Leukaemia Flashcards
Clinical Features of Acute Leukaemia?
BM Function Failure - Anaemia, Thrombocytopenia, Neutropenia
Organ Infiltration - Hepatomegaly, splenomegaly, lymphadenopathy, bone pain, CNS, skin, gun hypertrophy
Causes of Acute Leukaemia
Unknown
Ionising radiation
Cytotoxic drugs
Benzene
Pre-leukaemic disorders - Myelodysplastic/proliferative
Down’s Syndrome
Neonates (30% develop transient myelopoiesis which resolves itself)
Diagnosis of Acute Leukaemia
Morphology + cytochemisty (stains)
Immunophenotyping (lineage, differentiation)
Cytogenetics (chromosomal translocations)
Molecular genetics (PCR, point mutations)
ALL affects which age group?
Children
Children get it ALL
AML affects which age group
Adulthood (increases with age)
Additional features of ALL
Lymphadenopathy
CNS involvement
Testicular enlargement
Thymic enlargement
Additional features of AML
Lymphadenopathy less common
M3 - Acute promyelocytic leukaemia - prone to DIC
M4+5: Monoblasts/monocytes - skin / gum infiltration + hypokalaemia
Diagnosis of ALL
High WCC
Lymphocytes or precursors
Diagnosis of AML
High WCC
Auer rods and granules
Myeloperoxidase and Sudan Black B stains
Treatment for ALL
Remission induction: Chemo with steroids
Consolidation: High dose multi chemo, CNS treatment
Maintenance: 2 years in girls and adults, 3 years in boys
Consider allo-Stem Cell tranplant
Supportive: BLood products, Abx, Allopurinol, fluid, electrolytes - prevent tumour lysis syndrome
Treatment for AML
Similar principles No CNS prophylaxis / maintenance therapy Consider Allo-SCT in young ATRA for M3 Supportive Prognosis worse with age
A myeloproliferative disease affecting middle-aged (40 to 60)
CML
Diagnosed on routine bloods
Massive splenomegaly
Investigations for CML
Ph+ve (Philadelphia chromosome) in 80% Chromosomal translocation (9;22) PCR for BRC-ABL Monitor disease and therapeutic response WBC, Neutrophils 50-500 Hypercellular BM with spectrum of immature and mature granulocytes in blood
Definintion and treatment of Chronic phase of CML
Blast phase
Definition of Accelerated phase of CML
> 10% Blasts in blood
Lasting up to one year
Increasing manifestations
Less responsive
Definition of Blast phase of CML
> 20% Blasts in blood
Months of weight loss, lethargy, night sweats
Resembles Acute leukaemia
Treatment is similar to AML with possible SCT for young
A lymphoproliferative disease with the same process as SLL but presents in the BM of the elderly mainly in men
CLL
Clinical features of CLL
Asymptomatic diagnosed on blood (80%)
Symmetrical painless lymphadenopathy
BM failure
Weight loss, low fever, night sweats
Hepatosplenomegaly (less prominent)
Assoc with autoimmunity (Evan’s syndrome) AIHA, ITP
Can progress to lymphoma (DLBC) - Richter’s transformation
Investigations of CLL
High WBC with lymphocytosis >5 (high % composed of small mature lymphocytes)
Low serum Ig
Smear CLLs seen on blood film
Abnormal BM - lymphocytic replacement
In CLL, if LDH is raised what is the prognosis?
Bad
In CLL, if CD38 is positive what is the prognosis
bad
In CLL, if there has been an 11q23 deletion what is the prognosis
Bad
In CLL if there is a hypermutated Ig gene what is the prognosis
Good
In CLL if there is a low ZAP-70 expresssion what is the prognosis
good
In CLL, if there has been a 13q14 deletion what is the prognosis
Good
What is Binet staging
Staging for CLL Stage A High WBC 3 groups of enlarged LNs Stage C Anaemia of Thrombocytopenia
Treatment for CLL
1st Line Clorambucil (chemo) Fludarabine (purine analogue) Alemtuzumab (anti-CD52) Steroids SCT in young and fit
