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INTRODUCTION

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Epidemiology

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Epidemiology
Incidence:

6,020 (male: 3,140; female: 2,880. Estimated new cases for 2014 in the United States)

1.7 per 100,000 male and female per year (1.9 per 100,000 male, 1.5 per 100,000 female)

Deaths: Estimated 1,440 in 2014 (male: 810; female: 630)
Median age:

B-cell lymphoblastic leukemia/lymphoma: 12 years

T-cell lymphoblastic leukemia/lymphoma: 18 years

Lymphoblastic leukemia/lymphoma unknown lineage: 12 years

Age-specific incidence patterns are characterized by a peak between the ages of 2 and 4 years and again during the sixth decade

Male to female ratio: 1.33

Dores GM. Blood 2012;119:34–43

Siegel R et al. CA Cancer J Clin 2014;64:9–29

Surveillance, Epidemiology and End Results (SEER) Program, available from http://seer.cancer.gov (accessed in 2013)

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Work-up

  1. H&P

  2. CBC, leukocyte differential, platelets, electrolytes, liver function tests, PT, PTT, fibrinogen, LDH, uric acid

  3. Bone marrow biopsy/aspirate

  4. HLA typing for patients who are candidates for allogeneic hematopoietic cell transplantation

  5. Cardiac scan if prior cardiac history or prior anthracycline use

  6. CT/MRI of head if neurologic symptoms

  7. CT chest for T-ALL patients

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Pathology

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Pathology

Common Chromosomal and Molecular Abnormalities in ALL

Cytogentics Gene Frequency in Adults Frequency in Children
Hyperdiploidy  7% 25%
Hypodiploidy  2%  1%
t(9;22)(q34;q11) Philadelphia chromosome (Ph) BCR-ABL1 25%  3%
t(12;21)(p13;q22) TEL-AML1  2% 22%
t(v;11q23), e.., t(4;11), t(9;11), t11;19) MLL 10%  8%
t(1;19) E2A-PBX1  3%  5%
t(5;14)(q31;q32) IL3-IGH <1% <1%
t(8;14), t(2;8), t(8;22) c-MYC  4%  2%
t(1;14)(p32;q11) TAL-1 12%  7%
t(10;14)(q24;q11) HOX11  8%  1%
t(5;14)(q35;q32) HOX11L2  1%  3%

Abnormalities observed exclusively in T-cell lineage ALL; all other occur exclusively or predominately in B-cell linage ALL

 

Prognostic Factors for Risk Stratification of Adult ALL
Characteristics High Risk Factors
Age >35 years
Leukocytosis

>30,000 mm3 (B lineage)

>100,000 mm3 (T lineage)

Karyotype t(9;22), t(4;11) (q21;q23), complex, hypodiploid
Therapy response

Time to morphology CR >4weeks

Persistent minimal residual disease (MRD)

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Expert Opinion

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Treatment regimens for acute lymphoblastic leukemia (ALL) have evolved empirically into complex schemes that use numerous agents in various doses, combinations, and schedules, and few of the individual components have been tested rigorously in randomized trials. However, the backbone of chemotherapy for ALL remains the sequence of induction, consolidation, and maintenance

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Remission induction: Most regimens include steroids, vincristine, an anthracycline, and usually asparaginase. Cyclophosphamide and cytarabine are often added. The combination of these agents results in CR rate to 80–90%; thus, treating physicians should use a regimen with which they are familiar and have experience in providing supportive care. See table below for different adult ALL regimens

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Consolidation: Eradication of minimal residual disease during hematologic remission is the primary aim of the consolidation phase. It is difficult to assess the value of individual components of treatment because the number, schedule, and combination of antineoplastic drugs vary considerably among studies. Consolidation therapy typically consists of several ...

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