Haematology - Chronic Leukaemia

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Author:
trincam2008
ID:
275733
Filename:
Haematology - Chronic Leukaemia
Updated:
2014-05-31 12:35:59
Tags:
path haem CML CLL
Folders:
path
Description:
CML & CLL, intro, Ix, staging, Mx
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  1. CML (Chronic myeloid leukaemia) intro

    (what nature, age group, diag from?, o/e)
    • - A myelo-proliferative diseas
    • - Middle aged, 40-60, median survival 3-5 years
    • - Dx often from routine blood (large no of differentiated neutrophils)
    • - o/e: splenomegaly - often massive
  2. CML - Ix
    • - Ph+ve (Philadelphia chromosome) in 80% = chromosomal translocation (9,22) 
    • (con meo lang, song philadelphia, an nhieu mo, 1 qua bong bay du cho 2 con vit)
    • - PCR for BCR-ABL (Phil Ch) fusion gene
    • - Monitor disease and therapeutic response
    • - WBC, neutrophils 50 - 500
    • - Hypercellular BM
    • (spectrung of immature e.g. myelocytes and mature granulocytic cells in blood)
  3. CML - 3 phases
    (what seen in BM/blood, Rx)
    • 1. Chronic phase
    • -  <5% blast in BM/blood
    • - WBC slowly increases over years (indolent)
    • - Rx:
    •   + imatinib or dasatibin for resistance
    •   + oral hydroxyurea/interferon - suppress WCC
    •   + BMT poten. curative  

    • 2. Accelerated phase
    • - >10% blasts 
    • - Increasing manifestations, e.g. splenomegaly, lasting up to 1 year
    • - Less responsive to treatment

    • 3. Blast phase
    • - >20% blasts in BM/blood
    • - resembles acute leuk, timeframe = months
    • - Rx: oral hydroxyurea/interferon; imatinib or dasatinib/nilotinib; poss with allogenic SCT for young adults
  4. CLL - intro 

    (what nature (+ some others) , CLL and SLL)
    • - a lympho-proliferative disease (others include ALL, MM, and Waldenstrom's)
    • - CLL and SLL (small lymphocytic lymphoma) same disease process, CLL - in BM, SLL in LN
    • - M>F, elderl (65-70)
  5. CLL - clinical features
  6. CLL - Ix
    • - asymp., routine bloods
    • - symmetrical painless lymphadenopathy
    • - BM failure - anaemia & thrombocytopenia symp, recurrent infections (50% deaths)

    - Assoc with autoimmunity (Evans syndreome) - AIHA, ITP- Can progress to a form of lymphoma (DLBC) - Richter's transformation

    • - WL, low grade fever, night sweats
    • - Hepato & spleno-megaly (less prominent)
  7. CLL - Prognostic factors
    • bad
    • LDH raised, CD38 +ve, 11q23 deletion
    • (map -> LDH, 38 = muffi', tot qua 23 name)

    - good

    • Hypermutated IG gene, low ZAP-70 expression, 13q14 deletion
    • (zap lai, case tru di 13 tam tar)
  8. CLL - Binet Staging A,B,C
    • Stage A.
    • - High WBC
    • - <3 groups of enlarged lymph nodes
    • - Usu no treatment required

    • Stage B
    • - >3 

    • Stage C
    • - anaemia and thrombocytopenia
  9. CLL - treatment
    • - Commenced as pt become symtomatic - 1st line chlorambucil 
    • - Can also use: fludarabine (purin analogue), alemtuzumab (anti-CD52), and steroids
    • - SCT considered in young, fit pt

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