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HAEMATOLOGICAL MALIGNANCIES (LYMPHOMAS (NON-HODGKINS (B Cell 80% (DLBL 3…

- - - - DLBL
        
        3
        
        BCL 6
      - FOLLICULAR
        
        18/14
      - MARGINAL ZONE
      - MALT
        
        11
      - BURKITT'S
        
        8 MYC
      - SLL/CLL
      - MANTLE
        
        11
- - - - CLL
        
        TREATMENT
        
        MAB CD20 INHIBITORS
        
        RITUXIMAB/MABTHERA
        +
        
        FLUDARABINE
        
        CYCLOPHOSPHOMIDE
        
        TARGETED
        
        IBRUTINIB
        
        VENETOCLAX
        
        CAR-T TREATMENT
        CHIMERIC ANTIGEN RECEPTOR
        
        FOR IBRUTINIB RESISTANCE
        
        GOALS
        
        TREAT SYMPTOMS
        
        NOT TO CURE
        
        IMMUNOSUPPRESSION
        
        CLINICAL PRESENTATION
        
        MILD SPLENOMEGALY
        
        ENLARGED NODES
        
        SUGGESTIVE FEATURES
        
        'SMUDGE' CELLS ON FILM
      - CML
        
        FEATURES
        
        BCA-ABL GENE
        
        PHILADELPHIA CHROMOSOME 9:22
        
        SPECIFIC TRANSLOCATION
        
        85±5% LONG TERM CURE RATE
        
        CLINICAL PRESENTATION
        
        TREATMENT
        
        TYROSINE KINASE INHIBITORS
        
        eg. IMATINIB (SUFFIX -NIB)
        
        COMPLICATIONS
        
        ASSOCIATED WITH BASOPHILIA
        
        PROGRESSION TO:
        
        AML
        
        ALL
        
        PROGRESSION
    - - ALL
        
        TREATMENT
        
        CHEMOTHERAPY
        
        INTENSIVE
        
        6-8 MONTHS
        
        OTHER
        
        CNS PROPHYLAXIS
        
        ?STEM CELL (UNCERTAIN ROLE)
        
        ALLOGENIC TRANSPLANT FOR HIGH RISK
        
        DETERMINING TREATMENT
        
        MINIMAL RESIDUAL DISEASE (MDR) ASSAYS
        
        PROGNOSIS
        
        85% CURE RATE
        
        45% LONG TERM
        
        EPIDEMIOLOGY
        
        AGES 3-8
        
        HIGHER INCIDENCE IN DOWN SYNDROME
      - AML
        
        GENERAL TREATMENT
        
        ICE
        
        IDARUBICIN
        
        CYTOSINE ARABINOSIDE
        
        ETOPOSIDE
        
        FLT3 INHIBITORS
        
        M3 TREATMENT
        
        ATRA (RETINOIC ACID)
        
        COMPLICATION:
        
        ATRA SYNDROME
        
        HIGH RISK
        
        ALLOGENIC TRANSPLANT
        
        FEATURES
        
        CONSIDERED A MYELOID NEOPLASM
        
        WITH MPN & MDS
        
        PATHOGNOMONIC FEATURE
        
        AEUR RODS
    - - MPN
        "CALMLY take your PET to the V ET, MY FRIEND"
        
        CML SEE CHRONIC SECTION
        
        EXCESSIVE WBC:
        
        ESP. BASOPHILIA
        
        POLYCYTHAEMIA VERA
        RRRRRR, BS! POLY VERA CRACKER?
        
        EXCESSIVE RBC
        
        BREAKDOWN CAN CAUSE GOUT
        
        RED SKIN
        
        BLURRY VISION + HEADACHE
        
        SPLENOMEGALY
        
        PRURITIS (ESP. AFTER HOT SHOWERS)
        
        VTE (MOST COMMON CAUSE OF BUDD CHIARI)
        
        CAUSES:
        
        JAK2 MUTATION
        
        1° & 2°
        
        IMPORTANT TO DISTINGUISH
        
        ESSENTIAL THROMBOCYTHAEMIA
        
        EXCESSIVE PLATELETS
        
        MYELOFIBROSIS
        
        BONE MARROW REPLACED WITH SCAR TISSUE
        
        BLOOD FILM SHOWING
        
        TEARDROP RBC CELLS
        
        ALSO ASSOCIATED WITH JAK2 MUTATIONS
        
        TREATMENT
        
        HAVA
        
        HYDROXYUREA
        
        ANTICOAGULANTS
        
        VENESECTION
        
        ANTIPLATELETS
        
        CLINICAL
        
        SPLENOMEGALY
        
        ABN. FBC
        
        THROMBOSIS
        
        BLEEDING
      - MYELODYSPLASIA SYNDROMES
        
        AGES BETWEEN 50-70
        
        ANAEMIA
        
        HYPERCELLULAR BONE MARROW
        
        PERIPHERAL CYTOPENIAS
        
        BLAST CELLS <20%
        
        MAY PROGRESS TO AML
        
        BLAST CELLS >20%
        
        POOR PROGNOSIS
        
        CAUSES DEATH:
        
        HAEMORRHAGE
        
        INFECTION
        
        PROGRESSION
    - - ASSOCIATIONS:
        
        AUTOIMMUNE
        
        HAEMOLYTIC ANAEMIA
      - TYPES
        
        CIRCULATING LYMPHOMA
        
        CLL
        
        HAIRY CELL LEUKAEMIA
        
        PROLYMPHOCYTIC LEUKAEMIA
      - DIAGNOSIS
        T & B CELL ANTIGENS IMPORTANT
        
        BLOOD FILM
        
        FLOW CYTOMETRY