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Chapter: Medicine Study Notes : Haematology and Immunology

Acute Leukaemia

Rapid onset, 100% mortality within 3 months if untreated

Acute Leukaemia

 

·        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 

Treatment

 

·        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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