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Leukaemias (Lymphoid (Acute (Acute Lymphoblastic Leukaemia (ALL) (Tx…

- - - - uncontrolled proliferation of B and T cell blastomas, marrow failure and tissue infiltration
      - Combination of genetic susceptibilities and an environmental trigger ( xrays, pregnancy and down syndrome)
      - Most common cancer in childhood. Rare in adults
      - CNS involvement is common
      - Signs and symptoms
        
        Infiltration: hepato and splenomegaley, lymphadenopathies (superficial or mediastinal), orchidomegaly, CNS (CN palsy, meningism)
        
        Common Infections: chest, mouth, perianal and skin. bacterial septicemia, zoster, CMV, measles, candidiasis, pneumocystis pneumonia (PCP HIV one)
        
        Marrow failure: decrased Hb, decreased WCC and decreased platelets - anaemia, infection and bleeding
      - Classification
        
        Immunological: Precursor B ALL, B ALL or T ALL
        
        Cytogenic: 85% have detecatable issues : translocations. good to detect prognosis and recurrence
        
        Morphological - L1,L2,L3
      - Tests: CXR/CT: lymphadenopathy - medistinum/abdo. Blood film: blast cells, WCC usually high. LP to determine CNS involvment
      - Tx
        
        Infections: neutropenia can be caused so inf are very serious. IV antibioits. Neutropenia regime ( proph antivirals, antifungals, antibiotics but may worsen neutropenia)
        
        Matched marrow transplant : once in first remission
        
        Transfusions, IV fluids, Allopurinol (prevents tumor lysis syndrome)
        
        chemo
        
        Consolidation
        
        CNS prophylaxis
        
        Remission Induction
        
        Maintenance
      - Prognosis: cure rates 70-90% for children. 40% if adults (imatibin/rituximab increase chances)
        
        Poor: adult, male, philidelphia chromosome, CNS signs, decreased HB or WCC. B Cell ALL. high PCR. relapse in Ph-ve.
  - - - Accumulation of Mature B cells that ave escaped cell death
      - most common leukaemia, males twice as often. pneumonia may be a triggering event
      - Rai staging:
        
        Stage 1: Lymphocytosis +lymphadenopathy - 8yrs
        
        Stage 2: lymphocytosis + hepato/splenomegaly - 5 yrs
        
        Stage 0: Lymphocytosis alone - median survival 13 yrs
        
        Stage 3: Lymphocytosis + anaemia <110g) 2 yrs
        
        Stage 4: Lymphocytosis + platelers <100 - 1 yr
      - Complications
        
        Autoimmune Heamolysis. warm autoimmune haemolytic anaemia in 10-15% of patients
        
        infection due to decreased IgG. bacteria, viral (herpes zoster) hypogammaglobulinaemia
        
        Marrow Failure
      - Natural Hx: status quo for a long time . slow progression, death due to infection (pneumococcus, Haemophilus, meningococcus, candida or aspergillus) or transformation to aggressive lymphoma (Richter's syndrome)
      - Symptoms: often none. may be anaemic and infection prone. If severe, wt loss, sweats, anorexia
      - Tests: increased lymphocytes, Later: autoimmune, haemolysis, marrow infiltration: decreased Hb, neutrophils and platelets. blood film: smudge cells (also known as smear cells)
      - Signs: enlarged rubbery lymph nodes, non-tender. Splenomegaley, hepatomegaly
      - Prognosis
        
        1/3 Progress Slowly
        
        1/3 Progress actively
        
        1/3 Never Progress
- - - - neoplastic neoplasm of blast cells
      - More common in adults
      - Rapid progression: death in 2 months if untreated. 20% survival rate for 3 yrs
      - this condition can be a long term effect of chemo
      - associatied with Myelodysplastic syndromes
        
        Myelodysplastic syndromes
      - Classification
        
        AML with recurrent genetic abnormalities
        
        MAL multiliniage dysplasioa (eg. secondary to pre-exsisting myelodysplastic syndrome
        
        AML treatment related
        
        AML, other
        
        acute leukaemias of ambiguous lineage ( both myeloid and lymphoid phenotype)
  - - - uncontrolled clonal proliferation of myeloid cells
      - 15% of Leukaemias
      - Myeloproliferative Disorder
      - Philadelphia Chromosome in >80% of cases. Ph -ve is worse prognosis