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This episode presents an approach to acute leukemia in children. Listeners will learn about the epidemiology, etiology, and pathogenesis of acute leukemia. Listeners will also develop an approach to the clinical presentation and initial management of acute lymphoblastic leukemia. This podcast was developed by Amarjot Padda in collaboration with Dr. Bev Wilson, a Pediatric Oncologist at the Stollery Children’s Hospital.
Here are excerpts:
Approach to Acute Leukemia in Children
According to the 2015 Canadian Cancer Statistics, leukemia represents a third of new cancer cases in children aged 0-14 years. Among the different types of acute leukemias, ALL is the
most common pediatric cancer (2), and therefore will be the focus of this podcast. ALL is most commonly diagnosed in children from 3 to 5 years of age and is slightly more common in boys. Although ALL is curable in most cases, a diagnosis of ALL has a significant impact on the lives of children, and their families (2, 4).
Leukemia occurs as a result of a series of genetic mutations leading to aberrant hematopoiesis in the bone marrow.
In children with ALL, this process [of hematopoies] is deregulated due to a series of genetic mutations, allowing clonal expansion of a single lymphoid progenitor cell or blast cell (1). The ability of this leukemic blast cells to proliferate and accumulate in the bone marrow essentially stops normal hematopoiesis or stops the normal bone marrow from
Depending on the stage of hematopoiesis at which these mutations occurred, ALL could be further sub-categorized into B-cell ALL, T-cell ALL, and ALL of ambiguous lineage (5).
Etiology and Risk Factors:
The exact etiology of ALL has not been determined.
While there may be an inherited genetic predisposition for some cases of leukemia, the majority are the result of somatic mutations or not related to inheritance (i.e. random events). There are no clearly identified environmental exposures that are linked to ALL other than radiation exposure.
A child with leukemia may present chronically with an array of non-specific symptoms to their family doctor or with
more acute symptoms in an Emergency Department. Recently, more and more cases of childhood ALL are picked up incidentally on blood work often done for another reason.
The most common clinical presentation of ALL includes fever, fatigue and pallor (1).
More generally, children with ALL tend to be irritable.
Because these symptoms have a very broad differential diagnosis and ALL is very rare, ALL should not be the first diagnosis in mind when these patients present to primary care. However, it is important to follow-up with patients regarding these non-specific symptoms if they are persistent to ensure a diagnosis of ALL is not missed. [Emphasis added]
The Essence of Pediatrics
The above paragraph contains the essence of pediatric care. The every patient must be carefully followed until symptoms resolve.
The big key in Pediatrics is that most cases the pediatric patient presents with non-specific sysmptoms, is sick for awhile, and then gets better.
The pediatrician’s job in evaluating the patient is three fold.
First, the physician detects signs and symptoms that suggest the possibility of life-threatening disease (for example, sepsis). And then the physician initiates life-saving supportive care and specific disease specific treatment (all of this usually by initiating transfer by EMS from the office to the emergency department).
Second, the physician initiates treatment for diseases that are not life threatening (for example, otitis media, strep pharnygitis, or a viral syndrome).
And third, the pediatrician must educate the patient’s parents on what actions to take if the symptoms change or get worse.
Since the vast majority of pediatric illness resolves in a week or so, patients who continue to appear ill should be carefully re-evaluated (even in the absence of apparent life-threatening illness) until symptoms resolve or a diagnosis and treatment are achieved.
Let’s first discuss the hematological symptoms of ALL.
Common manifestations of anemia in ALL patients include pallor, fatigue, lethargy, and possible dyspnea (4).
Parents will often describe how their child has a lack of energy, can no longer keep up with other kids, and no longer enjoys their usual activities.
Neutropenia can present as recurring bacterial or invasive
fungal infections, although this is a very rare presentation of ALL (1).
Lastly, thrombocytopenia can present as a bleeding abnormality like bruising, petechaie, purpura, as well as mucosal bleeding (1).
Infiltration of the lymphatic system is responsible for possible lymphadenopathy, hepatomegaly, and/or splenomegaly all of which can be presenting symptoms of childhood leukemia (1).
In addition to the hematological symptoms of leukemia, there can be a wide variety of extra medullary symptoms.
Of all the sites of extramedullary invasion, bone and joint
involvement is relatively common. Up to 25% of patients present with bone or joint pain as the initial symptoms of concern (4).
In addition to bone pain, children with bone and joint involvement may present with a limp.
ALL can also involve the Central Nervous System and
boys can have testicular involvement. These will not be discussed in detail in this podcast.* However, for these reasons it is important to do detailed neurological and testicular exams in
addition the rest of the complete physical exam.
*For additional information on ALL, please see [from emedicine.medscape.com]:
Diseases/Conditions, Feb 20, 2020
Diseases/Conditions, Jan 2, 2019
It is also important to note that ALL can present as an emergency in certain situations. Emergent complications related to ALL include febrile neutropenia, sepsis, hyperleukocyotsis, superior vena cava syndrome or a mediastinal mass which can compress the trachea (5, 6).