Acute Promyelocytic Leukemia: Early Intervention andSurvival Outcomes
- sunshine4cancerkid
- Aug 5
- 22 min read
Updated: Aug 7
Thanh Hoang | Writer/Researcher
Canadian International School Saigon
Olivia Su | Writer/Researcher
Spring-Ford 9th Grade Center
Harini Sethuraman | Writer/Researcher
Mount Hebron High School
Sarina Khodayari | Writer/Researcher
Thornlea Secondary School
Neha Shiju | Writer/Researcher
Amsterdam International Community School
Table Of Contents
Table Of Contents..............................................................................................................................2
Abstract...................................................................................................................................................3
Introduction............................................................................................................................................ 4
Leukemia........................................................................................................................................... 4
Causes................................................................................................................................................4
Discussion................................................................................................................................................5
Why APL?......................................................................................................................................... 5
How to Diagnose Patients..................................................................................................................... 6
Signs and Symptoms......................................................................................................................... 6
Medical Equipment and Tests............................................................................................................6
Bone Marrow Biopsy..................................................................................................................6
Complete Blood Count (CBC)....................................................................................................6
PCR Test..................................................................................................................................... 6
Peripheral Blood Test..................................................................................................................6
Flow Cytometry.......................................................................................................................... 7
Immunophenotyping Test........................................................................................................... 7
Cytogenetic Test..........................................................................................................................7
Treatments.............................................................................................................................................. 8
The First Stage of APL Treatment: Induction................................................................................... 8
The Second Stage of APL Treatment: Consolidation........................................................................8
The Last Stage of APL Treatment: Maintenance.............................................................................. 8
Another Crucial Therapy: Supportive Therapy.................................................................................9
Factors Affecting Treatment Plan......................................................................................................9
Medical Professions..............................................................................................................................10
Pathologist....................................................................................................................................... 10
Role in Treatment......................................................................................................................10
Education.................................................................................................................................. 10
Hematologist-Oncologist.................................................................................................................11
Role in Treatment......................................................................................................................11
Education...................................................................................................................................11
Statistics................................................................................................................................................ 12
Patients Diagnosed Annually...........................................................................................................12
Survival Rates with Early Diagnosis...............................................................................................12
Table 1.......................................................................................................................................12
Table 2.......................................................................................................................................13
Impacts..................................................................................................................................................14
Physical Impacts..............................................................................................................................14
Mental Impact..................................................................................................................................14
Financial Impact.............................................................................................................................. 15
Conclusion.............................................................................................................................................16
Real World Relevance and Future Research................................................................................... 17
Raising Awareness...........................................................................................................................18
Bibliography (MLA 9th Gen)..............................................................................................................19
Abstract
Acute Promyelocytic Leukemia (APL), a rare and now treatable disease, is classified
by the overproduction of immature white blood cells in the bone marrow. Bone marrow is the
spongy part within the bone that produces blood cells for the body. This continuous
production supports proper bodily functions and maintains healthy blood. All blood cells
originate from stem cells, which have the potential to develop into specialized cells
(totipotent stem cells). Examples of specialized cells include red blood cells, platelets,
monocytes, and granulocytes. Myeloid stem cells are a key example of stem cells that can
further advance into various important blood cells. In APL, the bone marrow generates an
excessive number of immature cells that are not fully differentiated and can not carry out
normal functions. To diagnose APL in early stages, a combination of methods, such as bone
marrow biopsies and complete blood counts, can be administered and analyzed by healthcare
professionals, including pathologists and hematologist-oncologists. The focus of this research
is centered on how early intervention in APL can impact survival outcomes. While APL used
to be a highly mortal disease, it is now one of the most curable, with survival rates of
approximately 80%, simply due to more advanced treatment methods such as
all-trans-retinoic acid (ATRA) and arsenic trioxide (ATO).
Introduction
Leukemia
Leukemia is a cancer of the blood resulting from abnormal proliferation of white
blood cells (WBCs). This condition hinders the WBCs’ ability to defend the body against
infection and negatively affects the bone marrow in the production of blood cells. Leukemia
is divided into chronic leukemia, which progresses slowly, compared to acute leukemia,
which demands urgent medical intervention. Additionally, leukemia is further categorized
into lymphocytic leukemia and myelogenous (myeloid) leukemia. In lymphocytic leukemia, a
mutation occurs in cells that will later differentiate into lymphocytes, a type of WBC that
plays an important role in the immune system. Whereas in myeloid leukemia, a mutation will
occur in cells that will mature into red blood cells, WBCs, and platelets (“Leukemia”).
Acute Promyelocytic Leukemia
Acute Promyelocytic Leukemia (APL), a rare subtype of acute myeloid leukemia
(AML), is a type of blood cancer in the bone marrow. APL is responsible for less than 10% of
pediatric AML cases (Conneely et al.). APL is characterized by the accumulation of
immature and abnormal neutrophils (a type of white blood cell) called promyelocytes in the
bone marrow (“Acute promyelocytic leukaemia (APML)”). This phenomenon leads to a
shortage of healthy blood cells for the body, causing an increased susceptibility to bleeding,
infections, and slow healing.
The cause of APL involves a translocation mutation in which part of the
Promyelocytic Leukemia (PML) gene on chromosome 15 and part of the Retinoic Acid
Receptor Alpha (RARα) gene on chromosome 17 fuse together, known as t(15;17). The
mutation produces a protein called the PML-RARα protein. The PML protein produced from
the PML gene acts as a tumor suppressor, preventing cells from dividing rapidly, while the
RARα protein produced by the RARα gene regulates gene transcription, particularly genes
that contribute to the maturation of white blood cells beyond the promyelocyte stage. The
PML-RARα protein strays from the normal function of the PML protein and RARα protein.
Blood cells are stuck at the promyelocyte stage and divide abnormally, leading to APL. 98%
of APL cases are the result of PML-RARα gene fusion (“Acute Promyelocytic Leukemia”).
APL cannot be inherited as the condition occurs through somatic mutation, which happens in
non-reproductive cells (“Acute Promyelocytic Leukemia”).
Previously, APL was reported as deadly due to its excessive bleeding. Decades later,
progress in APL treatment has significantly enhanced, giving rise to leukemia treatment such
as all-trans retinoic acid (ATRA) and arsenic trioxide (Schuh). According to Schuh, positive
outcomes were mostly observed in leukemia patients who received immediate diagnosis and
treatment. Patients who lacked such care did not recover successfully. Hence, this research
will concentrate on how early detection and intervention impact survival rates in patients
diagnosed with Acute Promyelocytic Leukemia.
Discussion
The topic of Acute Promyelocytic Leukemia intrigued us due to the historically low
survival rates, but in the 1980s, the breakthrough of a cure for this rare disease boosted the
survival rates of those diagnosed early. The discovery of the ATRA and ATO treatments was
a major contributor to current APL survival rates and curability (Osman et al.). Moreover,
APL is one of the few cancers produced as a result of a chromosomal translocation t(15;17),
creating the PML-RARα gene fusion. Interestingly, a small translocation in a gene is
responsible for a complex disease (“Acute Promyelocytic Leukemia (APL)”).
Early intervention in complicated diseases like APL can drastically improve survival
rates. Researching this topic helps us understand the impact of early intervention, the
increased curability of APL, and the increased survival rates due to modern treatments.
Without proper treatment, APL can have early mortality in patients, but with early
intervention and the use of modern treatments, patient survival rates have improved, and APL
has become a highly curable disease. For a disease that was once considered highly mortal, to
now becoming the most curable type of leukemia, APL’s modern treatments and the effect of
early intervention should be studied to understand how the survival rates drastically improved
over time.
Diagnose
Signs and Symptoms
Some signs and symptoms of APL include low red blood cell counts (anemia), fatigue
due to anemia, low white blood cell counts, high number of infections, unintentional weight
loss, low number of platelets, bruising and excessive bleeding, headaches from bleeding in
the brain (intracranial hemorrhage), and bloody poop due to gastrointestinal bleeding (“What
Is Acute Promyelocytic Leukemia (APL)?”).
Medical Equipment and Tests
APL can be diagnosed using a variety and combination of methods, each one
contributing to earlier treatment intervention methods and higher survival rates. There are
specific diagnosis methods used to confirm the presence of the PML-RARA fusion gene,
which is the gene causing APL to occur. Specific tests ordered by specialists to diagnose APL
include bone marrow biopsy, complete blood count (CBC), PCR test, peripheral blood smear,
flow cytometry, immunophenotyping test, and cytogenetic test (“Acute Promyelocytic
Leukaemia Diagnosis - Leukaemia Foundation” and “What Is Acute Promyelocytic
Leukemia (APL)?”)
Bone Marrow Biopsy
A bone marrow biopsy involves extracting a sample of bone marrow to evaluate the
proportion of leukemic cells. It provides critical information for confirming the diagnosis of
APL and assessing disease severity (“What Is Acute Promyelocytic Leukemia (APL)?”).
Complete Blood Count (CBC)
A CBC measures the levels of red blood cells, white blood cells, and platelets in the
blood. In APL, it often reveals low platelets, anemia, and abnormal white blood cell counts
(“What Is Acute Promyelocytic Leukemia (APL)?”).
PCR Test
PCR is used to detect the PML-RARA fusion gene, which is the abnormal gene
causing APL. It helps confirm the diagnosis of APL at the molecular level (“What Is Acute
Promyelocytic Leukemia (APL)?”).
Peripheral Blood Smear
This test involves examining a stained blood sample under a microscope. In APL, it
may show Auer rods in promyelocytes or high levels of granules, which are key indicators of
the disease (“What Is Acute Promyelocytic Leukemia (APL)?”).
Flow Cytometry
Flow cytometry analyzes the specific protein patterns expressed on the surface of
abnormal blood cells to identify the leukemia subtype. In APL, it typically shows strong
expression of markers like CD13, CD33, and CD117 and absent expression of markers like
CD34 and HLA-DR (Horna et al., 2014b).
Immunophenotyping Test
This test classifies cells based on their surface markers, helping distinguish APL from
other forms of leukemia. It is often done alongside flow cytometry for accurate subtype
identification (“Acute Promyelocytic Leukaemia Diagnosis - Leukaemia Foundation”).
Cytogenetic test
Cytogenetic tests detect chromosomal abnormalities, particularly the t(15;17)
translocation in APL. Fluorescence in situ hybridization (FISH) or karyotyping can visually
confirm this genetic change (“What Is Acute Promyelocytic Leukemia (APL)?”).
Treatments
Treating Acute Promyelocytic Leukemia (APL) requires a variety of different
therapies and drugs. As of 2023, the remission rate for APL is 90-95%, with over 80% of
patients being free of the disease for 5 years (Yin et al. 2). However, it is important to note
that there are still chances that APL can come back despite the administration of these
treatments (“Acute Promyelocytic Leukemia (APL)”). It is a medical condition that requires
immediate attention since it has a significantly high pre-treatment mortality (Cingam and
Koshy).
The First Stage of APL Treatment: Induction
The first stage of APL treatment is induction, which involves putting the APL into
remission, effectively reducing the cancer cells to negligible amounts. The main drug
involved at this stage is all-trans-retinoic acid (ATRA). Alongside this drug, arsenic trioxide
(ATO) and chemotherapy can also be used. ATO is considered when there is a lower chance
of APL returning after initial treatment since it has fewer side effects. Administration of only
ATRA and ATO has a high remission rate. However, these drugs can only be used in patients
with no sign of cardiac dysfunction (“Treatment of Acute Promyelocytic Leukemia (APL)”).
In the case where there is a high risk of APL returning or if the white blood cell count
increases, chemotherapy or a targeted drug called gemtuzumab ozogamicin (Mylotarg) may
be utilized (“Treatment of Acute Promyelocytic Leukemia (APL)”).
Induction typically lasts around 2 months. A month into the treatment, a bone marrow
biopsy and genetic testing are performed to monitor the progress of leukemia (“Treatment of
Acute Promyelocytic Leukemia (APL)”).
The Second Stage of APL Treatment: Consolidation
The second stage of APL treatment is consolidation. This stage ensures that the APL
is kept in remission and attempts to kill the remaining leukemia cells. The drugs used in
consolidation depend on what was administered in induction, as well as other factors that
potentially impact the patient’s health (for example, allergies). Consolidation takes quite a
few months, varying based on the drug usage (“Treatment of Acute Promyelocytic Leukemia
(APL)”).
The Last Stage of APL Treatment: Maintenance
The last stage is maintenance, which is especially important for individuals at a higher
risk of APL returning. Maintenance therapy uses a smaller dosage of drugs for a longer period of time. This therapy usually lasts around a year. Patients may also be monitored for
up to 2 years with PCR post-treatment (Cingam and Koshy). This stage is omitted for
individuals at a lower risk of APL returning (“Treatment of Acute Promyelocytic Leukemia
(APL)”).
Another Crucial Therapy: Supportive Therapy
Alongside these treatments, another crucial therapy is supportive therapy. This
therapy aims to improve the patient’s quality of life by managing the side effects of the
cancer. For example, one of the complications of the disease is bleeding diathesis (increased
susceptibility to bleeding). This should be kept above 30 - 50 × 10/1, and fibrinogen (a
protein made in the liver that is involved in blood clot formation) should be above 100 mg/dl
to 150 mg/dl (Cingam and Koshy).
Factors Affecting the Treatment Plan
The treatment plan for APL varies for every individual as their preexisting allergies,
conditions, and preferences have to be considered. However, one of the major factors
affecting the type of treatment chosen is whether the patient is at a high risk or low risk. As
discussed in the above stages of treatment, the drugs administered may vary depending on the
risk of APL returning. Generally, a non-high-risk/low-risk patient is defined by a white blood
cell count that is less than or equal to 10 × 10 . A high-risk patient has a white blood 9/L cell count that is higher than 10 × 10 (Sanz et al.). Intrathecal therapy is performed in 9/L high-risk patients. Other factors can affect the prognosis of a patient. These include higher
age, male patient, elevated serum creatinine, and fibrinogen levels (Cingam and Koshy).
If patients relapse or are unable to withstand or undergo the therapies for APL, bone
marrow transplantation is an option. However, this is not preferred in regular cases since APL
has a high cure rate (Cingam and Koshy).
Medical Professions
Early intervention in Acute Promyelocytic Leukemia would not be possible without
the contributions of key medical professionals. From making the initial diagnosis to
organizing a treatment plan with chemotherapeutic and non-chemotherapeutic drugs, experts
like pathologists and hematologist-oncologists play essential roles (“Acute Promyelocytic
Leukemia (APL)”).
Pathologist
The main duty of a pathologist is to analyze samples derived from bodily fluids and
tissues to determine if you have a certain condition, like cancer (“Pathologist”). In the case of
Acute Promyelocytic Leukemia, pathologists examine the flow cytometry, a lab test used to
assess the characteristics of cells from blood, bone marrow, and other fluids (“Flow
Cytometry”). They specifically check for unusual protein patterns that can confirm the
presence of APL. Additionally, pathologists may investigate the chromosomes in abnormal
cells further to confirm an APL diagnosis (“Acute Promyelocytic Leukemia (APL)”). When a
diagnosis of APL is established through chromosomal analysis, it often involves the
identification of a chromosomal translocation. In the case of APL, this translocation occurs
between chromosomes fifteen and seventeen, where a fragment has detached and reattached
itself (Ryan). Becoming a pathologist begins with obtaining a bachelor’s degree in a science
or pre-med program. Afterward, you must attend a medical school in either an Allopathic
(M.D. degree), where you will be required to take the United States Medical Licensing
Examination (USMLE) Step 1 and 2, or Osteopathic (D.O. degree), where you must take the
COMLEX exams. Following this, it is mandatory to attend a three-year residency studying
either anatomic or clinical pathology. However, if you choose to study both, a four-year
residency would be necessary. Unlike a residency, board certification is not required to
become a licensed pathologist; however, it is common for institutions such as hospitals and
clinics to request it, and it is typically obtained as a demonstration of knowledge and
expertise. Similar to the residency, there are two exams you may take, either anatomic or
clinical pathology. Pathology is a vast field; per your specialty, fellowships can range from
one to two years. A fellowship is not necessary to find a job but, it may increase your
compensation (Torres). The salary for a pathologist ranges from $45,500 to $387,000,
depending on your specialty and state of practice (“Pathologist Salary”).
Chromosomal translocation resulting in PML-RARα fusion (MDPI)
Hematologist-Oncologist
A hematologist specializes in the study and treatment of blood disorders. However, a
hematologist-oncologist also addresses cancers affecting the blood, including Acute
Promyelocytic Leukemia (“Hematologist”) and (Kumar). These specialists are involved in
ordering tests, for instance, a biopsy of your bone marrow to examine for leukemia cells, and
any additional tests on blood and bone marrow samples to look for genetic changes (“Acute
Promyelocytic Leukemia (APL)”). If they suspect APL, they are required to immediately
admit patients and initiate the treatment process using All-Trans Retinoic Acid (ATRA), even
before a diagnosis, due to the urgency of the disease. In patients who relapse, they combine
the ATRA with chemotherapy or with ATO (Arsenic Trioxide) (Cingam and Koshy). A
hematologist-oncologist’s career path involves completing a bachelor’s degree in a pre-med
or science program. Then, attending a four-year program at a medical school where one
should focus their classes on hematology and blood systems (“What is a Hematologist and
How to Become One”). Following medical school, you must complete a three to five-year
residency, with internal medicine and pediatrics being the most common. Once you have
finished your residency, you must begin your fellowship, which may be adult/pediatric
hematology, coagulation, or, in this case, hematology-oncology. Finally, you are eligible to
obtain your board certification from the American Board of Internal Medicine or the
American Board of Pediatrics and pass the United States Medical Licensing Examination
(USMLE) to be licensed in your state of practice (“Hematology”). A
hematologist-oncologist's salary may range from $115,500 to $400,000 annually, with the
average being $337,179, which may differ according to your location, years of experience,
and seniority (“Hematologist-Oncologist Salary”).
Statistics
Acute Promyelocytic Leukemia is a type of promyelocytic cancer in which survival
rates may increase due to early detection and treatment. Discovering the disease early on
allows for treatment to be done at an earlier, less intense stage of the illness. This often leads
to more successful interventions and thus improves patient survival rates. Acute
Promyelocytic Leukemia (APL) also affects relatively few patients annually, with ages
ranging from <18-60+ years old.
Patients Diagnosed Annually
According to an article on the National Organization for Rare Disorders, it states that
“[APL]...develops in about 600 to 800 individuals each year in the United States, most often
in adults around the age of 40 but also children” (“Acute Promyelocytic Leukemia” ). This
statistic shows that although APL is an uncommon disease, it is still a widespread disease
predominantly affecting middle-aged individuals.
Survival Rates with Early Diagnosis
Table 1: A 3-year experiment conducted in California highlighting the correlation between
early APL diagnosis and survival rates.
This piece of data from the California Cancer Registry demonstrates how early diagnosis of
APL can lead to greater chances of survival in patients. Based on the data, the 3-year survival
rate increased from approximately 80% before the ACA (Affordable Care Act, which
coincided with and improved healthcare in California) to 92.7% in the full ACA era” (Kentsis
et al., 2022). Additionally, the chi-squared test* also confirmed that there was a connection
between early detection and mortality rates.
Table 2: A 3-year experiment conducted in California, highlighting how early APL diagnosis
affects the survival rates of different age groups
In earlier experiments conducted by Yin, Xue-Jiao, et al., it came to a conclusion that
there was a relationship between the individual's age and their survival rates. Building upon
this, a 3-year experiment conducted in California further discovered how early diagnosis of
APL (Acute Promyelocytic Leukemia) affected patients of different age groups. For instance,
among patients aged ≤39 years diagnosed with APL in California, 30-day death rates
decreased from 26% before ATRA (1988-1995) to 14% after ATRA (2004-2011) (Abrahão,
Renata et al.).This statistic highlights the vital role of early diagnosis and intervention, as it
showed a significant increase in survival rates in younger patients, and it led to fewer early
deaths.
____________________________________
* Chi-squared test: A statistical test used to determine if there is a relationship between the
two categorical variables. A significant p-variable (<0.005) suggests that there is a
connection between the two.
Impacts
Physical Impact
Although APL has relatively high survival rates, children may suffer physically while
being diagnosed with APL. Some common symptoms include paleness and shortness of
breath when the patient is physically active due to a lack of red blood cells and low oxygen
levels. Children with APL will also experience infections more often and heal more slowly
compared to healthy children, as they lack neutrophils. In addition, there is an increase in
serious bleeding and bruising due to low platelet count and clotting factors, in certain cases,
can lead to hemorrhage (“Acute Promyelocytic Leukaemia (APML)”).
Not only will children with APL suffer from their symptoms, but they are also left
with long-term side effects after treatment. These impacts include permanent fatigue and
differentiation syndrome incidence. Differentiation syndrome is a deadly complication in
children who undergo ATRA therapy or are treated with arsenic. Peripheral edema,
hypotension, acute renal failure, and interstitial pulmonary infiltrates are some common
symptoms of differentiation syndrome. Up to 27% of patients who had ATRA or arsenic for
APL treatment suffered differentiation syndrome (Reyhanoglu, Gizem et al.). Additionally,
according to an article on the American Society of Clinical Oncology, it states that more than
one-fifth of patients reported experiencing long-term fatigue as a result of being diagnosed
with APL (Efficace et al.). Survivors suffering from ongoing fatigue could find their quality
of life start to diminish as their abilities to work, interact, and the performance of daily
activities are all being impacted.
Mental Impact
APL can have profound emotional effects on children and the youth from not only the
condition itself, but also the complicated treatment process. Certain procedures like
chemotherapy and radiotherapy can harm the normal function of the ovaries, which can either
temporarily or permanently decrease their fertility later in life. This could affect children
emotionally when they want to start a family later in life. In addition to that, children with
APL have to cope with isolation and hopelessness from not being able to engage in physical
activities, prolonged hospitalization, a reduction in quality of life, and fear of relapse
(Amonoo, H.L et al.). Children with APL will be suspended from going to school, and over
time, they may feel pressured and inferior when seeing their classmates progress in school.
Individuals may also fear that they will get diagnosed with cancer once more, even after they
have been declared “cured”. This fear, once it stockpiles, could turn into anxiety, affecting the
individual on a day-to-day basis.
Financial Impact
Cost is another important factor that should also be considered when examining how
APL affects children's lives. While the cure rate of APL is high, the financial burden, on the
other hand, is costly, and thus, the treatment might not be accessible to all patients with APL.
In an article by Dieguez, G et al, over the following three years after leukemia diagnosis,
patients with acute leukemia had a cumulative average allowed spending of up to $800,000
USD. The family of the children will have to pay for the travelling expenses back and forth to
the hospital. It is important to acknowledge that gathering such medical fees and additional
costs, such as travelling and frequent monitoring in case of a relapse, is tremendously
challenging - especially for households that come from a low-income background.
Conclusion
Summary of Findings
This study investigated the role of early intervention on the survival rate and overall
outcome of APL patients. By looking into the various treatments for APL, the diagnosis
procedure, the statistics of APL patients, the impact of the disease, and the medical
professions involved, the key focus of the research was addressed. From our research on
treatments, it was found that there are 3 main stages of treatment. These include induction,
consolidation, and maintenance. Another therapy that can improve a patient’s quality of life is
supportive therapy. This is a basic framework for APL treatment. However, the treatment
may be adjusted based on the risk of APL returning, the patient’s history, and their needs.
Once extremely deadly, APL is now one of the most curable leukemias. This was due
to the fact that scientists and researchers were able to locate a specific cause for it and
develop specific medicines such as ATRA. This article summarizes the importance of finding
and treating APL quickly. Early diagnosis and treatment not only benefit the patient in the
long run but are also critical for immediate survival. Because APL can cause dangerous
bleeding and clotting, it is important to identify it quickly and take medications to treat it as
soon as possible.
It can be concluded that physical, emotional, and financial impact are essential
elements when taking into account the effect that APL has on children during treatment and
on their lives post-treatment. For children, they might be insecure about their appearance and
overall health as a result of the bruise, loss of hair during chemotherapy or radiotherapy, and
inability to engage in physical activities like their peers. It is therefore important for their
families and medical teams to conduct counselling frequently and encourage APL patients to
engage with their community. Likewise, one of the challenging aspects when managing
children with APL is the risk of having Disseminated Intravascular Coagulation (DIC) - a
life-threatening complication that necessitates instant medical action or else can lead to
hemorrhage. This posits a clinical challenge where receiving a timely diagnosis is equally
important as having sufficient resources to begin APL treatment (Anggraini, F.F). This is an
obstacle for children and their families in developing countries, where immediate treatment
and resources such as advanced medical equipment are not always readily available. It is
therefore valuable to investigate a rare condition such as acute promyelocytic leukemia. By
emphasizing the advancement in our current treatment, the barrier that prevents children from
accessing quality healthcare, and suggestions made, this could help inform the public how
early intervention in APL could influence patients’ outcomes.
It is essential to study the role of early interventions and their impact on the survival
outcomes of patients, as it allows us to develop a more streamlined approach to cancer
treatment. Over the past years, cancer research has seen immense progress, with the
development of better treatments, new technology for detection and curing, etc. However, in
order to protect patients to the greatest extent, they should endure as little suffering as possible, attempting to maintain and improve their quality of life. It is estimated that the total
number of new cancer cases in females will increase from 0.603 million in 2011 to 0.935
million in 2026 (Dsouza et al.). This is only a fraction of the world’s population that is
accounted for. Several other demographic groups are not considered. With cancer becoming
an increasingly important area, it is beneficial to examine the possibility of early intervention
for higher survival rates and better quality of life.
Again, even though survival rates of APL are relatively high compared to other
diseases, an APL diagnosis can significantly impact the individual socially and emotionally.
Patients are often left with long-term fatigue even after treatment, causing their overall
quality of life to go down. When this factor decreases, it affects how the individual behaves
in friend groups; they may want to withdraw from conversations and isolate themselves.
Patients may fear that they will develop cancer again after they have been “cured”. Once this
fear builds up, it turns into anxiety and may lead to depression. Ultimately, while APL had
high mortality rates, patients still face many challenges, whether it's socially, emotionally, or
financially, and it is important to understand and address these struggles. Our group chose
this topic not only to highlight the importance of early diagnosis and intervention of APL, but
also to provide an overview of APL as a whole. This includes how to diagnose APL, how to
treat APL, the impacts of being diagnosed, and medical professionals centered around this
disease.
Real World Relevance and Future Research
APL is an example of success in leukemia treatment due to modern therapies
transforming a once lethal disease into a highly curable disease today. The use of
differentiation therapy, such as ATRA and ATO, has significantly improved complete
remission and long-term survival rates in clinical settings (Bidikian et al.). However,
real-world data shows that this progress is not the same across all populations. Early
mortality, primarily due to hemorrhagic complications linked to coagulopathy at diagnosis,
remains a critical concern. In large-scale observational studies, there are significantly better
outcomes when ATRA is administered immediately upon clinical suspicion of APL, thus
showing the importance of a prompt diagnosis and treatment initiation even before genetic
confirmation (Bewersdorf et al.).
Despite the existence of standardized treatment protocols, outcome disparities exist
across different demographics and healthcare systems. Older adults and patients treated at
non-academic centers experience disproportionately higher early mortality rates and lower
survival outcomes, displaying systemic barriers in access to timely care (Teng-Fei et al.).
Additionally, APL patients with high-risk features such as elevated white blood cell counts
face more challenges, reinforcing the need for early treatment and supportive care. Many
cases of early death occur before full therapy initiation, which emphasizes the importance of
immediate responses in emergency settings (Bidikian et al.).
In low and middle-income countries, efforts to reduce these disparities have included
establishing clinical networks and using streamlined communication protocols. These
strategies have led to substantial improvements in mortality and survival rates (Puttirangsan
et al.). They demonstrate that the key to better real-world outcomes is not only dependent on
therapeutic advancements but also on structural reforms to allow for rapid diagnosis and
access to ATRA and ATO.
Future research should focus on identifying and addressing delays in treatment
initiation and expanding the use of oral ATO formulations to improve accessibility in
environments with a lack of resources (Teng-Fei et al.). Earlier intervention to increase
survival outcomes will require efforts in research, structural reforms to reduce healthcare
disparities, and increased accessibility to treatment.
How to Raise Awareness?
We can raise awareness about APL through social media, community events, posters, etc.
September is blood cancer awareness month, which is a key month to raise awareness. This
can happen by partnering with organizations like the Leukemia and Lymphoma Society to
host community events like webinars, walks, and information booths, and publicize them by
using hashtags such as #BloodCancerAwareness and #FightAPL. Raising awareness about
diseases requires dedication and passion, but it can be achieved through the utilization of
online media and events such as fundraisers that both bring people together and inform them
about a cause.
Works Cited
Abrahão, Renata, et al. “Early Mortality and Survival Improvements for Adolescents and
Young Adults with Acute Promyelocytic Leukemia in California: An Updated
Analysis.” Haematologica, U.S. National Library of Medicine, 1 Mar. 2022,
“Acute Promyelocytic Leukaemia (APL).” Acute Promyelocytic Leukaemia (APL or APML) |
Cancer Research UK, 26 Apr. 2024,
te-promyelocytic-leukaemia.
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