Hematological Flashcards
Physiology of Blood
Transportation - transports oxygen from lungs to cells and carbon dioxide from cells to lungs
Regulation - homeostasis of all body fluids
Protection - blood can clot, which protects against excessive blood loss
Components of blood
Blood plasma and formed elements
Types of blood
Red - contain haemoglobin to carry oxygen
Platelets - assists in clotting
White blood cells - fights infections, disease and foreign bodies 2 main types - neutrophils and lymphocytes
WBC Function
Monocytes - in blood are mobile and phagocytic, others become macrophages
Lymphocytes - t cell responsible for immunity and B cell with antibody production
Granulocytes - neutrophils protect against foreign materials
Hematological malignancies
Leukaemia’s
Multiple Myeloma
Polycythaemia Rubra Vera
Leukaemia types
ALL - Acute lymphoblastic leukaemia (common in children)
AML - Acute Myeloid leukaemia (mainly adults but can occur in children and adolescents)
CLL - Chronic lymphocytic leukaemia (affects adults)
CML - Chronic myeloid leukaemia (can occur at any age but uncommon below age of 20)
Leukemia and RT
Treating leukaemia that has spread to CNS
Or to the testicles
to treat symptoms caused when swollen internal organs, such as the spleen, press on other organs
May be used before stem cell transplant
Used to relieve bone pain
Treatment for Leukemia depends on
the stage of the disease • the location of the cancer • the severity of symptoms
Standard treatment for acute leukaemia
• Remission induction (3-8 weeks) - no abnormal leukaemic cells
• Consolidation (Intensification of treatment to kill resistance cells, can last 6-9months)
• Maintenance (continuing treatment) - low dose treatment
Disease Classification
Classification based on age, white cell count and cryogenetics of the leukaemia cells
Standard risk - majority of ALL patients
High Risk - all adolescents and younger children with higher leukocyte counts at presentation
Very High Risk - 1-2% with B cell
Special risk patients - Philadelphia chromosome or slow response to induction treatment
ALL Epidemiology and cause
Bone marrow makes too many lymphocytes, gets worse quickly
40% occurs between 2-5 years
Male/female 1:1
Less common than AML
ALL Aetiology
Down syndrome
Environmental agents - viruses
Radiation exposure
ALL Signs and symptoms adults
Peripheral lymphadenopathy
Splenomegaly
Liver palpable
Bone marrow failure - infection due to leucopoenia, bruising and bleeding
Petechiae (flat, pinpoint spots under skin caused by bleeding)
Fever
Shortness of breath
Loss of appetite or weight loss
ALL signs and symptoms Children
oral and pharyngeal ulceration, anaemia, infection, bone pain, few weeks of malaise, fever , haemorrhages in eye, lymph node enlargement, usually pale, bones tender. Meningeal involvement if: headache, stiff neck, vomitting
Adverse prognostic features in childhood ALL
Adverse cytogenic markers, CNS disease, early marrow relapse, testicular relapse, t-cell phenotype, philadelphia chromosome
Above 12yrs, prognosis worse (35% in adults) and 70% in children
Total white cell count - more than 20000 poorer prognosis
Diagnosis for ALL
Physical exam and patient history
Full blood count
Peripheral blood smear
Cytogenic analysis
Erythrocyte sedimentation rate
Immunophenotyping
Liver function
Radiography
Bone marrow biopsy and aspiration
ALL Clinical Management - children
Curative
Multidrug therapy
CNS targeted treatment - common as chemo cannot enter this area due to barrier
Remission Induction - Vincristine, Doxorubicin
Consolidation - Methotrxate - drugs are myelosuppressive
Maintenance - Methotraxate - 2 years, any lower -> increased relapse
87% survival at 5 years
CNS Prophylaxis - intrathecal methotrexate, cranial irradiation
ALL treatment Side Effects
Low neutrophil and platelet count - infections, bleeding
Bone marrow suppression
Hair loss
Kidney damage - increase fluid intake
ALL RT options and dose
Cranial Irradiation
Opposing lateral fields
18Gy in 8-10#
More recently 12Gy
Testicular relapse
24-26Gy in 12-13#
Child in frog-leg position with soles of the feet touching
ALL Relapse
20-25% will relapse
High risk disease -> higher rate of relapse
Relapsed ALL more resistant to treatment than original disease -> leukaemic cells become drug resistant
ALL Relapse Treatment
Repeat remission induction programme
Increased intensity
ALL Adult clinical management
Remission Induction - more intense to children
Consolidation
Maintenance - testicular relapse not common, cranial irradiation not given
ALL Prognostic factors for Adults
Age over 60 years
Late achievement of complete response
B-cell ALL
Philadelphia chromosome
AML epidemology
Rare- accounts for 0.8% of all tumpours in australia
Men slightly higher incidence
More common in adults over age of 60
3.7 in 100,000 in australia don’t survive