Leukaemia Flashcards
What’s leukaemia?
A malignant condition of blood and blood forming tissue in the bone marrow and spleen.
What’s the aetiology?
- Predisposing factors – genetic / environmental
- Chronic exposure to chemicals (benzene)
- Radiation exposure (Bombs / Nuclear Power Plants/ treatment)
- Cytotoxic therapy for breast, lung, hodgkins and testicular cancer
- Down’s Syndrome, Fanconi’s Anaemia, haemolytic anaemia
- Myelodysplasia / Myeloprolferation
What are the signs & Symptoms of Bone Marrow Failure (in general)?
• Anaemia (fatigue / dyspnoea)
• Bleeding (bruising / gums / menorrhagia)
• Opportunistic infections (bacterial / viral / fungal / protzoal)
• Oral: – Palelips/skin
– Bleedinggums – Herpesinfections – Candidalinfections
• Hyperviscosity (CML) – Visual disturbance, headache, priapism
What are the symptoms & Signs of Organ Infiltration?
- Tender Bones (sternal in AML)
- Enlarged Lymph nodes
- Mediastinal Compression
- Meningeal Involvment
- Deposits
- Cranial Nerve Palsy
- Hepatosplenomegaly
- Testicular Swelling
- Retinal Haemorrhage
What are the types of Leukaemia?
• • • • Types of Leukaemia Acute Lymphoblastic (Lymphoid) – Lymphoblasts undergo malignant transformation and proliferation, subsequent replacement of normal marrow elements, leading to bone marrow failure and infiltration into other tissues. Chronic Lymphocytic (Lymphoid) – Cell accumulation in blood, bone marrow, liver, spleen and LN due to impaired apoptosis. Loss of Function mutation in P2X7 gene (codes for apoptotic protein) (Trisomy 12, 11q and 13q deletions) Acute Myeloblastic (Myeloid)(ASBO) – Myeloblasts, arrested at an early stage of development with varying cytogenic abnormalities undergo malignant transformation and proliferation, with replacement of normal marrow elements and bone marrow failure Chronic Myeloid (Myeloid) – Malignant transformation and proliferation of stem cells with characteristic chromosomal translocation (t9:22) resulting in the Philadelphia Chromosome.
What are the investigations that can be done?
– Blood Film
– Bone Marrow Aspirate – Cytogenic Analysis – Immunophenotyping – FBC
What are the General Principles of Management?
- Decide if going to cure
- Systemic, combination chemo may not be appropriate
- Correct Anaemia & Thrombocytopenia
- Blood Products / Platelet transfusions
- DIC: FFP
- Treat any infection
- Leucophoresis
- Life saving, centrifugal removal of blast cells
- Hyperuricaemia
- Allopurinol
- Tumour Lysis Syndrome
- Hypercalcaemia, phosphate and potassium
- Check uric acid level before start chemo
- Fluids
What’s acute myloblastic leukaemia?
- Subdivided in to morphological variants
- FAB classification – M0-M7
- M4 & M5 with gingival hypertrophy
- Main stay of treatment is combination chemotherapy • Stem Cell Transplantation
- Prognosis – Depends on age – cure rate 40-50% if under 60 and Chemo (more if BMT) – 5-10% long term survival if over 60
What’s acute lymphoblastic leukaemia?
• Sub classified by FAB • L1: small lymphoblasts, scanty cytoplasm • L2: larger, heterogenous lymphoblasts • L3:large lymphoblasts • BMA/Trephine • Hypercellular • Immunophenotyping • Cytogenetics • Cytochemistry • Lumbar Puncture • CXR / X-sectional imaging • Bone Radiographs • Combination Chemotherapy – Remission Induction, Consolidation/Intensification, CNS Tx, Maintenance – Supportive • Stem Cell Transplant • Prognosis – Childhood ALL 70% cure – Adult ALL 30% cure – 10 poor prognosis, males, translocations (4,11) (9,22) CNS involvement at presentation
What are Chronic Myeloid Leukaemias?
• Majority are older and die with in 5 years
• Progressive clinical phase
• Accerated Phase
• Blastic Phase (acute leukaemia: AML60% ALL30%)
– 6 months survival
• Philadelphia chromosome – Glivec – 90%complete haematological response – 30% 5 year survival to 89% with Glivec
• Other Treatments – Hydroxurea
– IFN (haem remission in 90%, cytological remission in 10%)
• Prognosis
– Can be good
What’s Chronic Lymphocytic Leukaemia?
- Incurable disease of the elderly
- Uncontrolledproliferationandaccumulationof mature B lymphocytes (T cell is rarer)
- Many don’t need treatment
- Bone Marrow Failure is cause of death (infection)
- Anaemia is due to BMI, hypersplenism or autoimmune haemolysis
- Prognosis relates to staging
- CT scan required to stage disease
What’s the treatment for chronic lymphocytic leukaemia?
• Early stage – Watch/wait
CLL continued
– No survival benefit for treatment of early slowly progressive disease
• Symptomatic Treatment in Progressive Disease
– Oral & IV combination chemotherapy
• Other
– Prednisilone – auto immune phenomena – Immunoglobulin Replacement – Antiobiotic Prophylaxis – Blood Products
– Splenectomy
– Monoclonal antibodies • NHL transformation
– Chemotherapy
• Stem Cell Transplantation
What are the staging systems for chronic lymphocytic leukaemia?
Rai and Binet staging systems .
What does a stem cell transplant do?
• Helps body recover after high dose chemotherapy
• Allogenic (Anthony Nolan BMT) – Consider signing up
• Autologous (your self)
• Destroys remaining Marrow, Cancer & Immune
system
• Replacewiththetransplant
• Hope it works / Supportive therapy
What is the dental relevance of leukaemia?
• Gingival Hyperplasia • Infection (mucosal) – bacterial / fungal / viral • Hyposalivation (6/day) • Odontogenic infection
– Err on side of extractions
– No pulpotomy / ectomy of primary teeth extract • Extraction timings
• Platelets • Antibiotic Coverage • Remove Orthodontic Appliances • Scale & Polish • Dietary Analysis & Prevention • During neutropenia – sterile water • Regular Review
• Dental & Craniofacial growth can be affected by chemo/radio therapy – Short thin tappered roots / hypo mineralisation
• ASK FOR ADVICE /HELP FROM HOSPITAL CONSULTANTS
• Platelets above 50 • White Cells above 2 (Absolute Neutrophil Count above 1)
