Leukemia Flashcards
Acute leukaemias - general description
Neoplastic processes of BM, acute - rapidly progressive, immature blasts > 20% BM cells
Features - pancytopenia & organ infiltration
ALL: childhood
- In addtion to pancytopenia & infiltration what features might you see (3)
- Dx (1)
- Treatment: remission, consolidation, maintenance
Features
- LNpathy
- CNS involvment
- testicular enlargement
- Thymic enlargement
Dx - High WCC, lymphocytes/ precursors +++
Rx - Remission induction: chemo + steroids, consolidation: High dose multi drug chemo, maintenance: 2 yrs (F), 3 yrs (M). CNS treatment
Consider allogenic SCT
AML: adulthood & under-2s
Subtypes:
- M3 - prone to what?
- M4&5
Ix (2)
Rx - similar to ALL but no CNS propjylaxis/ maintenance needed
- M3 - acute promyelocytic leukaemia - prone to DIC
- M4+5: monocytes/monoblasts > skin/gum infiltration + hypokalaemia
Ix - High WCC, Auer rods. myeloperoxidase & Sudan black B stains
Rx -ATRA for M3, prognosis worse with age
CML: O/E what is often noted?
Ix (3)
A myeloproliferative disorder affecting middle-aged
O/E - massive splenomegaly
Ix-
- Ph+ve in 80% t(9:22)
- PCR for BCR-ABL fusion gene
- often dx on routine bloods - large no. of neutrophils
- Hypercellular BM with immature & mature granulocytes in blood
CML can be categorized into 3 phases depending on % of blast cells:
- Chonic phase
- Accelerated phase
- Blast phase
Chronic phase
- <5% blast cells, WBC increase slowly ocer yrs
- Rx: hydroxyurea/ interferon supress WCC
- imatinib (BCR-ABL TK inhibitor) very effective
- BMT curative
Accelerated pahse
- >10% blasts
- Increasing manifestations e.g. splenomegaly, less responsive to therapy
Blast phase
- >20% blast cells - like AML
- Get B symptoms, treatment similar to AML - consider SCT
CLL: epidemiology
Clinical features (4)
Ix (3)
A lymphoproliferative disease of the elderly, M>F
F
- Asymp
- symmetrical painless LN pathy
- B symptoms
- BM failure - pancytopenia
- Assc with autoimmunity - ITP, AIHA
- Richter’s transformation - can progress to form of lymphoma
Ix
- High WBC with lymphocytosis; small & mature
- low serum Ig
- Smear CLLs (smear cells on blood film)
Good (3) & bad (3) prognostic factors in CLL:
Good: low zap-70, hypermutated Ig gene, 13q14 deletion
Bad: LDH raised, CD38 +ve. 11q23
Binnet Staging - CLL; A, B & C
Stage A - no treatment required, <3 groups of enlarged LNs
Stage B - >3 groups enlarged LNs
Stage C - anaemia/thrombocytopenia
Treatment - commenced as pt becomes symptomatic - 1st line chlorambucil, alemtuzuman (anti-CD52) & steroids. SCT