Haematology Flashcards
What are the most common childhood malignancies?
1 in 500 by 15 o Leukaemia – 32% o Brain and spinal tumours – 24% o Lymphomas – 10% o Neuroblastoma – 7% o Soft tissue sarcomas – 7% o Wilms tumours – 6% o Bone tumour – 4% o Retinoblastoma – 3% o Others – 7%
What are the sign sand symptoms of ALL?
Peak at 2-5yrs Malaise, anorexia Anaemia- pallor, lethargy Neutropenia- infection Thrombocytopenia- bruising, petechiae, nose bleeds Bone pain Hepatosplenomegaly Lymphadenopathy, superior mediastinal obstruction CNS- headaches, vomiting, nerve palsies Testes- testicular enlargement
What are the investigations for ALL?
FBC- low Hb, thrombocytopenia and evidence of circulating leukaemic blast cells
• Bone marrow examination is essential to confirm the diagnosis and to identify immunological and cytogenetic characteristics which give useful prognostic information
CXR- identify a mediastinal mass characteristic of T cell disease
immunological phenotyping further subclassifies ALL
o Common subtype- 75%
o T-cell subtype- 15%
What are the induction medications in ALL?
Vincristine
Steroid (dex)
L-asparaginase
Intrathecal methotrexate
What are the consolidation and CNS protection medications in ALL?
IT methotrexate
Vincristine
Steroid
Thiopurine
What are the interim and maintenance therapy medications for ALL?
Up to 3 years
Monthly vincristine and pulsed (5 days) steroid (dex)
Daily 6-mercaptopurine
Weekly oral methotrexate
Prophylactic co-trimoxazole- pneumocystis carinii pneumonia
IT methotrexate
What are the delayed intensification medications for ALL?
Vincristine Dex Doxorubicin L-asparaginase IT methotrexate Cyclophosphamide Cytarabine
How can blood transfusions reduce symptoms in ALL?
Platelets and whole red cells
Reduce anaemia and breathlessness
Reduce bleeding and bruising
What is tumour lysis syndrome?
metabolic derangements cause by the systemic and rapidly release of intracellular contents as chemotherapy destroys leukaemic blast cells
hyperuricaemia, hyperphosphataemia,hypocalcaemia and hyperkalaemia
to prevent complications electrolyte and uric acid levels should be monitored along with IV fluid therapy
Allopurinol
What is required during remission induction in ALL?
Transfusion of RBC and platelets and infection treated
• Additional hydration and allopurinol (or urate oxidase when the white cell count is high and the risk is greater) are given to protect renal function against the effects of rapid cell lysis
• Four weeks of combination chemotherapy is given and current induction treatment schedules achieve remission rates of 95%.
What is used in treatment of relapse?
• High-dose chemotherapy, usually with total body irradiation (TBI) and bone marrow transplantation, is used as an alternative to conventional chemotherapy after a relapse.
What are poor prognostic factors in ALL?
Age- <1 or >10
WCC (tumour load): >50x109/L
Cytogenetic/molecular genetic abnormalities in tumour
cells: MLL rearrangement, t(4;11). Hypodiploidy (<44
chromosomes)
Speed of response to initial chemotherapy: Persistence of leukaemic blasts in the bone marrow
Minimal residual disease assessment (MRD): High
How are tumour cells in ALL classified?
o L1 – small uniform cells
o L2 – large varied cells
o L3 – large varied cells with vacuoles
How do different forms of ALL require different treatments?
- With a T-cell ALL the addition of cyclophosphamide and intensive treatment with asparaginase is beneficial
- Mature B-cell needs treating like a lymphoma with short-term intensive chemotherapy including high dose methotrexate.