MYELOMA

• AKA Multiple myeloma, plasma cell neoplasms
• This is a proliferation of neoplastic plasma cells derived from one B lymphocyte and producing a homogeneous immunoglobulin (M protein) without any apparent antigenic stimulation.
• Plasma cells produce osteoclast-activating factor leading to extensive bone loss, severe pain, and pathologic fractures.
• Abnormal immunoglobulin affects renal function, platelet function, resistance to infection, and may cause hyper viscosity of blood.

INCIDENCES

• It accounts approximately 13% of all hematologic malignancies.
• Affecting adults of any age but men are slightly more affected than women
• The mean age at diagnosis is approximately 65–70 years of age.

ETIOLOGY

• Exact cause is unknown.
• Risk factors include:
  • Genetic and environmental factors
    • chronic exposure to low levels of ionizing radiation
    • Exposure to certain exotoxins, e.g. benzene, Agent Orange.
  • Monoclonal gammopathy of unknown significance (MGUS) - a condition in which an abnormal protein — known as monoclonal protein or M protein is in our blood. The protein is produced in plasma cells in our bone marrow.
  • Pernicious anaemia.
  • History of thyroid cancer.

CLINICAL MANIFESTATIONS

• Fatigue and weakness
• Unintentional weight loss.
• Severe bone pain caused by bone lesions and pathologic fractures sites commonly affected include thoracic and lumbar vertebrae, ribs, skull, pelvis, and proximal long bones.
• Vertebral collapse (may lead to spinal cord compression).
• Hypercalcaemia.
• hyperuricemia
• Anaemia.
• Infection.
• Renal impairment.
• Bruising.

DIAGNOSTIC INVESTIGATIONS

• Bone marrow aspiration and biopsy—demonstrate increased number and abnormal form of plasma cells.
• Bloods: peripheral blood smear (normocytic, normochromic anaemia), U&Es, creatinine, LFTs, ESR, CRP, calcium levels, alkaline phosphatase, beta-2 microglobulin.
• Urine and serum analysis for presence and quantity of abnormal immunoglobulin.
• Serum and urine electrophoresis: paraprotein (M protein), Bence Jones proteinuria.
• Skeletal X-ray for bone deformities, e.g. pepper pot skull and generalised skeletal osteopaenia.
• MRI scan may be useful.

COMPLICATIONS

• Spinal cord compression.
• Pathological fracture.
• Hypercalcaemia.
• Acute kidney injury.
• Increased risk of infection.
• Anaemia.

MEDICAL MANAGEMENT

• Patients with "smoldering" multiple myeloma do not require treatment.
• Management of multiple myeloma depends on the age of the patient and their state of health.
• If they are <70/65 years and without significant co-morbidities like renal failure, few bone lesions, and good organ function then they are eligible for autologous bone marrow transplant, which is the most effective treatment. This involves an induction phase using the VAD regimen: vincristine, adriamycin, dexamethasone. After transplant the patient receives long-term therapy with melphalan.
• Patients who are ineligible for autologous bone marrow transplant receive long-term treatment with melphalan and prednisolone.
• Supportive care options:
  • Plasmapheresis to treat hyperviscosity or bleeding.
  • Radiotherapy may be required to treat bone pain and spinal cord compression.
  • Biphosphonates (eg, pamidronate), potent inhibitors of bone resorption, to treat hypercalcemia and alleviate bone pain.
    • administered as IV infusions, generally during 4 or more hours
    • rapid IV administration may cause renal failure
    • Long term use - jaw osteonecrosis
    • Other side effect are transient temperature elevations, hypophosphatemia, hypomagnesemia, hypocalcemia,
  • Allopurinol and fluids to treat hyperuricemia.
  • Hemodialysis to manage renal failure.
  • Surgical stabilization and fixation of fractures.
• Surgical: Kyphoplasty surgery

Kyphoplasty surgery

• A small incision is made in the back
• Through which places a narrow tube using fluoroscopy guide
• Inserts a special balloon through the tube and into the vertebrae,
• Then gently and carefully inflates it.
• As the balloon inflates, it elevates the fracture, returning the pieces to a more normal position.
• It also compacts the soft inner bone to create a cavity inside the vertebrae.
• The balloon is removed and specially designed instruments under low pressure to fill the cavity with a cement-like material called polymethylmethacrylate (PMMA).
• After being injected, the pasty material hardens quickly, stabilizing the bone.

NURSING MANAGEMENT

Acute Pain related to destruction of bone and possible pathologic fractures.

(Controlling Pain)

• Assess for presence, location, intensity, and characteristics of pain.
• Administer pharmacologic agents, as ordered, to control pain.
• Teach the use relxation therapies, such as music therapy, relaxation breathing, progressive muscle relaxation, distraction, and imagery, to help manage pain.
• Assess effectiveness of analgesics and adjust dosage or drug used, as necessary, to control pain.

Impaired Physical Mobility related to pain and possible fracture.

(Promoting Mobility)

• Encourage patient to wear back brace for lumbar lesion.
• Advise the physical and occupational therapy consultation.
• Discourage bed rest to prevent hypercalcemia but ensure safety of environment to prevent pathological fractures.
• Assist patient with measures to prevent injury and decrease risk of fractures.
• Advise avoidance of lifting and straining; use walker and other assistive devices.

Fear related to poor prognosis.

(Relieving Fear)

• Develop trusting, supportive relationship with patient and family members.
• Encourage patient to discuss medical condition and prognosis with health personals.
• Assure patient that you are available for support, to provide comfort measures, and to answer questions.

Risk for Injury related to complications of disease process.

(Monitoring for Complications)

• Report any sudden, severe pain, especially of back, which could indicate pathologic fracture.
• Watch for nausea, drowsiness, confusion, polyuria, which could indicate hypercalcemia caused by bony destruction or immobilization.
• Monitor serum calcium levels.
• Monitor blood urea nitrogen (BUN), creatinine, and urine protein tests to detect renal insufficiency, caused by nephrotoxicity of abnormal proteins in multiple myeloma.
• Encourage the patient to maintain high fluid intake (2 to 3 L/day) to avoid dehydration and prevent renal insufficiency and monitor intake and output, and weigh patient daily.

LYMPHOMA

• The lymphomas are neoplasms of cells of lymphoid origin.
• Lymphomas are malignant disorders of the reticuloendothelial system that result in an accumulation of dysfunctional, immature lymphoid-derived cells.
• These tumors usually start in lymph nodes but can involve lymphoid tissue in the spleen, GI tract (eg, the wall of the stomach), liver, or bone marrow.
• They are classified according to the predominant cell type and by the degree of malignant cell maturity (eg, well differentiated, poorly differentiated, or undifferentiated).
• Lymphomas can be broadly classified into two categories: Hodgkin lymphoma and non-Hodgkin lymphoma (NHL).

HODGKIN'S LYMPHOMA

• Hodgkin's lymphoma originates in the lymphoid system and involves predominantly lymph nodes. Characterized by appearance of "Reed-Sternberg" multinucleated giant cell in tumor.
• Generally spreads via lymphatic channels, involving lymph nodes, spleen, and ultimately extralymphatic sites. May also spread via bloodstream to such sites as GI tract, bone marrow, skin, upper air passages, and other organs.
• Hodgkin lymphoma is a relatively rare malignancy.
• It accounts for about 12% of all lymphomas.
• More common in men than women
• Peaks of incidence: in the early 20s and after 50 years of age.
• Disease occurrence has a familial pattern:
  • First-degree relatives have a higher-than-normal frequency of disease, but the actual incidence of this pattern is low.
• The 4 most common histological subtypes are:
  • Lymphocyte-predominant.
  • Nodular sclerosing.
  • Mixed cellularity.
  • Lymphocyte-depleted.

STAGING

Based on Ann Arbor classification- The principal stage is determined by location of the tumor:

• Stage I - cancer is located in a single region, usually one lymph node and the surrounding area, with not have outward symptoms.
• Stage II - cancer is located in two separate regions, an affected lymph node or lymphatic organ and a second affected area, and that both affected areas are confined to one side of the diaphragm—that is, both are above the diaphragm, or both are below the diaphragm.
• Stage III - cancer has spread to both sides of the diaphragm, including one organ or area near the lymph nodes or the spleen.
• Stage IV - diffuse or disseminated involvement of one or more extralymphatic organs, including involvement of the liver, bone marrow, or nodular involvement of the lungs.

CAUSE

• Exact cause is unknown.
• Risk factors include:
  • Male sex.
  • Infection with Epstein–Barr virus (EBV).
  • Immunosuppression, e.g. HIV patients.
  • Chronic immunosuppressive therapy eg, for renal transplant
  • Exotoxin exposure - in veterans of the military who were exposed to the herbicide Agent Orange.

CLINICAL MANIFESTATION

• Nontender lymphadenopathy.
• Unintentional weight loss.
• Fever (constitutional 'B signs': fever >38°C, night sweats, weight loss).
• Dyspnoea.
• Splenomegaly.
• Hepatomegaly.

DIAGNOSTIC INVESTIGATIONS

• Blood – Peripheral blood Smear, WBC Count, Urea & Electrolyte's, C-Reactive Protein, ESR, lactate dehydrogenase, creatinine,
• Lymph node biopsy—determines type of lymphoma. Histology: Reed–Sternberg cells seen.
• Bilateral bone marrow aspirate and biopsy—determine whether bone marrow is involved.
• Radiographic tests (eg, x-rays, PET scan, CT scan, MRI—detect deep nodal involvement.
• Gallium-67 scan—detects areas of active disease and determine aggressiveness of disease.
• Liver function tests—determine hepatic involvement; liver biopsy - if results abnormal.
• Lymphangiogram—detects size and location of deep nodes involved, including abdominal nodes, which may not be readily seen via CT scan.

COMPLICATIONS

• Increased risk of infection.
• Recurrence and metastasis.
• Increased risk of cardiovascular disease.
• Complications of chemotherapy.
• Neurological complications.

MEDICAL MANAGEMENT

• Conservative: patient education
• Choice of treatment depends on extent of disease, histopathologic findings, and prognostic indicators or depends on Ann Arbor classification
• Radiation therapy.
  • Treatment of choice for localized disease.
  • Areas of body where lymph node chains are located can generally tolerate high radiation doses.
  • Vital organs are protected with lead shielding during radiation treatments.
• Chemotherapy.
  • Initial treatment commonly consists of
  • ABVD regimen of doxorubicin (Adriamycin), bleomycin, vinblastine (Velban), and dacarbazine;
  • MOPP regimen of nitrogen mustard (Mustargen), vincristine (Oncovin), procarbazine, and prednisone.
  • BEACOPP regimen: - bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, procarbazine, prednisolone.
• Three or four drugs may be given in intermittent or cyclical courses with periods off treatment to allow recovery from toxicities.
• Autologous or allogeneic bone marrow or stem cell transplantation.

NURSING MANAGEMENT

Impaired Tissue Integrity related to high-dose radiation therapy.

(Maintaining Tissue Integrity)

• Encourage patient to keep treated area clean and dry.
• Bathing area gently with tepid water and mild soap.
• Avoid rubbing or application of powders, deodorants, lotions, or ointments on treated area.
• Application of heat and cold to treated area.
• Encourage wearing loose-fitting clothes.
• Advise patient to protect skin from exposure to sun, chlorine, and temperature extremes.

Impaired Oral Mucous Membrane related to high-dose radiation therapy.

(Preserving Oral and GI Tract Mucous Membranes)

• Encourage frequent small meals
• Using bland and soft diet at mild temperatures. Avoid extreme food temperatures.
• Advice patient to avoid irritants, such as alcohol, tobacco etc.
• Encourage mouth care at least twice per day
• After meals using gentle flossing, soft toothbrush, and mild mouth rinse.
• Administer or teach self-administration of pain medication or anti-emetic before eating or drinking, if needed.
• Assess for ulcers, plaques, or discharge that may be indicative of superimposed infection.
• For diarrhoea, switch to low-residue diet and administer antidiarrheal, as prescribed.

NON-HODGKIN'S LYMPHOMA

• This is a group of malignancies that are either B cell or T cell in origin.
• Non-Hodgkin's lymphomas are a group of malignancies of lymphoid tissue arising from T or B-lymphocytes or their precursors. It includes both indolent and aggressive forms.
• In the United States B-cell lymphomas represent about 80% of all cases.
• Types of Non-Hodgkin's lymphomas include
• B cell neoplasms
  • Small lymphocytic lymphoma SLL
  • Burkitt's lymphoma: Associated with Epstein–Barr virus (EBV)
  • Diffuse large B cell lymphoma
  • primary cutaneous B-cell lymphoma.
  • Mantle cell lymphoma
  • Follicular lymphoma
• T cell neoplasms
  • Adult T cell lymphoma; caused by human T-lymphotrophic virus-1 (HTLV-1)
  • Sézary syndrome - cutaneous T-cell lymphoma

ETIOLOGY

• Exact cause is unknown.
• Risk factors include:
  • Male sex.
  • Viral Infection, e.g. HTLV-1, EBV, human herpes virus (HHV)-8, hepatitis C.
  • Helicobacter pylori - Helicobacter gastritis (for gastric B-cell lymphoma),
  • Association with defective or altered immune system
    • Immunosuppression, e.g. HIV patients.
    • Patients receiving immunosuppression for organ transplant
  • Family history,
  • White ethnicity,
  • Autoimmune diseases such as rheumatoid arthritis,
  • History of hodgkin's lymphoma,
  • History of radiation therapy,
  • Diet high in meats and fat,
  • Exposure to certain pesticides.

CLINICAL MANIFESTATIONS

• Nontender lymphadenopathy (generally unilateral)
• Unintentional weight loss
• Fever, chills
• Night sweats
• Unexplained pain in chest, abdomen, or bones
• Dyspnoea
• Splenomegaly and Hepatomegaly

DIAGNOSTIC EVALUATION

• Blood – Peripheral blood Smear, WBC Count, Urea & Electrolyte's, C-Reactive Protein, ESR, lactate dehydrogenase, creatinine, alkaline phosphatase, serum cytokine levels, soluble CD25 level.
• Incisional or excisional lymph node biopsy to detect type.
• Bone marrow aspirate and biopsy to detect bone marrow involvement.
• CT scan of the chest, abdomen, and pelvis with oral and intravenous contrast or
• PET with CT scan to detect deep nodal involvement.
• Liver function tests, liver scan to detect liver involvement.
• Hepatitis B testing is recommended due to risk of reactivation.
• Lumbar puncture to detect CNS involvement (for some lymphoma types).
• Surgical staging (laparotomy with splenectomy, liver biopsy, multiple lymph node biopsies).

COMPLICATIONS

• Increased risk of infection.
• Recurrence and metastasis.
• Increased risk of cardiovascular disease.
• Complications of chemotherapy.
• Neurological complications.

MEDICAL MANAGEMENT

• Radiation therapy is generally palliative, not curative.
• Chemotherapy: various regimens including
  • CHOP - cyclophosphamide, doxorubicin (Adriamycin), vincristine (Oncovin), and prednisone
  • BACOP - bleomycin, doxorubicin (Adriamycin), cyclophosphamide, vincristine (Oncovin), and prednisone.
  • CD20 positive lymphomas - Monoclonal antibody therapy: rituximab alone or with chemotherapy to.
• Autologous or allogeneic bone marrow or stem cell transplantation.

NURSING MANAGEMENT

Risk for Infection related to altered immune response because of lymphoma and leukopenia caused by chemotherapy or radiation therapy.

(Minimizing Risk of Infection)

• Care for patient in protected environment with strict handwashing observed.
• Avoid invasive procedures, such as urinary catheterization, if possible.
• Assess temperature and vital signs, breath sounds, LOC, and skin and mucous membranes frequently for signs of infection.
• Notify health care provider of fever greater than 101° F (38.3° C) or change in condition.
• Obtain cultures of suspected infected sites or body fluids.

LEUKOPENIA

• It is a condition in which there are fewer leukocytes than normal, results from neutropenia (diminished neutrophils) or lymphopenia (diminished lymphocytes). Even if other types of leukocytes (eg, monocytes, basophils) are diminished, their numbers are too few to reduce the total leukocyte count significantly.
• In adults leukopenia is a total WBC count <3700 cells/mm3. Most cases result from absolute neutropenia (<2500 cells/mm3); rare cases are secondary to absolute lymphopenia (<1500 cells/mm3).

ETIOLOGY

• Blood cell or bone marrow conditions
  • Aplastic Anemia
  • Hypersplenism or overactive spleen
  • Myelodysplastic syndromes
  • Myeloproliferative syndrome
  • Myelofibrosis
• Cancer like leukemia and treatments for cancer
  • chemotherapy
  • radiation therapy (especially when used on large bones, such as those in your legs and pelvis)
  • bone marrow transplant
• Congenital problems
  • Severe congenital neutropenia (Kostmann syndrome)
  • Myelokathexis
• Infectious diseases
  • HIV or AIDS
  • Tuberculosis
• Autoimmune disorders
  • SLE, systemic lupus erythematosus
  • Rheumatoid arthritis
• Malnutrition - vitamin or mineral deficiencies
  • vitamin B-12
  • folate
  • copper
  • zinc
• Medications
  • clozapine
  • cyclosporine
  • interferons
  • minocycline
  • penicillin
  • sodium valproate
  • steroids
• Viral infections
• Sarcoidosis - by formation of granulomas form in your bone marrow, leukopenia can result.

CLINICAL MANIFESTATIONS

• Insidious onset, may be discovered during routine physical examination.
• Commonly sign and symptoms of infections can appear as:
  • Fever
  • Ulcers
  • Abscesses (collections of pus)
  • Rashes
  • Wounds that take a long time to heal

DIAGNOSTIC INVESTIGATION

• Monitor complete blood count (CBC) and differential daily especially
• absolute neutrophil count [ANC] - ANC less than 1000/mm³ Critical
• lymphocyte count- less than 1500/mm³ Critical
• Globulin, albumin, total protein levels.
• All culture and sensitivity test
• x-ray

MEDICAL MANAGEMENT

• Depending on its cause
• Medication induced, the offending agent is stopped immediately, if possible.
• Underlying neoplasm - can temporarily, but with bone marrow recovery, may improve it. Withholding or reducing the dose of chemotherapy or radiation therapy may be required immunologic disorder - Corticosteroids may be used
• The use of growth factors such as G-CSF or granulocyte-macrophage colony-stimulating factor can be effective when the cause of the neutropenia is decreased production.
• If the Leukopenia is accompanied by fever,
  • The patient is considered to have an infection
  • Admitted to the hospital.
  • Cultures of blood, urine, and sputum, as well as a chest x-ray done
  • Adequate therapy against the infectious organisms, broad-spectrum antibiotics are initiated as soon as the cultures are obtained, The antibiotics may be changed after culture and sensitivity results are available.

NURSING MANAGEMENT

Risk for infection secondary to impaired immunocompetence

• Everyone must perform hand hygiene before entering patient's room each and every time.
• Allow no one with a cold or sore throat come in contact with patient at home.
• Use private room for patient if ANC is < 1000/mm³ or lymphocyte count- < 1500/mm³.
• Change water in containers every shift (include O² humidification systems every 24 hours).
• Ensure room is cleaned daily.
• Provide low-microbial diet.
• Encourage adequate hydration.
• Avoid suppositories, enemas, rectal temperatures.
• Practice deep breathing (with incentive spirometer) every 4 hours while awake.
• Ambulate; wear high-efficiency particulate air (HEPA) filter mask if neutropenia is severe.
• Prevent skin dryness with water-soluble lubricants, especially in high-risk areas (eg, lips, corners of mouth, elbows, feet, bony prominences).
• Provide meticulous total body hygiene daily (preferably with antimicrobial solution), including perineal care after every bowel movement.
• Provide thorough oral hygiene after meals and every 4 hours while awake; warm saline, or salt and soda solution, is effective; avoid use of lemon-glycerine swabs, commercial mouthwashes, and hydrogen peroxide.
• Avoid plastic cannulas for peripheral IVs when ANC is <500/mm³ if possible; a central vascular access device is preferred for long-term or intensive IV therapy.
• Inspect IV sites every shift; monitor closely for any discomfort; erythema may not be present.
• Maintain meticulous IV site care.
• Cleanse skin with antimicrobial solution before venepuncture (unless patient is allergic).
• Moisture-vapor–permeable dressings are permissible with strict adherence to institutional protocol.
• Change IV tubing per institution policy, using aseptic technique.
• Administer antimicrobial agents on time.

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