Leukemia, Chronic

DRG Category:	841
Mean LOS:	6.4 days
Description:	MEDICAL: Lymphoma and Non-Acute Leukemia With CC

Leukemia is a malignant disease of the blood-forming organs that leads to a transformation of stem cells or early committed precursor cells and thus to an abnormal overproduction of certain leukocytes. Two types of chronic leukemia commonly occur: chronic lymphocytic leukemia (CLL) and chronic myelogenous leukemia (CML).

CLL, the most common form of leukemia in adults in the United States, involves lymphocytes (B cells), which derive from stem cells and circulate among blood, lymph nodes, and lymphatic organs. In CLL, an uncontrollable spread of abnormal, small lymphocytes occurs in the bone marrow, lymphoid tissues, and blood. In CLL, an underproduction of immunoglobulins (antibodies) leads to increased susceptibility to infections. Some patients also develop antibodies to red blood cells and platelets, which then leads to anemia and thrombocytopenia.

CML is characterized by the abnormal overgrowth of myeloblasts, promyelocytes, metamyelocytes, and myelocytes (all granulocytic precursors) in body tissues, peripheral blood, and bone marrow. In CML, the bone marrow becomes 100% cellular (rather than 50% cellular and 50% fat, the normal composition). The spleen enlarges with a greatly expanded red pulp area. CML has two phases: insidious chronic phase and acute phase. In the insidious chronic phase, chronic leukemia originates in the pluripotent stem cell, with an initial finding of hypercellular marrow with a majority of normal cells. After a relatively slow course for a median of 4 years, the patient with chronic leukemia invariably enters a blast crisis, or acute phase.

Both CLL and CML may metastasize to the blood, lymph nodes, spleen, liver, central nervous system, and other organs. The American Cancer Society predicts that approximately 45,000 new cases of all types of leukemia will occur in the United States annually; an estimated 16,000 of those cases will be CLL.

Causes

Several risk factors have been identified, but they do not account for all cases of chronic leukemia. Environmental factors such as exposure to high-dose radiation may increase the risk of CML. In most patients with CML, there is the presence of the abnormal Philadelphia (Ph1) chromosome (see below). Long-term contact with herbicides and pesticides may increase the risk of CLL. Heredity is a factor: Members of the immediate family (parents, siblings, children) who have had CLL create an inherited risk factor. Finally, smoking has been shown to increase the risk of leukemia.

Genetic considerations

Approximately 90% of cases of CML result from the Ph chromosome, which may be induced by radiation or carcinogenic chemicals. Several genetic disorders increase the risk of leukemia. Persons with Down syndrome have a lifetime leukemia risk that is 15 times greater than that of the general population. The heritable disorders Fanconi’s anemia, Bloom’s syndrome, and ataxia telangiectasia also increase the likelihood of leukemia. Somatic mutations creating fusions between BCR and ABL genes cause chronic myeloid leukemia. Several susceptibility loci have also been mapped for chronic lymphocytic leukemia.

Gender, ethnic/racial, and life span considerations

Chronic leukemia affects mostly older adults over age 50. Only about 2% of chronic leukemia patients are children. CLL affects adults, and CML affects mostly adults in the fourth and fifth decades of life; uncommonly, CML affects children and the elderly. Twice as many males as females develop chronic leukemia. There are no racial and ethnic considerations for CML; the incidence of CLL is higher in whites with European ancestry than in blacks/African Americans.

Global health considerations

The global incidence of leukemia is approximately 6 per 100,000 males per year and 4 per 100,000 females per year. The incidence of a leukemia diagnosis is three times higher in developed regions as compared to developing regions. Chronic leukemia, and CLL in particular, is uncommon in people living in Asian countries.

Assessment

History

Often, symptoms of chronic leukemia are nonspecific and vague. In CML, sometimes the first symptom is a dragging sensation caused by extreme splenomegaly, or it may be left upper quadrant pain that is caused by a splenic infarct. In CLL, swollen lymph nodes or enlarged liver and spleen may cause discomfort.

Elicit a history of signs and symptoms such as fatigue and anorexia. Some patients may report any of the following symptoms that are associated with either anemia or increased metabolism because of rapid cell turnover: weakness, weight loss, dyspnea, decreased stamina during exercise, or headache. Ask about bleeding from the gums or nose or easy bruising (signs of thrombocytopenia), abdominal discomfort, or pain in the chest and rib areas. Question the patient about recent weight loss or appetite loss; blood in the urine; or black, tarry stools. Bone and joint tenderness may occur from marrow involvement. Determine if the patient has been running a low-grade fever. Take an occupational history to determine possible exposure to radiation or carcinogenic chemicals.

Physical examination

Observe the patient’s general appearance for pallor and inspect for ecchymoses and bruises. Examine the patient’s eyes for retinal hemorrhage. Palpate the lymph nodes to determine the presence of lymphadenopathy and palpate the abdomen for enlargement of the spleen or liver. Palpate the patient’s thorax for signs of sternal or rib tenderness, which may be indications of infiltration of the periosteum. Inspect the ankles for edema. Note a low-grade fever. Examine the skin for macular to nodular eruptions, signs of skin infiltrations, bruising, and opportunistic fungal infections. Pulmonary infiltrates may appear when lung parenchyma is involved. Assess the patient’s breathing for dyspnea. Auscultate the heart for signs of tachycardia and palpitation.

Psychosocial

Despite advances in treatment and cure, the diagnosis of cancer is an emotionally laden one. Determine the past coping mechanisms used to manage situations of severe stress. Assess the patient’s home situation to determine the possibility of home health care. Assess the support systems available, including emotional, religious, financial, and social.

Diagnostic highlights

Test	Normal Result	Abnormality With Condition	Explanation
Complete blood count and differential	Red blood cells (RBCs) 4–5.5 million/μL; white blood cells (WBCs) 4,500–11,000/μL; hemoglobin 12–18 g/dL; hematocrit 37%–54%; reticulocyte count 0.5%–2.5% of total RBCs; platelets 150,000–400,000/μL	Increased WBC counts; may be only slightly increased or > 200,000/μL; RBCs, decreased; platelets, increased (early in CML) or decreased (CLL and late CML)	Overproduction of WBCs halts production of RBCs and platelets
Bone marrow aspiration/bone marrow biopsy	No leukemia cells present	Leukemic blast phase cells present; leukemic surface markers on cells	Thin needle used to draw up small amount of liquid bone marrow. In biopsy, small cylinder of bone and marrow (about 1/2–in long) is removed. Site of both samples is usually at back of the hipbone

Other Tests: Coagulation studies (prothrombin time, activated partial thromboplastin time), peripheral blood smear, chromosomal analysis, x-rays, computed tomography scan, magnetic resonance imaging, ultrasound

Primary nursing diagnosis

Diagnosis

Risk for infection related to decreased primary and secondary responses

Outcomes

Immune status; Knowledge: Infection control; Risk control; Risk detection; Nutrition status; Treatment behavior: Illness or injury; Hydration; Knowledge: Infection control

Interventions

Infection control; Infection protection; Surveillance; Fluid/electrolyte management; Medication management; Temperature regulation

Planning and implementation

Collaborative

In CLL, because treatments destroy normal cells along with malignant ones, therapy focuses on the prevention and resolution of complications from induced pancytopenia (anemia, bleeding, and infection in particular). When diagnosed, most patients do not require chemotherapy unless they have weight loss of more than 10%, extreme fatigue, fever related to leukemia, or night sweats. Other signs that warrant chemotherapy are progressive bone marrow failure; anemia or thrombocytopenia that does not respond to corticosteroid treatment; or progressive splenomegaly, lymphadenopathy, or lymphocytosis (> 50% in 2 months or doubling of count in less than 6 months). Chemotherapy is therefore employed to reduce symptoms.

Total body irradiation or local radiation to the spleen may also be given as a palliative treatment to reduce complications. Two complications during later stages of CLL are hemolytic anemia (caused by autoimmune disorder) and hypogammaglobulinemia, which further increases the patient’s susceptibility to infection. Antibiotics, transfusions of RBCs, and injections of gamma globulin concentrates may be required for patients with these problems.

Therapy in the chronic phase of CML focuses on (1) achieving hematologic remission (normal complete blood cell count and physical examination without organomegaly), (2) achieving cytogenetic remission (normal chromosomes), and (3) achieving molecular remission (negative polymerase chain reaction result for mutated RNA). Leukapheresis (separation of leukocytes from blood and then returning remaining blood to patient) may be performed to lower an extremely high peripheral leukocyte count quickly and to prevent acute tumor lysis syndrome, but the results are temporary. Platelet pheresis (separation of platelets from blood and then returning remaining blood to patient) may be required for thrombocytosis as high as 2 million. Apheresis (separating blood into components) is usually performed with the use of automated blood cell separators that are designed to remove the selected blood element and return the remaining cells and plasma to the patient.

Allogeneic (belonging to same species) bone marrow transplantations before blast crisis offer the best treatment option. Chemotherapy is used in treating CML, but at this time, it has not proven satisfactory in producing long-term remission. Supportive care and management of complications from chemotherapy are handled pharmacologically with antibiotics, antifungals, and antiviral drugs. Some patients also need transfusions with blood component therapy to control infection and prevent bleeding and anemia. To relieve the pain of splenomegaly, irradiation or removal may be used.

Pharmacologic highlights

Medication or Drug Class	Dosage	Description	Rationale
Prednisone	20–60 mg daily initially, with gradual dose reduction	Corticosteroid	Decreases autoimmune response; manages immune hemolytic anemia or immune thrombocytopenia
Chemotherapy	Varies with drug	CLL: Fludarabine, cyclophosphamide, and rituximab (FCR); Pentostatin, cyclophosphamide, and rituximab (PCR); Fludarabine, cyclophosphamide, and mitoxantrone (FCM); Cyclophosphamide, vincristine, and prednisone (CVP); Cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP)	CLL: Controls symptoms and prevents proliferation of white blood cells. The patient is generally on chemotherapy for 2 wk and off for 2 wk
		CML: Busulfan (Myleran) and hydroxyurea; when a blast crisis occurs, other drugs are used: cytosine arabinoside, omacetaxine, daunorubicin, methotrexate, prednisone, vincristine, imatinib	CML: Can destroy blast cells, prevent leukemic cells from inhibiting formation of normal granulocytes, or transform the blast cells into normal granulocytes

Other Drugs: Interferon therapy, monoclonal antibodies

Independent

Management focuses on providing comfort, support, and patient education and managing complications. Determine how the patient is coping with the disease and where you can best provide support. For some patients, improving their comfort is the highest priority, either physically, such as with a bed bath or back rub, or emotionally, such as by listening to fears and concerns and providing interesting distractions. Teach stress- and pain-reduction techniques. Provide mouth care to lessen the discomfort from oral lesions. Support the patient’s efforts to maintain grooming and a positive body image.

Institute measures to control infection and maintain a safe environment. To limit the risk of bleeding, hold firm pressure on all puncture wounds for at least 10 minutes or until they stop oozing. Limit the use of intramuscular injections and intravenous catheter placement when the patient is pancytopenic. Avoid taking rectal temperatures, using rectal suppositories, or performing a rectal examination.

Assist the patient in minimizing the discomfort of splenomegaly. Provide small, frequent meals. Maintain adequate fluid intake and a high-bulk diet. Prevent constipation. Encourage the patient to cough and perform deep-breathing exercises as a prophylactic for atelectasis.

For patients who are undergoing outpatient chemotherapy, teach about side effects, emphasizing dangerous ones such as bone marrow suppression. Emphasize that the physician should be called in case of a fever over 100°F, chills, redness or swelling, a sore throat, or a cough. Explain the signs of thrombocytopenia. Emphasize that the patient needs to avoid aspirin and aspirin-containing compounds that might exacerbate bleeding. Emphasize the need for adequate rest and the importance of a high-calorie, high-protein diet.

If the patient does not respond to treatment, be honest about prognosis. Implement strategies to manage pain, fever, and infection to ensure the patient’s comfort. Work with the patient, significant others, and chaplain to help the patient plan for a terminal illness and achieve a compassionate death.

Evidence-Based Practice and Health Policy

Efficace, F., Baccarani, M., Breccia, M., Cottone, F., Alimena, G., Deliliers, G. L., …Mandelli, F. (2013). Chronic fatigue is the most important factor limiting health-related quality of life of chronic myeloid leukemia patients treated with imatinib. Leukemia, 27(7), 1511–1519.

An observational study among 422 patients with CML revealed that lower levels of fatigue were significantly associated with better outcomes overall (p < 0.01).
In this sample, fatigue symptoms never occurred in isolation and were highly correlated with musculoskeletal pain (r, 0.51; p ≤ 0.001) and muscular cramps (r, 0.45; p ≤ 0.001).
Fatigue was also significantly associated with job problems (p < 0.001), affecting 34% of the 344 patients who were working at the time of diagnosis.

Documentation guidelines

Physical response: Vital signs, physical assessment, signs of infection, signs of bleeding, ability to tolerate activity
Response to chemotherapy or radiation treatments
Comprehension of treatment plan, including care: Purpose and potential side effects of radiation and chemotherapy; bone marrow transplant
Presence of complications: Infection, bleeding, poor wound healing, ineffective coping by the patient or significant others

Discharge and home healthcare guidelines

Teach the patient and significant others about the course of the disease, the treatment options, and how to recognize complications. Explain that the patient or family needs to notify the physician if any of the following occur: fever, chills, cough, sore throat, increased bleeding or bruising, new onset of bone or abdominal pain. Urge the patient to maintain a realistic but positive attitude. The return to an independent lifestyle is possible with the efforts of a competent healthcare team and the patient’s cooperation. Provide a list of referral agencies as appropriate, such as the American Cancer Society, hospice, and support groups.

Discuss the patient’s home environment to limit the risk of exposure to infections. Encourage the patient to avoid close contact with family pets because they carry infections. The patient should not clean birdcages, litter boxes, or fish tanks. Additional sources of bacteria in the home include water in humidifiers, standing water in flower vases, and water in fish tanks. Encourage the patient to have air filters in furnaces and air conditioners changed weekly. Explain that raw fruits, vegetables, and uncooked meat carry bacteria and should be avoided. If the patient is injured, encourage him or her to apply pressure, use ice to the area, and report excessive bleeding. Teach the patient to avoid blowing or picking the nose or straining at bowel movements to limit the risk of bleeding.

Explain the proper administration and potential side effects of any medications. Teach the patient how to manage pain with the prescribed analgesics and other side effects specific to each chemotherapeutic agent. Explain that the chemotherapy may cause weight loss and anorexia. Encourage the patient to eat a diet high in calories and protein and to drink at least 2,000 mL of fluids per day. If the chemotherapy leads to anorexia, encourage the patient to eat frequent, small meals several times a day. Arrange for a dietary consultation if needed before discharge. If the patient has oral lesions, teach the patient to use a soft toothbrush or cloth and to avoid hot, spicy foods and commercial mouthwashes, which can irritate mouth ulcers.