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L2 lymphoblasts are larger cells with a moderate amount of cytoplasm, dispersed chromatin,
and multiple nucleoli (picture 4 and picture 2). In some studies, L2 has been associated with
worse prognosis than has L1 [44,46]. However, when patients are stratified according to age,
sex, and initial WBC, differences in prognosis between L1 and L2 are no longer observed [45].
Eleven to 14 percent of cases of ALL in children are classified as FAB L2 [44,45].
or
hypodiploidy
(fewer
than
45
Several
abnormalities,
including
hepatic
dysfunction,
coagulation
abnormalities,
hypercalcemia, hypocalcemia, hyperkalemia, and hyperphosphatemia, may be noted [38].
Diagnosis The diagnosis and classification of leukemia are based upon specialized tests
that are performed on cells derived from a bone marrow aspirate or tissue biopsy specimens.
When clinical circumstances preclude bone marrow examination, the diagnosis can be made
from cells obtained from the peripheral blood or pleural effusions. The exact peripheral blood
minimum blast percentage has also not been defined for an accurate diagnosis in pediatrics.
The diagnosis of CNS leukemia requires one of the following:
Cytologic confirmation of the presence of leukemic cells in the cerebrospinal fluid (CSF)
Clinical signs of CNS leukemia such as facial nerve palsy, brain/eye involvement, or
hypothalamic syndrome
A tumor mass involving the CNS as determined by imaging studies
ALL can present clinically as either a mass lesion or as leukemia. Although the distinction in
some patients is arbitrary, ALL is the preferred term in the US when the bone marrow contains
more than 25 percent lymphoblasts, whereas lymphoma is the preferred term when the
process is confined to a mass lesion with minimal or no blood and bone marrow involvement
[70].
CNS involvement is often categorized into three groups:
CNS1 no blasts in the CSF
CNS2 <5 blasts in the CSF with or without RBC
CNS3 >5 blasts in CSF
It was initially thought that patients with low levels of CSF leukemia involvement (CNS2) did
as well as those with no CNS disease. However, with improvement in current treatment
regimens, it has become evident that patients with only low levels of leukemia have a lower
event-free survival, largely due to an increased rate of CNS relapse [71]. Although
controversial, many institutions elect to treat patients with CNS2 disease with more
aggressive CNS regimens.
Differential diagnosis As noted above, the presenting signs and symptoms of ALL are
nonspecific. A variety of malignant and nonmalignant conditions must be considered in the
differential diagnosis. They include [38,72]:
Juvenile idiopathic arthritis (see appropriate topic reviews)
Osteomyelitis
Epstein-Barr virus
Immune thrombocytopenia (ITP)
Pertussis, parapertussis
Aplastic anemia