· Rapid onset, 100% mortality within 3 months if untreated
· Very undifferentiated (anaplastic) cells: blasts, no normal cells in blood
· Types:
o Acute Myeloblastic Leukaemia (AML). Chance of cure with chemo alone = 20 – 40%. With transplant = 60%. Has Auer rob in blast
o Acute Lymphoblastic Leukaemia (ALL)
· Presentation:
o Tired due to anaemia, breathless
o Bleeding due to ¯platelets, nose bleeds
o Bacterial infection
o Hepatosplenomegaly, lymph nodes, bone pain (push on sternum)
· Investigations:
o FBC: ¯Hb, ¯platelets, white count: High, normal or low (sometimes leukaemia cells stay in marrow)
o Bone marrow: > 30% of nucleated cells in the marrow are leukaemic blasts
· Classification:
o Cytochemistry:
§ Staining. PAS - +ive stain for glycogen Þ lymphoblastic
§ Sudan black +ive for peroxidase Þ myeloblastic
o Immunology: flow cytometry
o Cytogenetics
· Supportive Care:
o Antibiotics, platelet/RBC transfusion
o Venous catheter: Hickman catheter
· Cytotoxic Treatment:
o Complex multi-drug protocols
o Remission induction: 1-4 weeks depending on protocol. FBC normal and < 5% blasts in marrow (that‟s normal). AML – achieved in 70 – 80%. ALL – achieved in 70 – 80% of adults, 95% of kids
o Consolidation: more drugs to mop up residual blasts, including CNS prophylaxis (some drugs don‟t penetrate CNS well)
o But 60 – 80% chance of relapse over next 2 – 4 years
· Bone Marrow Transplantation:
o = Haematopoietic stem cell transplantation
· Kill of leukaemic cells with dose: but limited by marrow toxicity. With marrow transplantation can push dose higher (limit is organ toxicity) if cancer is responsive
· Process: patient and donor preparation, conditioning (chemo & high does radiation), stem cell infusion, neutropenic phase, post neutropenic phase
· Sources of stem cells: Self (autologous), twin (syngenic), HLA matched sibling (allogenic), HLA partial matched sibling, matched unrelated donor (MUD)
· Peritransplant mortality = 20%
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