|
Leukemia
Leukemia is a group of blood diseases
characterized by malignancies (cancer) of the blood-forming tissues.
The word leukemia tends to
be used as an umbrella term. In the 19th century, it was seen as one
single, homogenous deadly disease, characterized by a white (leuko-)
appearance of blood samples. However, with growing understanding of
pathologic and cytologic processes, doctors are now able to differentiate
numerous diseases which require different treatment.
Overview
Leukemia, first recognised
by the German pathologist Rudolf Virchow in 1847, with the first case
described by British pathologist John Hughes Bennett in 1845, starts when
bone marrow cells multiply abnormally. This is caused by mutations in the
DNA in stem cells. Bone marrow stem cells produce billions of red blood
cells and white blood cells each day, respectively carrying oxygen and
fighting infection throughout the body. Leukemia is characterised by an
excessive production of abnormal white blood cells, overcrowding the bone
marrow and often spilling out into the peripheral blood. The infiltration
of the bone marrow results in decreased production and function of normal
blood cells. Leukemia, dependent on the type, can spread to the lymph
nodes, spleen, liver, central nervous system and other organs.
Types
Leukemia is a broad term
covering a spectrum of diseases. In leukemia, the diseases are classified
according to the type of abnormal cell found most in the blood. Leukemia
is also clinically split in to its acute and chronic forms. The four main
forms are:
· Acute lymphocytic leukemia (ALL)
· Acute myelogenous leukemia (AML)
· Chronic lymphocytic leukemia (CLL)
· Chronic myelogenous leukemia (CML)
The most common forms in
adults are AML and CLL, whereas in children ALL is more prevalent.
Acute leukemias are
characterised by the rapid growth of immature blood cells. This crowding
makes the bone marrow unable to produce healthy blood cells. Acute forms
of leukemia are most common in children and young adults (in fact it is a
more common cause of death for children in the US than any other type of
malignant disease). Immediate treatment is required in acute leukemias
due to the rapid progression and accumulation of the malignant cells,
which then spill over into the bloodstream and spread to other organs of
the body. If left untreated, the patient will die within months or even
weeks.
Chronic leukemias are
distinguished by the excessive buildup of relatively mature, but still
abnormal, blood cells. Typically taking months to 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. Chronic leukemia mostly
occurs in older people, but can theoretically occur in any age group.
Whereas acute leukemia must be treated immediately, chronic forms are
sometimes monitored for some time before treatment to ensure maximum
effectiveness of therapy.
Symptoms
Damage to the bone marrow
results in a lack of blood platelets, which are important in the blood
clotting process. This means people with leukemia may become bruised or
bleed excessively. Similarly, the red blood cell deficiency leads to
anemia, which may cause shortness of breath and fatigue. Bone or joint
pain may occur because of cancer spreading to these areas. Headaches and
vomiting are indicative of the cancer having disseminated to the central
nervous system. Enlarged lymph nodes or splenomegaly (an enlarged spleen)
may occur in some types. All symptoms may also be attributable to other
diseases; for diagnosis, blood tests and a bone marrow biopsy are
required.
Causes
All leukemias are due to
mutations in the DNA. Chromosomal translocations (crossing over of parts
of chromosomes to others) are common, disrupting specific genes that
mediate cell division rate. Mutations may occur spontaneously or as a
result of exposure to radiation orcarcinogenic substances. Cytogenetics
and immunophenotyping are two laboratory investigations used to determine
the type and aggressiveness of the leukemia and the necessity of urgent
and active treatment, as well as an indication of prognosis.
Viruses have also been
linked, with varying levels of speculation, to some forms of leukemia. Some
forms of T-cell leukemia have recently been confirmed to be the result of
two viruses.
In the early 1990s concern
was raised in the UK about the effect of nuclear power plants on unborn
children, when clusters of leukemia cases were discovered nearby to some
of these plants. The effect was speculative because clusters were also
found where no nuclear plants were present, and not all plants had
clusters around them. Using statistical analysis researchers at
Southampton University concluded that a link was present, deducing that
radiation damage to men working at the plants had caused genetic
abnormalities in their children. After this report British Nuclear Fuels
initially advised workers who were being exposed to high levels of
radiation not to father children, although they have since withdrawn this
advice.
Treatment
The mainstay of treatment
of leukemia is chemotherapy, sometimes with the addition of radiation
therapy. Because of the severity of some courses, bone marrow transplants
are sometimes necessary. Healthy bone marrow transplanted in to the body
helps rebuild tissue damaged by the treatment.
Prognosis
27,900 adults and 2,300
children are diagnosed each year with leukemia in the US. Over the last
thirty years, the chances of survival have doubled from a 22 per cent
survival rate in 1970 to 43 per cent rate in the 1990s. There is however
a wide range in prognosis amongst the different types of leukaemia: the
outlook for an elderly patient with AML remains very poor, whilst 8 out
of 10 children with ALL will now be cured.
|