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Lymphoblastic
Leukemia
Definition
Neoplastic disease which results from a
mutation in a single lymphoid progenitor
cell at one of several discrete stages of
development
B Cell or T Cell
Epidemiology
Most common childhood acute leukemia,
~80%
Incidence in adults ~20%
Bimodal distribution of occurrence:
Peak at age 2-5
Second increased incidence after age 50
Pathogenesis
Acquired Genetic Change in Chromosome
Change in number, ie ploidy
Change in structure
Translocations (most common)
Inversions
Deletions
Point mutations
Amplifications
Changes in normal means of cell differentiation, proliferation, and
survival
Mechanisms of Leukemia Induction
1 – Activation of a proto- 2 – Loss or inactivation
oncogene OR creation of of ≥ 1 tumor
a fusion gene with suppressor gene
oncogenic properties - p53 (p16 mutation)
- Ph Chromosome t(9;22)
Etiology
Unknown
? Genetic Predisposition
Increased incidence amongst monozygotic and dizygotic twins
Down Syndrome
Disorder with chromosomal fragility:
Fanconi’s anemia
Bloom Syndrome
Ataxia-Telangiectasia
? Infections
HTLV1 in T cell leukemia/lymphoma
EBV in mature B cell ALL
HIV in lymphoproliferative DO
Presentation
Nonspecific Symptoms
Fatigue/decreased energy
Fever
Easy bruising
Bleeding
Dyspnea
Dizziness
Infection
Joint, extremity pains
CNS involvement
Clinical Presentation
Physical Exam Lab Abnormalities
Pallor anemia
Ecchymoses wbc vary
Petechiae 0.1 (20-40%) - >100 k
(10-16%)
LAD
Platelets – usually ↓
Hepatosplenomegaly ↑ LD, uric acid
CXR: eval for thymic mass
CSF to eval for
involvement
Diagnosis
Morphologic
French American British Classification
L1: small uniform blasts (pediatric ALL)
L2: larger, more variable sized blasts (adult ALL)