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Chronic myeloid leukaemia (CML) (Complications (Infections, Sepsis,…
Chronic myeloid
leukaemia (CML)
Pathophysiology
Defect
Type of MPS, accumulation of
myeloid progenitors
Most have Ph chromosome (t(9;22),
forming fusion protein BCR-ABL
Mechanism
BCR-ABL is a constitutively active TK
Stimulates proliferation
Natural history
Chronic
Often asymptomatic
Lasts months-years
Accelerated
Increasing WCC proliferation
Increasing symptoms
Blastic
High WCC proliferation, BM failure
Features of acute leukaemia
Can rapidly progress to death
Clinical
presentation
Accelerated/blastic
Fever
Fatigue
Weight loss
Night sweats
Bleeding/bruising
Bony pains
Chronic
Asymptomatic
Diagnosis
Examination
Abdo exam
Hepatomegaly, splenomegaly
Investigations
Bloods
FBC (low Hb, low Plt, high WCC), U+E, LFT (deranged),
clotting (may be deranged), blood cultures (?sepsis)
Blood film (hypercellular)
Biopsy
BM (hypercellular), immunotyping, cytogenetics (Ph+)
Bedside
Obs (fever)
Height/weight (weight loss)
History
PC/HPC
Insidious or acute onset of
fatigue, malaise, weight loss,
night sweats, bleeding/bruising
PMH
Medical conditions,
cancers
DH
Meds, allergies
FH
Cancers
SH
Living arrangements, occupation,
smoking, alcohol
Management
Medical
TKI's
Indication: 1L CML
E.g. imatinib, dasatinib
MOA: BCR-ABL inhibitors,bind and
prevent ATP binding thus TKI activity
Surgical
Stem cell transplant
Indication: 1L CML in young, fit patients
MOA: allogenic transplantation,
can be curative
Conservative
Information, advice, support
MDT (speciality medic and surgeon, speciality nurse,
psychologist, counselling)
Monitoring (MRD by PCR)
Complications
Infections
Sepsis
Tumour lysis syndrome
Gout
Pancytopenia
DIC
VTE
Prognosis
Survival ~5y
Treat per ALL if
transformation
Definition
Malignant neoplasm
of leukocytes of the
myeloid lineage
Epidemiology
Rare in childhood
Middle age (40-60y)
M>F