Acute Lymphocytic Leukemia

Tammy Rascic FNP-S

What is Acute Lymphocytic Leukemia

(ALL)?

A malignant proliferation and accumulation of

lymphocytes that are immature
The following systems are affected:
hematologic, lymphatic, immunologic

Epidemiology

Usually occurs between the ages of 35-40

years, incidence increases with age
Males >Females slightly

Incidence & Etiology

In the United States: 1,000 adult cases per year
Etiology is unknown
Epstein-Barr virus is implicated in Burkitt
leukemia/lymphoma

Risk Factors

Age >60
Incidence increases after exposure to benzene,
radiation (but acute myeloid leukemia is more
common)
Can occur after aplastic anemia

Etiology
Increased incidence in children with Down
Syndrome or in rare familial diseases such as
bloom syndrome or neurofibromatosis
Can rarely occur in adult identical twins

History
Anemia: fatigue, shortness of breath,
lightheadedness, angina, headache
Thrombocytopenia: easy bruising
Neutrocytopenia: fever, infection
Lymphocytosis: bone pain
CNS: confusion

Physical Exam
Anemia: pallor
Thrombocytopenia: petechiae, ecchymoses,
epistaxis, retinal hemorrhages
Neutrocytopenia: fever, infection
Lymphocytosis: lymphadenopathy,
splenomegaly, hepatomegaly (less often)
CNS: cranial nerve palsies, confusion

Diagnostic Tests
CBC with differential, liver function tests, uric
acid, ESR, or C-reactive protein:
Anemia: normochromic, normocytic
Thrombocytopenia
Peripheral blood lymphoblasts
Elevated lactate dehydrogenase
Elevated uric acid

Special Considerations

Pathological findings

Diffuse replacement of marrow and lymph node by malignant lymphoblasts

Imaging

Chest radiograph to check for mediastinal mass or hilar adenopathy and for
pulmonary infiltrates suggestive of infection

Ultrasound exam to check for splenomegaly or renal enlargement suggestive of

leukemic infiltrate

Special tests

Immunophenotyping of marrow/blood lymphoblasts: B-lineage, T-lineage,

common ALL antigen, human leukocyte antigen & more

Cytochemical stains: myeloperoxidase, negative; sudan black B, usually

negative; TdT positive; periodic acid Schiff, variable

Differential Diagnosis
Malignant disorders: other leukemias
especially acute myeloid leukemia, malignant
lymphomas, multiple myeloma, bone marrow
metastases, myelodysplastic syndromes
Nonmalignant disorders: aplastic anemias,
myelofibrosis, autoimmune diseases,
infectious mononucleosis, pertussis,
autoimmune thrombocytopenic purpura,
leukemoid reaction to infection

Treatment
Optimal treatment is not yet known
It should be treated in a comprehensive oncology
center
Treatment regimens for ALL are still
investigational, but effective in some fraction of
patients
Some examples of medications for remission
induction include: cyclophosphamide,
daunorubicin, vincristine, asparaginase,
prednisone, filgrastim, imatinib mesylate

Treatment Continued

Medication for consolidation (repeat twice in 8 weeks): cyclophosphamide,

intrathecal methotrexate, mercaptopurine, cytarabine, vincristine, asparaginase

CNS prophylaxis and interim maintenance: intrathecal methotrexate,

mercaptopurine, oral methotrexate

Late intensification: doxorubicin, vincristine, dexamethasone, cyclophosphamide,

thioguanine, cytarabine

Prolonged maintenance: vincristine, prednisone, mercaptopurine, oral methotrexate

Radiation therapy is sometimes used to treat leukemia that has spread to the brain,
spinal cord, or to the testicles. It also could be used to decrease pain when the
leukemia has spread to a bone if the chemotherapy medication has not helped.
Radiation to the whole body is done as a part of a stem cell transplant

Surgery to place a percutaneous, silastic, double lumen central venous catheter

Additional Treatment
Appropriate health care- ALL can become a fatal
disorder quickly so if it is suspected, patients should be
referred quickly to the appropriate oncology center
Impatient care during remission induction
chemotherapy
Postremission therapy is usually outpatient
Protective isolation from infection
Adequate calcium and vitamin D supplementation to
reduce risk for bone injury from corticosteroids and
avascular necrosis of large joints

Follow Up
Ambulation as tolerated
Patient monitoring
Daily during induction chemotherapy for
metabolic and infectious complications
Weekly during remission consolidation
chemotherapy
Monthly during maintenance therapy
Every 3 months after

Diet
Nutritional support, including IV
hyperalimentation if needed
Avoid alcohol
Calcium and Vitamin D

Patient Education
Risks of infection, transfusion, chemotherapy
should be discussed
Encourage smoking cessation if applicable

Prognosis
80-90% of patients that are less than 60 years
old will receive a complete remission and 3560% will be free of this disease for 5 years
People over 60 years old are less likely to do
well. 80% may have a complete remission
Individuals with unfavorable cytogenic
subtypes should undergo allogenic stem cell
transplantation in the first remission if an
HLA- identical donor were available

Complications

Infections such as P. carinii pneumonia, bacterial pneumonia or sepsis, fungal

pneumonia

Bleeding

Coagulopathy

Need for transfusions

Sterility from treatment

Arachnoiditis and CNS effects from intrathecal chemotherapy and radiation

Pancreatitis and liver dysfunction from chemotherapy

Osteonecrosis of joints related to corticosteroids

Relapse of ALL in marrow or extramedillary sites (CNS, testis)

Reference

Domino,

F. J (2013). The 5-Minute Clinical

Consult 2013. Massachusetts: Wolters Kluwer,
Lippincott Williams & Wilkins.