
- stroke
- heart failure
myeloma bone disease
increased osteoclast + suppressed osteoblast
–> due to cytokines being released from abnormal plasma cells
osteoclast causes calcium reabsorption from bone to blood –> hypercalcaemia
presentation of CML
60-70yrs
- increase in granulocytes at different stages of maturation –> raised neuts, mono, eosin etc
- may be thrombocytosis
- may be marked splenomegaly - abdo discomfort
may undergo blast transformation
- AML in 80%
- ALL in 20%
