ACUTE
MYELOID LEUKAEMIA
is a malignant
tumour of haemopoietic precursor cells of non-lymphoid lineage, almost
certainly arising in the bone marrow. Incidence
1
in 50,000 annually. Increasing
frequency with age (median 60 years).
Cause unclear – association with heavy radiation dose exposure eg.
post-Chernobyl disaster, chronic benzene exposure, alkylating agents and
hereditary predisposition in Down’s and Fanconi’s syndromes,
pre-existing myeloproliferative disorders. Classification
– morphological (French-American-British, FAB) M0
- Undifferentiated
M1 - Early myeloblastic (minimal differentiation)
M2 - late myeloblastic (differentiation)
M3 - promyelocytic
M4 - myelomonocytic
M5 - monoblastic
M6 - erythroleukaemic
M7 – megakaryoblastic ·
Immunophenotyping
useful in diagnosis of M0, 4, 5, 6 and 7 ·
Cytogenetics
important in detecting translocations and deletions -
t(15;17) in
M3 -
t(8;21) in
M2 -
inv (16) in
M4Eo, are all associated with better prognosis -
monosomy 7
and multiple breakages are poor prognosis
·
Acute
presentation common; often critically ill. ·
Common
symptoms include malaise, sweats, anaemic symptoms (breathlessness,
faintness and palpitations) ·
Infections
– particularly chest, mouth, perianal, skin (Staph.pseudomonas, HSV,
Candida). ·
Bleeding,
purpura, menorrhagia and bleeding nose, gums, rectal, retina (especially
M3-DIC) and gum and skin infiltration (M4,M5). ·
Leucostatic
signs of hypoxia, retinal haemorrhage or diffuse pulmonary shadowing. Investigations
and diagnosis ·
FBC and
blood film. ·
Bone marrow
and biopsy. ·
Total WBC
usually with
blast cells on film – but may be low. ·
Hb. and
Plts usually ¯. ·
Marrow
heavily infiltrated with blasts 9>30%) – immunophenotyping and
karyotyping on blood and marrow allows classification as above. Emergency
treatment ·
Seek expert
help immediately ·
Cardiovascular
and respiratory resuscitation may be needed in septic shock of massive
haemorrhage. ·
Leucapheresis
if peripheral blast count high or signs of leucostasis (retinal
haemorrhage, reduced conscious level, diffuse pulmonary shadowing on CXR
or hypoxia) Supportive
treatment ·
Give
explanation and offer counselling. ·
RBC and
platelet transfusion support will continue through treatment. ·
Start
neutropenic regimen as prophylaxis. ·
Start
hydration and allopurinol PO. Specific
treatment Discuss with haematologist. |
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