Haematology Flashcards
AML translocation
8:21
AML Epidemiology
Older people (mean age 70)
AML histology
1) 20% blasts in marrow
2) Large myeloblasts
3) Auer Rods - crystallised myeloperoxidase
AML presenting
- Pallor
- Fatigue
- Gingivitis
- Splenomegaly
AML Investigations
Bone Marrow aspiration and trephination
FBC Clotting Screen Blood film CXR, ultrasound ECG Echo
AML Management
Inpatient chemotherapy
Stem cell transplant
CML translocation
Philadelphia chromosome (9;22)
CML epidemiology
Older people 60-65
CML presentation
- Incidental finding
- Splenomegaly, bruising
- B symptoms (fever, night sweats, weight loss)
- Abdominal fullness
- Gout
CML investigations
- FBC
- Peripheral blood smear
- U&Es
- Aspiration and trephination -> Flow cytometry, cytogenetics (Philadelphia), molecular (PCR studies)
CML management
1) Tyrosine kinase inhibitors
ALL epidemiology
Children & 40+
ALL risk factors
Down’s syndrome
EBV
Genetic
ALL translocation
Philadelphia poor prognosis
ALL presenting
- Fatigue, dizziness, palpitations
- Severe & unusual bone pain
- Fever
- Dyspnoea (mass symptoms if large thymic mass)
ALL investigations
- FBC
- Peripheral blood smear
- U&Es
- CLOTTING
- Aspiration and trephination -> Flow cytometry, cytogenetics (Philadelphia), molecular (PCR studies)
Imaging
- CXR (mediastinal mass)
- Echocardiogram
Goals of chemotherapy in ALL
To eliminate more than 99% of the initial burden of leukaemic cells.
To restore rapidly normal haematopoiesis.
To restore previous performance status.
Special management after remission in ALL
CNS prophylaxis - intrathecal chemotherapy
CLL presentation
Often incidental
CLL epidemiology
Older people
CLL management
Watch and wait
Chemo
CLL special progression
5% transform into DBCL - all lymph nodes should be checked at examination
CML monitorings (CML!!)
Monitor cytogenetic response throughout
treatment – FISH looking at % of PH+ cells
CLL histology
Smudge cells
When to treat CLL
- doubling of lymphocyte count in 6 months
- marrow failure
- recurrent infections
- massive or progressive splenomegaly
- systemic symptoms
- immune mediated cytopenias
CML additional treatment (CML!!)
Stem cell transplant
- graft versus host
- veno-occlusive disease
CLL management
Chemotherapy if active disease
Stem cell transplant
Causes of massive (>8cm) splenomegaly in all people
CML
Malaria
Lymphatic filiriasis
Cirrhosis
Causes of massive splenomegaly in young people
- Glandular fever
- Haemolytic disease -thallasaemias
- ALL
What considerations need before starting hydroxycarbamide
hepatic and renal function
fertility and contraception
How to measure CML remission
Haematologic remission (normal FBC count, physical examination, ie no organomegaly).
Cytogenetic remission (normal chromosome returns with 0% Ph-positive cells).
Molecular remission (negative PCR result for the mutational BCR-ABL m-RNA).
Three phases of CMLA
Chronic
Accelerated
Blast phase
What to do if treatment with imatinib but no cytogenetic remission?
Different TKI
How to investigate anaemia
FBC
B12, folate, ferritin
Coagulation screen
What medication to give in AML before definitive treatment
hydration, allopurinol
blood transfusion
aciclovir & anti-fungal
hydroxycarbamide
AML prognosis (3)
age, cell type and the burden of the diseas
AML remission in younger patients (young adult & child)
In younger patients, complete remission rates of at least 80% may be reached, with five-year overall survival about 40%.
Myeloma presentaiton
Calcium metabolism
Renal problems
Anaemia
Bone pain
Myeloma investigations
Myeloma screen - serum PROTEIN electrophoresis - serum free light chain Urine microscopy - Bence jones protein
Myeloma disease response measurement
Paraproteins
Multiple myeloma vs asymptomatic myeloma vs MGUS
MM - >10% bone marrow plasma cells - >30g/l monoclonal protein -> CRAB symptoms Asymptomatic -> as above but no CRAB symptoms MGUS -> <10% bone marrow plasma cells -> <30g/l monoclonal protein -> No CRAB
Management MM
Young - autologous bone marrow transplant
Elderly - melphalan and steroids
Hodgkin’s lymphoma key bits
- Lymphadenopathy
- Pain with alcohol
- Mediastinal mass
- B symptoms Fever, night sweats, weight loss
- Anne Arbor staging
- younger patients
Key considerations in management of young HL
fertility
What scan used in HL
PET scan
Four types NHL
Low grade/indolent
- Follicular, MALT
High grade/agressive
- DBCL, Burkitt’s
Polycythaemia rubra vera presentation
- routine
- fatigue, itchyness
- thrombosis (stroke/DVT)
- Budd chiari
What is Budd chiari
Hepatic vein thrombosis resulting in RUQ pain, ascites,
Three causes of secondary polycythaemai
COPD
EPO use
Eisenmenger syndrome
Mutation associated with PCRV
JAK2
Other myelodysplastic syndrome
Essential thrombocythaemia
Myelofibrosis
How to manage PCRV
Aspirin
Phlebotomy
Hydroxycarbamid
Essential thrombocythaemia symptoms
Bleeding + thrombosis
Essential thrombocythaemia management
Hydroxycarbamide