Leukemia

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Leukemia

Hematopoietic malignancy characterized by unregulated proliferations of leukocytes (white blood cells in circulation)

Leukocytosis > ­↑ level of leukocytes

Classified by stem cell

Lymphoid

Stem cells that produce lymphocytes

Myeloid

Stem cells that produce nonlymphoid blood cells

Classified as acute or chronic

Based on the time it takes for symptoms to evolve & the phase of cell development that is halted

Acute Myeloid Leukemia (AML)

Description

Defect in hematopoietic stem cell that differentiates into all myeloid cells

Monocytes, granulocytes, erythrocytes, & platelets

Any age group affected: infrequently occurs before age 45, peak incidence at age 67

Most common non-lymphocytic leukemia

Prognosis is variable

Survival rates are variable as well

Worse prognosis in those patients who have had preexisting myelodysplastic syndrome, myeloproliferative disorders, or who have received alkylating agents for cancer

Prior to chemo the survival rate (median) was only 6 weeks

While platelet & aggressive chemo improved stats—survival rates remain suboptimal

Cure is possible in some individuals

Either with stem cell transplant or with chemotherapy

Death is usually due to infection or bleeding

Clinical Manifestations

Without warning typically over few weeks

Signs & symptoms result from insufficient production of normal blood cells

Fever & infection result from neutropenia (↓ neutrophil count)

Weakness, fatigue, dyspnea on excretion, & pallor from anemia

Ecchymosis, bleeding, & petechiae from thrombocytopenia

↓ platelet count <5,000/mm3 common

Rare:

Lymphadenopathy

Enlargement of lymph nodes

Splenomegaly

Enlargement of the spleen

Medical Management

Induction therapy (chemotherapy, aggressively)

Vitamin “A”

With the goal to achieve complete remission, when there is no evidence of residual leukemia in bone marrow

Monitor Absolute Neutrophil Count (ANC) for neutropenia: opportunistic infections

The aim for induction therapy is to eradicate leukemic cells; however, this therapy is also accompanied by the eradication of normal types of myeloid cells—making the PT severely neutropenic

An ANC of ZERO is uncommon

Blood product administration (RBC & platelets) & promotion of treating infections

Neupogen (filgrastim)—can shorten the period of significant neutropenia by stimulating the bone marrow to produce leukocytes more quickly

Hematopoietic stem cell transplantation (HSCT)

Another aggressive TX

At times in combination with radiation

Goal of destroying the hematopoietic function of the PT bone marrow

­ risk of infection & graft versus host disease

Chronic Myeloid Leukemia

Description

Mutation in the myeloid stem cell

Normal myeloid cells continue to be produced

Pathologic increase in production of blast cells

Blast forms to mature neutrophils

Uncontrolled proliferation of cells causes the marrow to expand into the cavity of the long bones & cells into organs causing an enlargement (liver & spleen)

BCR-ABL gene present in almost all patients with CML (Section of DNA missing from chromosome 22 translocated to chromosome 9)

Results from chromosomal translocation, DNA is shifted from chromosome 22 to chromosome 9 – specifically on the BCR gene on chromosome 22 & ABL gene on chromosome 9

When BCR & ABL genes fuse they produce abnormal proteins that cause leukocytes to divide rapidly

Uncommon in patients < 20 years of age, incidence increases with age

Average age is 64 years old

10-15% of all leukemia's

Overall median life-expectancy nearly equal to general population

Clinical Manifestations

Leukocytosis

Detected by CBC—performed for another reason

Leukocyte count may exceed 100,000/mm3

Asymptomatic

Short of breath or confusion due to decreased perfusion

Due to extremely high leukocyte count

­↑leukocyte volume inhibits blood flow through capillaries

Splenomegaly, spleen & liver tenderness

Lymphadenopathy is rare

Three stages: chronic, transformation, accelerated or blast crisis

Chronic phase: few symptoms & complications from disease itself & problems with infections & bleeding are rare

Transformation to the acute phase (blast crisis), more difficult to treat

PT develop more symptoms & complications as disease progresses

Medical Management

imatinib (Gleevec) therapy

Oral medication of chemo

Blocks signals within the leukemia cells that express the BCR-ABL protein, preventing chemical reactions that cause the cell to grow & divide

Antacids & grapefruit may limit absorption & large doses of acetaminophen can cause hepatoxicity

Side effects of imatinib >> periorbital edema is common

HSCT

Another treatment option when imatinib taken at conventional doses does not elicit remission or when remission is not maintained—HSCT can be used

PT can be cured with HSCT, younger than 65 and otherwise healthy

PT who receive transplants in the chronic phase tend to have greater chance for cure than those who receive it in the acute phase

Blast crisis: induction therapy

Transformation phase can be rapid or insidious marking the evolution to the acute form of leukemia (blast crisis)

Bone pain & report of fevers (without obvious signs of infection) & weight loss

Even with chemo, spleen may continue to enlarge

PT may become anemic & thrombocytopenic

TX of blast crisis in CML may resemble induction therapy for acute leukemia; using the same medication for AML or ALL

PT whose diseases evolve into blast crisis are likely to be able to reenter chronic phase after induction therapy

Acute Lymphoid Leukemia

Description

Uncontrolled proliferation of immature cells (lymphoblasts) derived from the lymphoid stem cell

Cell of origin is the precursor to the:

B-lymphocyte precursor

75% of cases

T-lymphocyte precursor

25% of cases

ALL is most common in young children (boys> girls)

Peaks at age 4; after 15 years of age relatively uncommon, incidence rises again after age 50

Responsive to treatment

Complete remission achievable

If relapse occurs start treatment with induction—swelling of testis & breasts

Clinical Manifestations

Immature lymphocytes proliferate in the marrow & impede the development of normal myeloid cells, resulting in:

Normal hematopoiesis is inhibited

Reduced number of granulocytes, platelets, erythrocytes

High numbers of immature leukocytes

With leukocytes either low or high

Leukemic cell infiltration > enlarged liver or spleen & bone pain, CNS involvement (PT may experience cranial nerve palsies or headache & vomiting B/C of meningeal involvement)

Medical Management

Based on genetic markers and other risk factors (primarily age)

Chemotherapy

HSCT may be considered during initial remission or postponed until time of relapse

Therapeutic goal at the time of relapse is to reinitiate treatment to obtain a remission & then move quickly to HSCT

HSCT can improve long-term disease-free survival, however it has risks associated with it, including death & long-term complications

imatinib (Gleevec)

Effective in patients with Ph+ (Philadelphia chromosome positive) ALL

Can be used in combination with conventional chemotherapy

Corticosteroids

Lymphoid blast cells are typically very sensitive to corticosteroids; therefore, these medications are an integral part of the initial induction therapy

Corticosteroids are more toxic to lymphoid cells & has better CNS penetration

­↑ the PTs susceptibility to infection; viral infections are common

Chronic Lymphoid Leukemia

Description

Common malignancy of older adults

72 years or older

Most common leukemia in US

Family history

Vietnam war: exposure to Agent Orange

Most leukemic cells in CLL are fully mature: escape apoptosis

Derived from malignant clone of B lymphocytes

The escape of apoptosis can result in excessive accumulation of the cells in the marrow & circulation

Greater risk for developing other cancers, typically bone, lung, and skin

Lymphocytes are small making them easily to travel through small capillaries within the circulation, & the pulmonary & cerebral complication of leukocytosis

Cells accumulate within the lymph nodes & spleen

Autoimmune complications can occur at any stage, wither hemolytic anemia or idiopathic thrombocytopenia purpura

Destroying the body’s own erythrocytes or platelets

PT have greater risk for developing other cancers

Clinical Manifestations

Asymptomatic; incidental finding from another disease treatment or exam

Lymphocytosis

­↑ lymphocyte count

Lymphadenopathy (common)

Severe & sometimes painful

Splenomegaly

“B symptoms”

Fevers

Drenching sweating (especially at night)

Unintentional weight loss

Medical Management

Chemotherapy

HSCT unclear

Leukemia: Nursing Management

Assessment

Health history for subtle symptoms reported by PT prior to physical

Example: dry cough, mild dyspnea, & diminished breath sounds may indicate a pulmonary infection

Monitor lab results, culture results

Including tracking the leukocyte count, ANC, Hct, platelet, creatinine, & electrolyte levels, & coagulation & hepatic function tests

Nursing Diagnosis

Risk for infection, bleeding, or both

Impaired oral mucous membranes

Due to changes in epithelial lining of the GI tract from chemotherapy or prolonged antimicrobial medications

Imbalanced nutrition

Less than body requirements related to hypermetabolic state, anorexia, mucositis, pain & nausea

Acute pain & discomfort

Related to mucositis, leukocyte infiltration of systemic tissues, fever, & infection

Fatigue & activity intolerance

Related to anemia, infection, inadequate nutrition, & deconditioning

Risk for imbalanced fluid volume

Related to renal dysfunction, diarrhea, bleeding, infection, ↑­ metabolic rate, hypoproteinemia, & need for multiple intravenous medications & blood products

Self-care deficits (bathing, dressing, toileting)

Due to fatigue & malaise

Related to pain

Anxiety and grieving

Due to uncertainty about future, anticipatory loss, & altered role functioning

Risk for spiritual distress

Deficient knowledge

About disease process, treatment, complication management, & self-care measures

PTs are at risk for everything because their cells are not working properly

Potential Complications

Infection

Bleeding/DIC

Renal dysfunction

Tumor Lysis Syndrome

Leukemia: Nursing Interventions

Preventing or managing infection and bleeding

Infection prevention

Assess for evidence of infections

Vital signs every 4 hours

Monitor WBC with differential each day

Inspect all sites that may serve as entry ports (IV sites, wounds, skin folds, bony prominences, perineum, oral cavity)

Report fever, chills, diaphoresis, swelling, heat, pain

Obtain cultures prior to antimicrobial treatment

Avoid IM injections, urinary catheters

Educate PT & family

Bleeding prevention

Monitor for risk factors

Educate family & Pt on signs & symptoms & ways to ↓ risk of bleeding

Blood work done daily

Managing mucositis

Assess PT for dehydration, infection, pain & nutritional impairment

Caused by chemotherapy & radiation

Resulting in impaired nutrient absorption

Impaired tissue integrity due to fatigue

Improving Nutritional Intake

Caused by severe oral pain

Often reduced because of pain & discomfort associated with stomatitis

Encourage or provide mouth care before & after meals & administering analgesic agents before eating can increase intake

Warn PT to chew with care when anesthetic agent is used to avoid inadvertently biting the tongue

Easing pain and discomfort

Recurrent fevers are common; usually accompanied by shaking chills (rigors)

Acetaminophen (Tylenol) usually given to ↓ fever, but it also ­↑ diaphoresis—sponging with cold water may help, but cold water & ice pack should be avoided because heat cannot dissipate from constricted blood vessels

Bed clothes should be frequently changed

Mucositis can cause discomfort as well

Decreasing fatigue and activity intolerance

Fatigue is common & an oppressive symptom

Nurses focus on assisting PT to establish a balance between activity & rest

PT are encouraged to do simple activity which can improve the PTs tidal volume & enhance circulation

Physical therapy is also beneficial

Maintaining fluid and electrolyte balance

Febrile episodes, bleeding, & inadequate or overly aggressive fluid replacement can alter PT fluid status

Persistent diarrhea, vomiting, & long-term use of certain antimicrobial agents cause deficits in electrolytes

Intake & output need to be measured accurately, along with daily weight

Assess PT for fluid overload

Improving Self-Care (bathing, dressing, toileting)

Must be done by the nurse when the PT can’t

Encourage PT to persevere their mobility & function as well as self-esteem

Empathetic listening is helpful as well

Realistic reassurance that these deficits are only temporary

Managing anxiety and grief

Assess how much information the PT wants to have regarding the illness, its treatment, & potential complications

Priorities must be identified so that procedures, assessments, & self-care expectations are adequately explained even to those who do not wish extensive information

Ensuring spiritual well-being

Assess PT spiritual & religious views to offer pastoral services

Assist PT to maintain help

Keep hope realistic