LEUKAEMIA

Leukaemia is a group of malignant disorders of the blood and bone marrow that usually begin in the bone marrow and result in an accumulation of cells high numbers of dysfunctional, immature white blood cells.

Classification

• They are classified as acute or chronic based on the development rate of symptoms, and further classified by the predominant cell type.

ACUTE LEUKAEMIAS

• It is characterize by a rapid increase in the number of immature blood cells with rapid progression of symptoms.
• Acute lymphocytic leukaemia (ALL)- When lymphocytes are the predominant malignant cell, the disorder is;
• Acute Myeloid/Myelogenous Leukemia (AML) - when monocytes or granulocytes are predominant, it is, sometimes called acute nonlymphocytic leukemia.
• Biphenotypic Leukemia - is an acute leukemia with both lymphocytic and myelogenous cell characteristics.

CHRONIC LEUKEMIA

• It is characterized by the excessive and gradual accumulation of relatively mature, but still abnormal, white blood cells.
• Typically, it will take months or years to progress.
• The cells are produced at a much higher rate than normal, resulting in many abnormal white blood cells.
• Chronic leukemia mostly occurs in older people, but can occur in any age group.
• Whereas acute leukemia must be treated immediately, chronic forms are sometimes monitored for some time before treatment to ensure maximum effectiveness of therapy.
• Chronic lymphocytic leukaemia (ALL)- When lymphocytes are the predominant malignant cell.
• Chronic Myeloid/Myelogenous Leukemia (AML) - when monocytes or granulocytes are predominant.

Acute lymphocytic leukaemia (ALL)

Acute lymphocytic leukemia (ALL) results from an uncontrolled proliferation of immature cells (lymphoblasts) derived from the lymphoid stem cell.

INCIDENCES

• The cell of origin is the precursor to the
  • B lymphocyte in approximately 75% of ALL cases;
  • T-lymphocyte ALL occurs in approximately 25% of cases.
• ALL is most common in young children.
• Boys affected more often than girls
• The peak incidence is 2 to 9 years of age (4 year).
• After 15 years of age, it is relatively uncommon.

ETIOLOGY

• Exact cause unknown.
• Possibly a genetic susceptibility coupled with an environmental trigger
• Risk factors include:
  • Exposure to ionizing radiation.
  • Exposure to certain chemicals and toxins (eg, benzene, alkylating agents).
  • Myeloproliferative disease
  • Familial susceptibility.
  • Genetic disorders (eg, Down syndrome, Fanconi's anemia).

CLINICAL MANIFESTATIONS

• Most signs and symptoms of ALL result from insufficient production of normal blood cells.
• Fever and recurrent infection due to neutropenia,
• Pallor, fatigue, weakness, Night sweats, weight loss due to anemia, and
• Abnormal bleeding and bruising tendencies due to thrombocytopenia.
• Additional symptoms are
  • Splenomegaly & hepatomegaly
  • Headache & neurologic dysfunction.
  • Hyperplasia of the gums, and
  • Bone and joint pain from expansion of marrow.

DIAGNOSTIC INVESTIGATIONS

• Bloods: peripheral blood smear, WBC Count, platelets Count, Urea and electrolytes (U&Es), LFTs, ESR, C-Reactive protein.
• Bone marrow biopsy, lymph node biopsy.
• Radiology: X-ray, ultrasound scan, CT scan, MRI.

COMPLICATIONS

• Death.
• Increased risk of infection.
• Haemorrhage: pulmonary, intracranial.
• Depression.
• Complication of chemotherapy.

MANAGEMENT

• Conservative - Patient education
• Childhood ALL is usually cured with chemotherapy alone (>75%), whereas only 30% to 40% of adults with ALL are cured.
• High-dose chemotherapy given as an induction course to obtain disappearance of abnormal cells in bone marrow and blood and then in cycles as consolidation or maintenance therapy to prevent recurrence of disease
• To induce remission:
  • Dexamethasone
  • Vincristine
  • Anthracycline antibiotics
  • Cyclophosphamide
• Maintenance:
  • Methotrexate
  • Mercaptopurine
  • Cytarabine
  • Hydrocortisone

Acute Myeloid Leukemia

AML results from a defect in the hematopoietic stem cell that differentiates into all myeloid cells: monocytes, granulocytes (eg, neutrophils, basophils, and eosinophils), erythrocytes, and platelets.

INCIDENCES

• AML is the most common nonlymphocytic leukemia.
• AML is one of the most common types of leukemia among adults. Approximately 85% of acute leukemias in adults are AML.
• AML is more common in men than women.
• AML is a disease of older people, all age groups are affected, although it infrequently occurs before age 40 and the incidence rises with age, with a peak incidence at age 67 years (ACS, 2008b).
• The prognosis is highly variable. Patient age is a significant factor; patients who are younger may survive for 5 years or more after diagnosis of AML. However, patients who are older have a worse prognosis.
• Death usually a result of infection or haemorrhage.

ETIOLOGY

• Exact cause unknown.
• Risk factors include:
  • Exposure to ionizing radiation.
  • Exposure to certain chemicals and toxins (eg, benzene, alkylating agents).
  • Myeloproliferative disease
  • Familial susceptibility.
  • Genetic disorders (eg, Down syndrome, Fanconi's anemia).

CLINICAL MANIFESTATIONS

• Most signs and symptoms of AML result from insufficient production of normal blood cells.
• Fever and recurrent infection due to neutropenia,
• Pallor, fatigue, weakness, Night sweats, weight loss due to anemia, and
• Abnormal bleeding and bruising tendencies due to thrombocytopenia.
• Additional symptoms are
  • Splenomegaly & hepatomegaly
  • Headache & neurologic dysfunction.
  • Hyperplasia of the gums, and
  • Bone and joint pain from expansion of marrow.

DIAGNOSTIC INVESTIGATIONS

• Bloods: peripheral blood smear, WBC Count, platelets Count, Urea and electrolytes (U&Es), LFTs, ESR, C-Reactive protein.
• Bone marrow biopsy, lymph node biopsy.
• Radiology: X-ray, ultrasound scan, CT scan, MRI.

COMPLICATIONS

• Death.
• Increased risk of infection.
• Haemorrhage: pulmonary, intracranial.
• Depression.
• Complication of chemotherapy.

MANAGEMENT

• Conservative - Patient education
• High-dose chemotherapy given as an induction course to obtain disappearance of abnormal cells in bone marrow and blood and then in cycles as consolidation or maintenance therapy to prevent recurrence of disease
  • Patients <60 years: chemotherapy with an anthracycline and cytarabine or methotrexate
  • Patients >60 years: palliative anthracycline, cytarabine or mitoxantrone
• If M3 type AML, i.e. acute promyelocytic leukaemia (APML), then add all-trans retinoic acid to the therapeutic regime

All-trans retinoic acid (Retinoid) - Dry skin and mucous membranes, headaches, eyesight changes, bone pain, flulike symptoms, bone marrow suppression, retinoic acid syndrome (includes weight gain, peripheral edema, dyspnea, and fever)

Cytarabine (Antimetabolite) - Bone marrow suppression, nausea and vomiting, pulmonary toxicity, mucositis, lethargy, cerebellar toxicity, dermatitis, keratoconjunctivitis

Daunorubicin (Antibiotic) - Bone marrow suppression, nausea and vomiting, alopecia, cardiotoxicity, vesicant

Doxorubicin (Antibiotic) - Leukopenia, nausea and vomiting, alopecia, cardiotoxicity, photosensitivity, vesicant

 

Chronic Lymphocytic Leukemia

Chronic lymphocytic leukemia (CLL) (ie, involving more mature cells than acute leukemia) is characterized by proliferation of morphologically normal but functionally inert B-lymphocytes. In CLL the abnormal lymphocytes are found in the bone marrow and blood,

Whereas in small lymphocytic lymphoma (SLL) the same abnormal lymphocytes are found predominantly in lymph nodes.

INCIDENCES

• CLL is typically derived from a malignant clone of B-lymphocytes (T-lymphocyte CLL is rare).
• CLL is a common malignancy of older adults. (Usually affects adults over 60 years old)
• The average age at diagnosis is 72 years (ACS, 2007).
• CLL is the most common form of leukemia in the United States and Europe.
• Rarely seen in Native Americans and infrequently among persons of Asian descent.
• Early-stage CLL - On average survival 15 years.
• Late-stage CLL - On average survival may be as low as 3 to 4 years.
• Positive ZAP-70 marker is associated with a worse prognosis

ETIOLOGY

• Exact cause unknown.
• Risk factors include:
  • Exposure to ionizing radiation.
  • Myeloproliferative disease
  • Familial susceptibility.

CLINICAL MANIFESTATIONS

• Usually Asymptomatic
• Insidious onset; may be discovered during routine physical examination.
• Early symptoms may include
  • Nontender lymphadenopathy (commonly in cervical area)
  • History of frequent skin or respiratory infections,
  • Mild splenomegaly and hepatomegaly,
  • Fatigue.
• Advanced disease symptoms include
  • Malaise, fever, night sweats, weight loss, pallor
  • Activity intolerance,
  • Easy bruising,
  • Skin lesions,
  • Bone tenderness,
  • Abdominal discomfort.

DIAGNOSTIC INVESTIGATIONS

• Bloods: peripheral blood smear, WBC Count, platelets Count, Urea and electrolytes (U&Es), LFTs, ESR, C-Reactive protein.
• Bone marrow aspiration and biopsy: lymphocytic infiltration of bone marrow.
• Lymph node biopsy to detect spread.
• Radiology: X-ray, ultrasound scan, CT scan, MRI.

MANAGEMENT

• Patient with newly diagnosed and indolent CLL is generally observed and followed closely until symptoms develop.
• Treatment is individualized and various chemotherapy and monoclonal antibody combinations may use.
• Lymphocyte proliferation suppression – chlorambucil, cyclophosphamide, prednisone
  • Monoclonal antibodies - Alemtuzumab & Rituximab
• Hairy cell leukemia, a distinctive type of B-cell leukemia with hairlike projections of cytoplasm from lymphocytes, may be successfully treated with cladribine, pentostatin, or alpha interferon.
• Splenic irradiation or splenectomy for painful splenomegaly or platelet sequestration, hemolytic anemia.
• Irradiation of painful enlarged lymph nodes.
• Allogeneic bone marrow transplant is also used to treat CLL.
• Supportive Care
  • Transfusion therapy to replace platelets and RBCs.
  • Antibiotics, antivirals, and antifungals, as needed, to control infections.
  • IV immunoglobulins or gamma globulin to treat hypogammaglobulinemia.

Chronic Myelogenous Leukemia

Chronic myelogenous leukemia (CML) (ie, involving more mature cells than acute leukemia) is characterized by proliferation of myeloid cell lines, including granulocytes, monocytes, platelets, and, occasionally, RBCs.

ETIOLOGY

• Specific etiology unknown,
• Associated with exposure to ionizing radiation and family history of leukemia. Results from malignant transformation of pluripotent hematopoietic stem cell.
• First cancer associated with chromosomal abnormality (the Philadelphia [Ph] chromosome), present in more than 90% of patients.
• Accounts for 25% of adult leukemias and less than 5% of childhood leukemias. Generally presents between ages 25 to 60 with peak incidence in the mid-40s.
• May progress to an accelerated phase or blast crisis, resembling an acute leukemia.

CLINICAL MANIFESTATIONS

• Insidious onset, may be discovered during routine physical examination.
• About 70% of patients have symptoms at diagnosis such as
  • Fatigue, pallor, Malaise
  • Activity intolerance,
  • Fever, weight loss, night sweats,
  • Abdominal fullness (Hepatosplenomegaly).

DIAGNOSTIC INVESTIGATIONS

• Bloods: peripheral blood smear, WBC Count, platelets Count, Urea and electrolytes (U&Es), LFTs, ESR, C-Reactive protein.
• Bone marrow aspiration and biopsy: hypercellular, usually demonstrates Philadelphia (Ph1) chromosome.
• Radiology: X-ray, ultrasound scan, CT scan, MRI.

MANAGEMENT

• Conservative - Patient education
• High-dose chemotherapy given as an induction
• Imatinib (biological therapy) 
• Patients <60 years may be considered for allogeneic stem cell transplantation
• Other treatments
  • Include alpha-interferon,
  • Vincristine,
  • Prednisolone,
  • Cytarabine
  • Daunorubicin
• In the acute form of CML (blast crisis), treatment may resemble induction therapy for acute leukemia, using the same medications as for AML or acute lymphocytic leukemia.

NURSING MANAGEMENT

NURSING DIAGNOSiS

• Risk for infection and bleeding
• Risk for impaired skin integrity related to toxic effects of chemotherapy, alteration in nutrition, and impaired mobility
• Impaired gas exchange
• Impaired mucous membranes due to changes in epithelial lining of the GI tract from chemotherapy or prolonged use of antimicrobial medications
• Imbalanced nutrition, less than body requirements, related to hypermetabolic state, anorexia, mucositis, pain, and nausea
• Acute pain and discomfort related to mucositis, leukocyte infiltration of systemic tissues, fever, and infection
• Hyperthermia related to tumor lysis or infection
• Fatigue and activity intolerance related to anemia, infection, and deconditioning
• Impaired physical mobility due to anemia, malaise, discomfort, and protective isolation
• Risk for excess fluid volume related to renal dysfunction, hypoproteinemia, and need for multiple IV medications and blood products
• Diarrhea due to altered GI flora, mucosal denudation, and prolonged use of broad-spectrum antibiotics
• Risk for deficient fluid volume related to potential for diarrhea, bleeding, infection, and increased metabolic rate
• Self-care deficit due to fatigue, malaise, and protective isolation
• Anxiety due to knowledge deficit and uncertainty about future
• Disturbed body image related to change in appearance, function, and roles
• Grieving related to anticipatory loss and altered role functioning