Leukaemia: Is a cancer of the blood or bone marrow and is characterized
by an abnormal
proliferation
(production by multiplication) of blood cells, usually white blood cells
(leukocytes).
Leukemia
is clinically and pathologically subdivided into several large groups. The
first
division
is between its
Acute
leukemia
Chronic
leukemia
Acute Leukaemia
Acute
leukemia is characterized by the rapid increase of immature blood cells or blasts
in
the bone marrow
This
crowding makes the bone marrow unable to produce healthy blood cells
Immediate
treatment is required in acute leukemia due to the rapid progression and
accumulation
of the malignant cells, which may then spill over into the bloodstream and
spread
to other organs of the body
Death
usually occurs within 6 months in most patients
Acute
forms of leukemia are the most common form of leukemia in children.
Chronic Leukaemia
Chronic
leukemia is distinguished by the excessive build-up of relatively mature, but
still
abnormal,
white blood cells.
Typically
taking months or years to progress, the cells are produced at a much higher
rate
than
normal cells, resulting in many abnormal white blood cells in the blood.
Compared
to acute leukemia which must be treated immediately, chronic forms are
sometimes
monitored for some time before treatment to ensure maximum effectiveness of
therapy.
Chronic
leukemia mostly occurs in older people, but can theoretically occur at any age
group.
Acute and chronic leukemia are further classified according to their cell
lineage
into two major categories, namely
Myeloid
Lymphoid
Thus
acute leukemia may either be
Acute
myeloblastic leukemia (AML) or
Acute
lymphoblastic leukemia (ALL)
Chronic
leukemia may either be
Chronic
myeloid leukemia (CML) or
Chronic
lymphocytic leukemia (CLL)
Four
Major Kinds of Leukaemia
Cell Type Acute Chronic
Lymphocytic
leukemia
(lymphoblastic)
Acute
lymphoblastic
leukemia
(ALL)
Chronic
lymphocytic
leukemia
(CLL)
Myelogenous
Leukemia
(Myeloid)
Acute
myeloblastic
leukemia
(AML)
Chronic
myeloid leukemia
(CML)
Risk Factors and Epidemiology of
Leukemia
Risk Factors
In
majority of patients the causes of leukemia is unknown
Several
factors are however associated with the development of leukemia and these
include
Ionizing
radiation
Cytotoxic
drugs
Exposure
to benzene in industry
Retroviruses-
human T-lymphocytic virus
Genetic
Immunological
causes
Epidemiology
Acute
leukemia occur at all ages
Acute
lymphoblastic leukemia (ALL) shows a peak of incidence in the 1-5 age group
All
forms of acute myeloid leukemia (AML) have their lowest incidence in young
adult
life
and there is a striking rise over the age of 50 years.
Chronic
leukemia occurs mainly in middle and old age
Clinical Features of Leukaemia
Clinical
features manifest due to
Infiltration
& replacement of normal bone marrow with leukemic cells resulting into
Anemia
Neutropenia
Thrombocytopenia
Infiltration
of other tissues & organs with leukemic cells-liver spleen and lymph
nodes.
Increased
metabolic rate when the tumor mass is large.
Patients
with anemia may present with
Easy
fatigability
Shortness
of breath
Palpitation
Weakness
Lassitude
Patients
with neutropenia may be prone to bacterial infections (particularly pneumonia
&
septicemia)
from:
Gram
negative bacteria such as
Escherichia coli
Klebsiella spp
Pseudomonas auregenosa
Gram
positive bacteria such as
Staphylococcus aureus
Staphylococcus epidermidis
Patients
with leukemia may also have decreased immunity that may predispose them to
opportunistic
infections (OIs) such
Fungal
infections (e.g. candidiasis and aspergillosis)
Pneumocystis
pneumonia (PJP/PCP)
Herpes
simplex
Herpes
zoster (shingles)
Patients
with thrombocytopenia may present with
Petechiae
hemorrhages
Epistaxis
(bleeding from the nose)
Bleeding
from the gums
Spontaneous
bruising
Eye
& internal ear-bleeding (depends on the extent of the disease)
Leukemia
may also lead to increased basal metabolic rate (BMR) that may manifest with
excessive
sweating and loss of weight.
Clinical Features of Acute
Leukaemia (ALL & AML)
Patients
are usually ill-looking
Symptoms
and signs develop over days or a couple of weeks. The presentation may
include
the following
Pallor
Fever
Various
infections
Abnormal
bleeding
Painful
& tender bones especially in ALL
Hepatosplenomegaly
(enlargement of liver and spleen)
Enlargement
of lymph nodes in ALL is not uncommon
Meningeal
infiltration (mostly in ALL) leads to
Headache
Nausea
Vomiting
Visual
disturbance
Clinical Features of Chronic
Leukaemia (CLL & CML)
Patients
with chronic myeloid leukemia (CML) may have the following features
Tiredness
Weight
loss
Breathlessness
Abdominal
pain & discomfort in about 90% of patients
Lethargy
Anorexia
Night
sweating
Abdominal
fullness
Bruising,
low-grade fever, & palpitation
The principal clinical finding is splenomegaly in 90%, and about 10% have massive
splenomegaly
Hepatomegaly
in about 50% of patients
About
25% of patients are asymptomatic at diagnosis
Note:
Lymphadenopathy is unusual
Clinical Features of Chronic
Lymphocytic Leukaemia (CLL)
This
form occurs in about 30% of patients
Its
onset is very insidious
In
70% of patients, the diagnosis of CLL is made incidentally
The
clinical features include
Anemia,
neutropenia & thrombocytopenia
Disturbance
of both humoral and cellular immunity (decreased antibodies & cell
mediated
immunity)
Sometimes, the autoimmune phenomenon may develop
Patient
may present with
Loss
of energy
Tiredness
Shortness
of breath
Infections
at time of diagnosis but often occur later
Hepatosplenomegaly
Painless
lymphadenopathy(enlarged lymph nodes)
Differential Diagnosis,
Investigations and Treatment of Leukaemia
Differential Diagnosis
• Lymphomas
• Secondary
malignancies from the breast, stomach, or lungs
Investigation
• The
investigations for leukemia may be done and interpreted correctly by experts
in the
capable
hospitals and therefore referral of patients suspected of having leukemia is
necessary.
• The
investigations that may be done include:
Blood
count reveals the following;
Low
Hemoglobin (HB)
Raised
or low white blood cell counts (WBC)
Low
Platelets
Blood the film will show blast cells or lymphocytes
Bone
marrow aspirate (biopsy) will show infiltration of immature white blood cells
Treatment
Treatment
of leukemia cannot be performed in the primary health care facilities
(dispensary
and health centers) therefore patients must be referred to hospitals for proper
treatment.
However,
pre-referral treatment should be offered to ensure that the patient reaches the
hospital
safely
IV
fluids when there is a fluid deficit
Antibiotics
when there is an infection
Counseling
and psychological support to the patient as well as relatives
Described
below are treatment options that can be achieved in capable hospitals.
Acute Leukaemia
Specific
therapy aims at destroying leukemic clone cells without destroying the residual
normal
stem cells.
The
main treatment option in the treatment of acute leukemia is chemotherapy
Drugs
that may be used include
Vincristine
Prednisolone
Daunorubicin
Etoposide
Cytarabine
Methotrexate.
There
are three phases namely
Induction
Consolidation
Maintenance
Another treatment option is allogeneic bone marrow transplantation
Chronic Leukaemia
Depends
upon the stage of the disease, the treatment of chronic leukemia may range
from
nontreatment to chemotherapy and chemotherapy combined with radiotherapy
Other
supportive therapy includes correction of anemia, bleeding tendency and
control
of infections.
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