Download Early diagnosis of childhood Acute lymphoblastic leukemia

Case Report
Early diagnosis of childhood Acute lymphoblastic leukemia
Vanitha Pandey1, Mahesh Kumar K2, Indira3
1,2
Assistant Professor, 3Professor & Head, Department of Pathology, Malla Reddy Institute of Medical Sciences, Suraram,
Hyderabad , Telangana, India.
Address for Correspondence: Dr. Kandukuri Mahesh Kumar, Assistant professor, Department of Pathology, Malla Reddy
Institute of Medical Sciences, Suraram, Hyderabad, Telangana, India.
Email id: [email protected]
ABSTRACT
Acute lymphoblastic leukemia (ALL) is characterized by
an overproduction of immature white blood cells, called
lymphoblasts or leukemic blasts. These cells crowd the bone
marrow, preventing it from making normal blood cells. They
can also spill out into the blood stream and circulate around
the body. Due to their immaturity, these cells are unable to
function properly to prevent or fight infection. Inadequate
numbers of red cells and platelets being made by the marrow
cause anemia, and easy bleeding and bruising. ALL is the most
common type of childhood leukemia, and the most common
childhood cancer. It is more common in males than in females.
The exact causes of ALL remain largely unknown but it is
thought to result from mutations in one or more of the genes
that normally control blood cell development. Chemotherapy
is the main form of treatment for ALL. A combination of drugs,
including steroids, is usually given in several cycles with a rest
period of a few weeks in between. Initially, the aim of treatment
is to destroy leukemic cells and induce a remission.
Keywords: Acute Lymphoblastic Leukemia, Subleukemic
leukemia, Atypical Lymphocytes, CD-20, Bone Marrow.
INTRODUCTION
Acute lymphoblastic leukemia (ALL) is a type of cancer of the
blood and bone marrow, the spongy marrow tissue inside the
bones where blood cells are made. Acute lymphoblastic
leukemia, also known as acute lymphocytic leukemia or acute
lymphoid leukemia (ALL), is an acute form of leukemia, or
cancer of the white blood cells, characterized by the
overproduction and accumulation of cancerous, immature
white blood cells, known as lymphoblasts . The word "acute"
in acute lymphoblastic leukemia comes from the fact that the
disease progresses rapidly and creates immature blood cells,
rather than mature ones. The word "lymphoblasts" in ALL refers
to the white blood cells, which ALL affects. Acute lymphocytic
leukemia is the most common type of cancer in children, and
treatments result in a good chance for a cure. Acute
lymphocytic leukemia can also occur in adults, though the
chance of cure is greatly reduced. About 6,000 cases are
reported in the United States every year 1. Internationally, ALL
is more common in Caucasians than in Africans; it is more
common in Hispanics and in Latin America2. Acute lymphocytic
leukemia occurs when a bone marrow cell develops errors in
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its DNA. The errors tell the cell to continue growing and
dividing, when a healthy cell would normally stop dividing and
eventually die. Signs and symptoms include recurrent
infections, fever, bleeding gums, swollen lymph nodes, fatigue
and organomegaly. We present a case of lymphoblastic
leukemia in subleukemic phase with the total leukocyte count
within normal range and with few atypical lymphocytes.
CASE REPORT
A 7 years old female child admitted with complains of
fever more than a month, fatigue, loss of appetite, yellow
discoloration of eyes and pain in the abdomen. Clinical
examination showed massive hepatomegaly , pallor,
continuous low grade fever and no enlarged lymph nodes.
Investigations showed Hemoglobin 6.8 gm %, total leukocyte
count (TLC) within normal limits and with 10 to 15 % of atypical
lymphoid cells. Initial platelet count was 70,000 /cu.mm, later
dropped to 20,000/cu.mm. Computerized Tomography (CT)
scan abdomen showed hepatomegaly , splenomegaly , minimal
ascites and collection in pouch of douglas (POD). Chest X-ray
showed bilateral minimal pleural effusion. Provisional clinical
diagnoses listed out were wilson’s disease, Non-Hodgkin’s
lymphoma, viral hepatitis and dengue fever. Patient is been
given supportive therapy and the pediatrician was asked to
send daily sample for complete blood picture (CBP). On daily
follow-up with CBP, we could find the atypical lymphoid cells
increasing in number by 5- 10 % with the TLC within normal
limits and bone marrow was advised. On day eight of the
admission, bone marrow was done and the marrow showed
more than 80% of the blasts (Figure 1). Diagnosis of Acute
Lymphoblastic Leukemia (ALL- L2) was given. Further
confirmation and CD-20 was done, which turned out to be
positive in all the cells indicating B- cell origin (Figure 2).
Figure 1:- Bone marrow aspirate showing Lymphoblasts
Figure 2:- CD-20 Positive in bone marrow aspirate smears (Brown Color)
Pandey, et al
DISCUSSION
Acute lymphoblastic leukemia (ALL) is a malignant
(clonal) disease of the bone marrow in which early lymphoid
precursors proliferate and replace the normal hematopoietic
cells of the marrow. ALL may be distinguished from other
malignant lymphoid disorders by the immunophenotype of
the cells, which is similar to B- or T-precursor cells. The
malignant cells of acute lymphoblastic leukemia (ALL) are
lymphoid precursor cells (ie, lymphoblasts) that are arrested
in an early stage of development. This arrest is caused by an
abnormal expression of genes, often as a result of chromosomal
translocations. The lymphoblasts replace the normal marrow
elements, resulting in a marked decrease in the production of
normal blood cells. Consequently, anemia, thrombocytopenia
and neutropenia occur to varying degrees. The lymphoblasts
also proliferate in organs other than the marrow, particularly
the liver, spleen, and lymph nodes. A bone marrow study
provides conclusive proof of ALL3.
Although overall incidence is rare, leukemia is the most
common type of childhood cancer. It accounts for 30% of all
cancers diagnosed in children younger than 15 years.
Epidemiologic studies of acute leukemias in children have
examined possible risk factors, including genetic, infectious,
and environmental, in an attempt to determine etiology. The
environmental risk factors include ionizing radiation, nonionizing radiation, hydrocarbons, pesticides, alcohol use,
cigarette smoking, and illicit drug use. Genetic conditions such
as Down’s syndrome, Neurofibromatosis, Shwachman
syndrome, Bloom syndrome, Ataxia telangiectasia are also
found associated in many studies 4. Children with ALL are often
divided into risk groups such as standard-risk, high-risk, or very
high-risk, with more intensive treatment given to higher risk
patients. Generally, children at low risk have a better outlook
than those at very high risk. While all of the following are
prognostic factors, only certain ones are used to determine
which risk group a child falls into. (The first 2 factors – age at
diagnosis and initial white blood cell count – are thought to
be the most important.) It’s important to know that even
children with some poor prognostic factors can often still be
cured.
Age at diagnosis: Children between the ages of 1 and 9 with
B-cell ALL tend to have better cure rates. Children younger than
1 year and children 10 years or older are considered high-risk
patients. The outlook in T-cell ALL isn’t affected much by age.
Initial white blood cell (WBC) count: Children with ALL
who have very high WBC counts (greater than 50,000 cells per
cubic millimeter) when they are diagnosed are classified as
high risk and need more intensive treatment. Typically, the
higher the white blood cell count, the worse the prognosis5.
Subtype of ALL: Children with pre-B, common, or early pre-Bcell ALL generally do better than those with mature B-cell
leukemia. The outlook for T-cell ALL seems to be about the
same as that for B-cell ALL as long as treatment is intense
enough.
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Gender: Girls with ALL may have a slightly higher chance
of being cured than boys.
Race/ethnicity: African-American and Hispanic children
with ALL tend to have a lower cure rate than children of other
races.
Spread to certain organs: Spread of the leukemia into the
cerebrospinal fluid, or to the testicles in boys, lowers the chance
of being cured. Enlargement of the spleen and liver is usually
linked to a high WBC count, but some doctors view this as a
separate sign that the outlook is not as favorable.
Number of chromosomes: Better prognosis observed in
patients with hyperploidy.
Chromosome translocations: Translocations occur when
chromosomes swap some of their genetic material (DNA).
Children whose leukemia cells have a translocation between
chromosomes 12 and 21 are more likely to be cured. Those
with a translocation between chromosomes 9 and 22 (the
Philadelphia chromosome), 1 and 19, or 4 and 11 tend to have
a less favorable prognosis6. Some of these “poor” prognostic
factors have become less important in recent years as
treatment has improved.
Response to treatment: Children whose leukemia responds
completely within 1 to 2 weeks of chemotherapy have a better
outlook than those whose leukemia does not. Children whose
cancer does not respond as well may be given more intensive
chemotherapy.
The earlier ALL is detected, the more effective the treatment.
The aim is to induce a lasting remision, defined as the absence
of detectable cancer cells in the body (usually less than 5%
blast cells in 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.
CONCLUSION
ALL is one of the commonest cancers of the childhood.
It has good prognosis if diagnosed in the early stages of the
disease. One should not ignore atypical lymphocytes which
give us a clue and indication for bone marrow study. In this
case the total leukocyte count was normal. This patient’s
prognosis was good as the case was diagnosed in subleukemic
phase and patient responded well with chemotherapy.
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Seiter, K (5 February 2014). Sarkodee-Adoo, C; Talavera,
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April 2014.
Please cite this article as: Pandey V, Mahesh K, Indira. Early
diagnosis of childhood Acute lymphoblastic leukemia.
Perspectives in medical research 2017;5(1):83-85.
Sources of Support: Nil,Conflict of interest:None declared.
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