CHAPTER I

INTRODUCTION

A. Background of the Study

Acute Lymphocytic Leukemia / Acute Lymphoblastic Leukemia

(ALL) is a form of leukemia, or cancer of the white blood cells characterized
by excess lymphoblasts.

Malignant, immature white blood cells continuously multiply and are

overproduced in the bone marrow. ALL causes damage and death by
crowding out normal cells in the bone marrow, and by spreading
(metastasizing) to other organs. ALL is most common in childhood with a
peak incidence at 2-5 years of age, and another peak in old age. The overall
cure rate in children is 85%, and about 50% of adults have long-term disease-
free survival.

'Acute' refers to the relatively short time course of the disease (being
fatal in as little as a few weeks if left untreated) to differentiate it from the very
different disease of Chronic Lymphocytic Leukemia which has a potential time
course of many years. It is interchangeably referred to as Lymphocytic or
Lymphoblastic (although lymphoblastic is becoming the preferred term). This
refers to the cells that are involved, which if they were normal would be
referred to as lymphocytes but are seen in this disease in a relatively
immature (also termed 'blast') state.

Symptoms
Initial symptoms are not specific to ALL, but worsen to the point that
medical help is sought. The signs and symptoms of ALL are variable but
follow from bone marrow replacement and/or organ infiltration.
  General weakness and Fatigue
 Anemia
 Frequent and unexplained fever / infection
 Weight loss and/or loss of appetite
 Excessive and unexplained bruising
 Bone pain, joint pain (caused by the spread of “blast” cells to the
surface of the bone or into the joint from the marrow cavity
 Breathlessness
 Enlarged lymph nodes, liver and/or spleen
 Pitting edema in the lower limbs and/or abdomen
 Petechiae, which are tiny red spots or lines in the skin due to low
platelet levels.

The signs and symptoms of ALL result from the lack of normal and
healthy blood cells because they are crowded out by malignant and immature
leukocytes (white blood cells). Therefore, people with ALL experience
symptoms from malfunctioning of their erythrocytes (red blood cells),
leukocytes, and platelets. Laboratory tests which might show abnormalities
include blood count tests, renal function tests, electrolyte tests and liver
enzyme tests.

Diagnosis
Diagnosing ALL begins with a medical history and physical
examination, complete blood count, and blood smears. Because the
symptoms are so general, many other diseases with similar symptoms must
be excluded. Typically, the higher the white blood cell count, the worse the
prognosis. Blast cells are seen on blood smear in 90% of cases. A bone
marrow biopsy is conclusive proof of ALL. A lumbar puncture (also known as
a spinal tap) will tell if the spinal column and brain has been invaded.

Pathological examination, cytogenetics (particularly the presence of

Philadelphia chromosome) and immunophenotyping, establish whether the
"blast" cells began from the B lymphocytes or T lymphocytes. DNA testing
can establish how aggressive the disease is; different mutations have been
associated with shorter or longer survival.

Medical imaging (such as ultrasound or CT scanning) can find invasion

of other organs commonly the lung, liver, spleen, lymph nodes, brain, kidneys
and reproductive organs.

Pathopysiology
The cause of most ALL is not known. In general, cancer is caused by
damage to DNA that leads to uncontrolled cellular growth and spread
throughout the body, either by increasing chemical signals that cause growth,
or interrupting chemical signals that control growth. Damage can be caused
through the formation of fusion genes, as well as the dysregulation of a proto-
oncogene via juxtaposition of it to the promotor of another gene, e.g. the T-
cell receptor gene. This damage may be caused by environmental factors
such as chemicals, drugs or radiation.

ALL is associated with exposure to radiation and chemicals in animals

and humans. The association of radiation and leukemia in humans has been
clearly established in studies of victims of the Chernobyl nuclear reactor and
atom bombs in Hiroshima and Nagasaki. In animals, exposure to benzene
and other chemicals can cause leukemia. Epidemiological studies have
associated leukemia with workplace exposure to chemicals, but these studies
are not as conclusive. Some evidence suggests that secondary leukemia can
develop in individuals who are treated for other cancers with radiation and
chemotherapy as a result of that treatment.

Prognosis
The survival rate has improved from zero four decades ago, to 20-75
percent currently, largely due to clinical trials and improvements in bone
marrow transplantation (BMT) and stem cell transplantation (SCT)
technology.

Five-year survival rates evaluate older, not current, treatments. New

drugs, and matching treatment to the genetic characteristics of the blast cells,
may improve those rates. The prognosis for ALL differs between individuals
depending on a variety of factors:

 Sex: females tend to fare better than males

 Ethnicity: Caucasians are more likely to develop acute leukemia than
African-Americans, Asians and Hispanics and tend to have a better
prognosis than non-Caucasians.
 Age at diagnosis: children between 1–10 years of age are most likely
to develop ALL and to be cured of it. Cases in older patients are more
likely to result from chromosomal abnormalities (e.g. the Philadelphia
chromosome) that make treatment more difficult and prognoses
poorer.
 White blood cell count at diagnosis of less than 50,000/µl
 Cancer spread into the Central_nervous_system (brain or spinal cord)
has worse outcomes.
 Morphological, immunological, and genetic subtypes.
 Patient’s response to initial treatment
  Genetic disorders such as Down's Syndrome

Epidemiology
The number of annual ALL cases in the Philippines is roughly
1000, 600 of which afflict children. At the Dr. Fe del Mundo Medical Center
(formerly Children’s Medical Center), a hospital in Manila, the Philippine
capital, fifty new cases are seen every year. 

ALL accounts for approximately 80 percent of all childhood leukemia

cases, making it the most common type of childhood cancer. It has a peak
incident rate of 2–5 years old, decreasing in incidence with increasing age
before increasing again at around 50 years old. ALL is slightly more common
in males than females. There is an increased incidence in people with Down
Syndrome, Fanconi anemia, Bloom syndrome, Ataxia telangiectasia, X-linked
agammaglobulinemia and Severe combined immunodeficiency

Treatment
The earlier acute lymphocytic leukemia is detected, the more effective
the treatment. The aim is to induce a lasting remission, defined as the
absence of detectable cancer cells in the body (usually less than 5% blast
cells on the bone marrow.

Treatment for acute leukemia can include chemotherapy, steroids,

radiation therapy, intensive combined treatments (including bone marrow or
stem cell transplants), and growth factors.

Chemotherapy
Chemotherapy is the initial treatment of choice. Most ALL patients end
up receiving a combination of different treatments. There are no surgical
options, due to the body-wide distribution of the malignant cells. In general,
cytotoxic chemotherapy for ALL combines multiple antileukemic drugs in
various combinations. Chemotherapy for ALL consists of three phases:
remission induction, intensification, and maintenance therapy.

Phase Description Agents

The aim of remission

induction is to rapidly kill
most tumor cells and get the
Combination of Prednisolone or
patient into remission. This is
dexamethasone (in children),
defined as the presence of
Remission vincristine, asparaginase, and
less than 5% leukemic blasts
induction daunorubicin (used in Adult
in the bone marrow, normal
ALL) is used to induce
blood cells and absence of
remission.
tumor cells from blood, and
absence of other signs and
symptoms of the disease.

Intensification Intensification uses high Typical intensification protocols

doses of intravenous use vincristine,
multidrug chemotherapy to cyclophosphamide, cytarabine,
further reduce tumor burden. daunorubicin, etoposide,
Since ALL cells sometimes thioguanine or mercaptopurine
penetrate the Central given as blocks in different
Nervous System (CNS), combinations. For CNS
most protocols include protection, intrathecal
delivery of chemotherapy methotrexate or cytarabine is
into the CNS fluid (termed usually used combined with or
intrathecal chemotherapy). without cranio-spinal irradiation
Some centers deliver the (the use of radiation therapy to
drug through Ommaya the head and spine). Central
reservoir (a device surgically nervous system relapse is
placed under the scalp and treated with intrathecal
used to deliver drugs to the administration of hydrocortisone,
CNS fluid and to extract methotrexate, and cytarabine.
CNS fluid for various tests).
Other centers would perform
 multiple lumbar punctures as
needed for testing and
treatment delivery.

For this purpose, daily oral

The aim of maintenance
mercaptopurine, once weekly
therapy is to kill any residual
oral methotrexate, once monthly
cell that was not killed by
5-day course of intravenous
Maintenance remission induction, and
vincristine and oral
therapy intensification regimens.
corticosteroids are usually used.
Although such cells are few,
The length of maintenance
they will cause relapse if not
therapy is 3 years for boys, 2
eradicated.
years for girls and adults.

Radiation Therapy
Radiation therapy (or radiotherapy) is used on painful bony areas, in high
disease burdens, or as part of the preparations for a bone marrow transplant
(total body irradiation). Radiation in the form of whole brain radiation is also
used for central nervous system prophylaxis, to prevent recurrence of
leukemia in the brain. Whole brain prophylaxis radiation used to be a common
method in treatment of children’s ALL. Recent studies showed that CNS
chemotherapy provided results as favorable but with less developmental side
effects. As a result, the use of whole brain radiation has been more limited.

B. Rationale of Choosing the Case

Leukemia is the foremost cancer in the children of the Philippines. It

accounts for roughly 1,000 new cases each year.
 One of the more common forms are Acute Lymphocytic Leukemia
(ALL).

At the Dr. Fe del Mundo Medical Center (formerly Children’s Medical

Center), a hospital in Manila, the Philippine capital, fifty new cases are seen
every year. 

For this reason, it is necessary for the healthcare team members and
the public to have an adequate knowledge regarding the case.

The researchers have decided to deal with the case of a 7-year old
male patient who experiences Acute Lymphocytic Leukemia. The researchers
would like to know about the disease and its different causes, contributing
factors, signs and symptoms complications, as well as its appropriate medical
management and nursing interventions that can be done to alleviate the
problem of the patient. The researchers would also want to refresh their
knowledge about the anatomy and physiology of the organs involved so that a
schematic diagram of the disease can be formulated.

At the same time, it is necessary for nurses to have an in-depth

understanding regarding the aforementioned disease because it has a great
involvement in rendering care to those patients who are experiencing the
same problem. Furthermore, the researchers can use and apply the acquired
theoretical knowledge into practice and will be able to educate the client
about the significant facts about her condition.
 C. Significance of the Study

This study was undertaken to contribute foremost to the following:

 Patient
Simultaneously with the conduction of this case
study, the patient having the condition will be made better as
well as the other patients having the same case. With the
new knowledge acquired and with innovative skills and
interventions learned, the state of the patient will have an
improvement.

 Patient’s Significant Others

This case study would also benefit the relatives of a
patient for they too could acquire knowledge about the
condition of the patient through teachings and relevant skills
by means of the practical demonstrations of the provider of
care. They developed also positive outlook on behalf of the
patient for they themselves witnessed the improvement of the
condition of the patient.

 Individual
This study caters every individual a closer view about the
disease, its etiology or causes, clinical manifestations,
complications, and its proper management.

 Community
This work can be very useful especially in the
community setting for this can be a tool for the development
 of awareness and understanding regarding the said condition
since it is prevalent in aforementioned setting. This could help
the people in the community to be equipping with sufficient
knowledge and skills so that similar condition could be
prevented. Such knowledge and skills will be imposed
through health teachings and seminars in the community
using this work as the reference guide.

 Nursing Profession
This study would serve as a reference guide for the
purpose of follow-up investigation and further acquisition of
knowledge for the aforementioned abnormality. In addition,
this work could be helpful to the nurses for the purpose of
continuity of care to be rendered to the particular patient. This
could also serve as a basis for future care of plan for those
patients with the same case that could be possibly
encountered by professional nurses in the future.

 Student Nurses
This work would become a valuable instrument in
achieving goals such as acquiring new knowledge about the
case, enhancing capabilities and skills that are very essential
to nursing practice and developing positive behavior of the
nursing students toward the betterment and personal growth
as the future professional nurses.

 Clinical Instructors
 This study would serve as a material that would help
the instructors evaluate the level of proficiency and
competency of the students. This study also helped the
instructors appraise and measure the progress and
professional growth of the students thus deliberate the
adequacy and deficiency of the students for the purpose of
betterment.

D. Scope and Limitation

This study is focused on a specific patient diagnosed of having Acute

Lymphocytic Leukemia (ALL) who was admitted in MCU-FDTMF Hospital,
Pediatric Ward. Gathering of data were based on the medical records of the
patient, the patient himself and his relatives.

This study emphasized the causes, risk factors, the signs and symptoms,
complications and appropriate medical and nursing management of the said
case. The anatomy and physiology of the organs involved were also discussed
for better understanding of the disease. Pathophysiology, drug studies of the
medications given during the shift, nursing care plan of three problems with the
highest priority, long term objectives, and discharge planning were also included.