Leukaemia: ALL, AML Flashcards
Pathophysiology of leukaemia?
Genetic mutation in one of the precursor cells in the bone marrow leads to excessive production of a single type of abnormal white blood cell.
Excessive production of the single type of cell can suppress other cell lines, which can lead to underproduction of different cell types.
E.g.
Pacytopenia = low RBCs (anaemia), low WBCs (leukopenia), and low platelets (thrombocytopenia)
What is leukaemia?
Refers to a cancer of a particular line of stem cells in the bone marrow, which causes unregulated production of a specific type of blood cell, mainly the white blood cells.
What are the four types of leukaemia?
- AML (acute myeloid leukaemia)
- CML (chronic myeloid leukaemia)
- ALL (acute lymphoblastic leukaemia)
- CLL (chronic lymphocytic leukaemia)
What is ALL (acute lymphoblastic leukaemia)?
Refers to a haematological malignancy characterised by the uncontrolled proliferation of IMMATURE LYMPHOID precursor cells in the bone marrow.
Leads to bone marrow failure and increased presence of LYMPHOBLASTS in the peripheral blood, disrupting the normal production of blood cells.
Who is commonly affected by ALL (acute lymphoblastic leukaemia)?
Commonly affects children under 5 and older adults (peaks at 35 AND 80-84).
Associated with Down’s syndrome.
How does ALL (acute lymphoblastic leukaemia) present?
Young child
B symptoms:
- weight loss (>10% in 6 months)
- fever
- night sweats
- painless lymphadenopathy
- hepatosplenomegaly
Marrow Infiltration:
- anaemia: fatigue, lethargy, SOB, pallor
- thrombocytopenia: easily bleeding or bruising, petechiae
- pain in joints and bones (more common in ALL)
CNS involvement (common in ALL than AML)
Testicular infiltration (painless unilateral testicular enlargement)
Progression over days to weeks
Investigations for ALL?
FBC (leucocytosis)
Blood film (large lymphoblasts with nucleoli and little cytoplasm and no granularity)
LFTs
U&Es
Bone marrow biopsy
- blast count (>20% blasts in bone marrow for diagnosis)
- immunophenotyping (distinguish between T or B cell)
- molecular studies
- cytogenics
Treatment for ALL?
Supportive tx:
- high risk of neutropenic sepsis
- blood transfusion
Steroids
Chemotherapy (induction and maintenance)
CNS prophylaxis
What is AML (acute myeloid leukaemia)?
Refers to a haematological malignancy characterised by the uncontrolled proliferation of MYELOID precursors in the bone marrow.
Leads to bone marrow failure and the accumulation of IMMATURE WBCs (blasts) in the peripheral blood.
Presentation of AML?
B symptoms:
- weight loss (>10% in 6 months)
- fever
- night sweats
- painless lymphadenopathy
- hepatosplenomegaly
- gum hypertrophy
Marrow Infiltration:
- anaemia: fatigue, lethargy, SOB, pallor
- thrombocytopenia: purpura, petechiae
- pain in joints and bones
Leukostasis/Leukocytosis
Progression over days to weeks.
What is leukostasis?
Excessive accumulation of WBCs, affected by acute leukaemia.
Associated with:
- very high WCC
- respiratory failure -SOB, hypoxia, intracranial haemorrhage
- CNS involvement -visual changes, headache, dizziness, tinnitus, confusion, increased risk of intracerebral brain hemorrhage
- it can affect any organ
MEDICAL EMERGENCY
Investigations for AML?
FBC
U&Es (assess tumour burden)
LFTs (assess tumour burden)
Blood film
Bone marrow biopsy (DIAGNOSIS)
- hypercellular marrow
- blasts >50%
- Sometimes Auer rods
Cytochemistry
- Sudan black stains lipid material in myoblasts
- Nonspecific esterase stains monocytic variants.
Immunophenotyping -distinguish AML from ALL.
Management of AML?
Supportive care
- infections (prophylactic antimicrobials)
Chemotherapy
- intensive, lasts 5-10days
- induction: removes bulk of leukaemic cells
- consolidation: removes residual disease
- +/- bone marrow transplant
Cytoreduction in leukostasis
- hydroxcycarbamide
Aim for complete remission
- relapse rate 50-70%
Complications of AML?
Infections (due to neutropenia)
Bleeding (due to thrombocytopenia)
Organ infiltration
Tx-related toxicities:
Myelosuppression
Increased risk of secondary malignancies.
Cardiorespiratory complications
Endocrine dysfunction
Infertility
Avascular necrosis of the hip – due to prolonged steroid exposure
Neuropsychological effects
