CHAPTER 33
Hemolytic Anemias:
***RBC's have shortened life span # of RBC's circulation is reduced Fewer RBC's result in tissue hypoxia which in turn stimulates EPO being released from the kidney*** **EPO stimulates bone marrow to compensate by the "sickling" process, because vasoconstriction slows the blood flow. O2 delivery can be impaired by an increased blood viscosity, with or without occlusion due to adhesion of sickled cells. ** **Sickle cell anemia is most severe of sickle cell disease. Most benign type.**
Evaluation : Expectec Outcomes of Sickle Cell Patients
**1. Control of pain - acute pain >> analgesics - uses relaxation techniques, breathing exercises, distraction to relieve pain** 2. Is free of infection - Has normal Temp - Shows WBC count within normal range 4500-1100 -Identifies importance of continuing antibiotics at home 3. Expresses improved sense of control - participates in goal setting and planning and implementing daily activities -Participates in decisions about care 4. Increases knowledge about disease process - Identifies situations and factors that can precipitate sickle cell crisis -Describes lifestyle changes needed to prevent crisis - Describes the importance of warmth, adequate hydration, and prevention of infection in preventing crisis - Absence of complications
Acute myeloid Leukemia (AML): Medical Management
**Achieve complete remission, in which there is no evidence of residual leukemia remaining in the bone marrow!!** **Attempts are made by chemotherapy called INDUCTION THERAPY which usually requires hospitalization for several weeks** **Induction involves high doses of Daunorubicin** **Aim of induction is to eradicate the leukemic cells, but is often accompanied by the eradication of normal types of myeloid cells!!! Patient becomes neutropenic, anemic, and thrombocytopenic. ** **During this time, the patient is very ill with bacterial, fungal, and occasional viral infections, bleeding, mucositis which causes diarrhea and marked decline in the ability to maintain adequate nutrition.**
Medical Management of Anemia
**Directed toward controlling and correcting the cause of anemia; if the anemia is severe, the RBC's that are lost or destroyed may be replaced with a transfusion of packed RBC's (PRBC's).**
CML: Medical Mgmt
**Drug called "Gleevec"** Combination of Interferon-alfa (Roferon-A) and cytosine. Administered daily as SUB-Q **Hydroxyurea to reduce WBC counts** **Daunomycin to bring WBC count down** Transformation phase- can be insidious, but it marks process of evolution (transformation) to the acute form of leukemia (blast crisis). **In transformation phase, patient may complain of bone pain and may report fever (without signs of infection) and weight loss.** **Even though spleen enlarges, patient may become anemic and thrombocytopenic, an INCREASED BASOPHIL LEVEL IS DETECTED BY THE CBC.** 25% OF PATIENTS WITH CML WILL TRANSFORM TO ACUTE LYMPHOID LEUKEMIA (ALL) WITH LYMPHOID APPEARING BLASTS. TRANSFORMATION INTO THE ACUTE PHASE CAN BE GRADUAL OR RAPID. **BLAST CRISIS- INDUCTION THERAPY FOR ACUTE LEUKEMIA USING SAME MEDS FOR AML OR ALL. PATIENTS WHO DISEASE EVOLVES INTO "LYMPHOID" BLAST CRISIS ARE MORE LIKELY TO BE ABLE TO REENTER A CHRONIC PHASE AFTER INDUCTION THERAPY.**
Chronic Lymphocytic Leukemia (CLL) Medical Mgmt:
**Early stages- CLL may require no treatment** When symptoms are severe (drenching night sweats, painful lymphadenopathy) or when the disease progresses to later stages (with resultant anemia and thrombocytopenia) chemotherapy with corticosteroids and chlorambucil is often used. Other useful agents: Cyclophosphamide, vincristine, and doxorubicin. Fludarabine has become front-line therapy medication for CLL; Fludarabine has side effect of prolonged bone marrow depression manifested by periods of neutropenia, lymphophenia, and thrombocytopenia. Patients are at risk for such infections as Pneumocystis Carinii, Listeria, mycobacteria, herpes virus, and cytomegalovirus. **Prophylactic use of antiviral agents and antibiotics {Trimethoprim and Sulfamethoxazole)= Septra is important and needs to continue for a minimum of 2 months after the patient stops treatment.** Because bacterial infections are common in patients with CLL, IV treatment with immunoglobulin may be given to selected patients.
Polycythemia Vera: Assessment and Diagnostic Findings
**Elevated RBC mass** Normal O2 sat level **enlarged spleen** ^ WBC and Platelets **EPO normal or slightly low**
Nursing Process: Sickle Cell Crisis: Assessment:
**Emphasis is placed on assessing for pain, swelling, and fever!!** **Respiratory System must be assessed carefully [ lung sounds, O2 sat, signs of cardiac failure, presence and extent of edema]** **Patient should be assessed for dehydration by history of fluid intake and careful examination of mucous membranes, skin turgor, urinary output, and serum creatinine and BUN values. ** Assessed for infectious process Monitor CBC's
Hemolytic Anemias: Clinical Manifestations
**Hemoglobin values between 7-10 g/dL Jaundice in sclerae Bone marrow expands in childhood in order to compensate to offset anemia which leads to enlargement of bones of the face and skull Tachycardia Cardiac murmurs Enlarged heart (cardiomegaly) Dysrhythmias and heart failure.** **Virtually any organ may be affected by thrombosis, but the primary sites involves those areas with slowed circulation, such as the spleen, lungs, and CNS. ** All tissues and organs are constantly vulnerable to microcirculatory interruptions by the sickling process and therefore susceptible to hypoxic damage or true ischemic necrosis. Patients with sickle cell anemia are susceptible to infection [** pneumonia and osteomyelitis***] **Complications of sickle cell anemia include: Infection, stroke, renal failure, impotence, heart failure, and pulmonary hypertension.**
Acute lymphocytic Leukemia (ALL): Clinical Manifestations
**Immature lymphocytes proliferate in the marrow and crowd the development of normal myeloid cells. As a result, normal hematopiesis is inhibited, resulting in reduced number of leukocytes, erythrocytes, and platelets.** Leukocyte counts may be either low or high, but there is always a high proportion of immature cells. **Manifestations of leukemic cell infiltration into other organs are more with ALL than with other forms of leukemia and include pain from an enlarged liver or spleen, bone pain, and headache and vomiting (because of meningeal involvement) inflammation of meninges (thin layer covering the brain)**
Megaloblastic Anemias
**In anemias caused by deficiencies of vitamin b12 or folic acid, identical bone marrow and peripheral blood changes occur, because both vitamins are essential for normal DNA synthesis. ** **RBC's that are produced abnormally large are called **megaloblastic RBC's**
Assessment and Diagnostic Findings of Anemia:
**In initial evaluation: HEMOGLOBIN, HEMATOCRIT, RETICULOCYTE COUNT,AND RBC'S ARE USEFUL. ** IRON STUDIES (serum iron level, TIBC, % saturation, and ferritin), as well as serum vitamin B12 and folate levels are also frequently obtained. Haptoglobin, and erythropoietin levels. **Remaining CBC values are useful in determining whether the anemia is an isolated problem or part of another hematologic condition, such as leukemia or myeldysplastic syndrome (MDS)**
Acute Myeloid Leukemia (AML): Complications
**Include: Bleeding, infection, the MAJOR CAUSES OF DEATH. The risk of bleeding correlates with the level of platelet deficiency (thrombocytopenia)** **Low platelet count can result in ecchymoses (bruises) and petechiae ( pinpoint red or purple hemorrhagic spots on the skin). ** **Major hemorrhages also may develop when the platelet count drops less than 10,000. The most common sites of bleeding are Gastrointestinal, pulmonary, and intracranial. ** **For undetermined reasons, fever and infection also increase the likelihood of bleeding. ** **Patients with leukemia are always threatened by infection!!! Likelihood of infection increased with the degree of duration of neutropenia; neutrophil counts that persist at less than 100 make systemic infection extremely high!!!** **As the duration of severe neutropenia increases, the patient's risk for developing fungal infection also increases.**
Chronic Lymphocytic Leukemia (CLL): Clinical Manifestations
**Increased lymphocyte count always present (lymphocytosis)** RBC's and platelets may be normal, but if later stages are decreased **Enlargement of lymph nodes (lymphadenopathy) is common. Can be severe and painful. ** **Spleen can also be enlarged. ** **Patients with CLL develop "B symptoms" as a constellation of symptoms including fevers, drenching sweating (especially at night) and unintentional weight loss.** **Patients have defects in their humoral and cell-mediated immunity systems; therefore, infections are common. ** The defect in cellular immunity is evidenced by an absent or decreased reaction to skin sensitivity tests (Candida, mumps) which is known as anergy. Problems with life-threatening infections are common; Viral infections such as herpes zoster can become widely disseminated.
AML: Complications of treatment
**Massive leukemic cell destruction from chemotherapy results in release of electrolytes and fluids within the cell to systemic circulation. ** **Increases uric acid levels, potassium, and phosphate!!! This process is called tumor lysis!!!!** **Increased uric acid, potassium, and phosphorous levels are seen. ** **Increased uric acid and phosphorous levels make patients vulnerable to renal stone formation and renal colic, which can progress to acute renal failure. ** **Hyperkalemia and Hypocalcemia can lead to cardiac dysrythmias, hypotension, neuromuscular effects such as muscle cramps, weakness, spasm/tetany, confusion, and seizure. ** **Patients require high fluid intake, alkalization of the urine, and prophylaxis with allopurinol to prevent crystallization of uric acid and subsequent stone formation. ** **GI problems may result from infiltration of abnormal leukocytes into the abdominal organs and from the toxicity of chemotherapeutic agents. Anorexia, nausea, vomiting, diarrhea, and severe mucositis are common. Because of the profound myelosuppressive effects of chemotherapy, significant neutropenia and thrombocytopenia typically result in serious infection and increased risk for bleeding.**
The tongue may be beefy red and sore in what type of anemia?
**Megaloblastic anemia.**
Hypoproliferative Anemias: Iron Deficiency Anemia
**Most common causes in men and postmenopausal women is bleeding (from ulcers, gastritis, inflammatory bowel disease, or GI tumors)**!!!!!! **Most common causes in premenopasual women is excessive menstrual bleeding and pregnancy with inadequate iron supplements. ** **Patients with chronic alcoholism often have chronic blood loss from GI tract, which causes iron loss and eventual anemia. ** **Other causes include iron malabsorption.**
Hypoproliferative : Iron deficiency : Assessment and Diagnostic findings:
**Most definitive method is bone marrow aspiration** Aspirate is stained to detect iron, which is at a low level or even absent. However, few patients with suspected iron deficiency anemia undergo bone marrow aspiration. Strong correlation between lab values measuring iron stores and levels of hemoglobin. After iron stores are depleted (reflected by low ferritin levels), the hemoglobin levels fall **(IRON & HGB DIRECTLY RELATED)** **Diminished iron stores cause small RBC's. ** ** MOST RELIABLE IS FERRITIN AND HEMOGLOBIN VALUES***
Neutropenia
**Neutrophils less than 2000 results from decreased production of neutrophils or increased destruction of these cells. ** Neutrophils are essential in preventing and limiting bacterial infection, both exogenous and endogenous (the GI tract and skin are common endogenous sources). **Risk for infection is NOT only on the severity of the neutropenia ( low neutrophil count), but also on the duration of neutropenia. ** Risk for infection increases proportionately with the decreased neutrophil count. **Risk is significant when the ANC is less than 1000*** **Risk is high when it is less than 500*** **Almost Certain when it is less than 100** **The risk of developing infection increases with the length of time during which neutropenia persists, even if it is fairly mild. Conversely, even a severe neutropenia may not result in infection if the duration of neutropenia is brief, as is often seen after CHEMOTHERAPY***
Hypoproliferative: Nursing Mgmt for Iron Deficiency
**Preventive education is important because iron deficiency anemia is common in menstruating and pregnant woman. ** **Food sources high in iron include organ meats (beef or calf's liver, chicken liver), other meats, beans (black, pinto, and garbanzo), leafy green vegetables, raisins, & molasses. Taking on iron-rich foods with a source of Vitamin C enhances the absorption of iron. ** Nurse encourages patient to continue iron therapy as long as it is prescribed , although they may no longer feel fatigued. **Because iron is best absorbed on an empty stomach, patients should be advised to take the supplement an hour before meals. Most patients can use the less expensive, more standard forms of ferrous sulfate.**
Polycythemia Vera:
**Proliferative disorder in which the myeloid stem cells seem to have escaped normal control mechanisms** Bone marrow is hypercellular and the RBC, WBC, and platelet counts in the peripheral blood are elevated.** RBC elevation is PREDOMINANT*** HCT can exceed 60% **Spleen resumes embryonic function of hematopiesis and enlarges. ** **Overtime, bone marrow may become fibrotic, with a resultant inability to produce as many cells (burns out)** Disease involves myeloid metaplasia with myelofibrosis or **AML in significant proportion of patients;**
Folate deficiency occurs in people who rarely eat?
**Rarely eat uncooked veggies.**
Hodgkin's Disease: Medical Mgmt
**Regardless of stage, management is CURE Treatment is by stage not histologic type** Standard Treatment: Combination chemotherapy for example with doxorubicin, bleomycin, vinblastine, and dacarbazine referred to as ABVD is now the standard treatment Recurrence: High doses of chemo agents followed by autologous BMT or stem cell transplantation (PBSCT), can be very effective in controlling the disease and extending survival time.
Nursing Process: Sickle Cell Crisis: Planning and Goals
**Relief of pain** decreased incidence of crisis enhanced self-esteem and power absence of complications
Acute Myeloid Leukemia (AML)
**Results from defect in the hematopoietic stem cells that differentiates into all myeloid cells. **Monocytes, granulocytes (neutrophils, basophils, eosinophils), erythrocytes, and platelets. **Incidence rises with Age with a peak at 60 yrs.** ***AML is the most common nonlymphocytic leukemia****
Polycythemia vera: Clinical Manifestations:
**Ruddy complexion Splenomegaly (enlarged spleen) Increased Blood Volume ( headache, dizziness, tinnitus, fatigue, paresthesias, and blurred vision) or from increased blood viscosity (angina, claudication, dyspnea, and thrombophlebitis) particularly if the patient has atherosclerotic blood vessels.** **Generalized pruritus (severe itching of skin); which may be caused by ^ histamine release due to the increased numbers of basophils. ** **Erythromelaglia, a burning sensation in the fingers and toes may be reported and is only partially relieved by cooling.**
Aplastic Anemias: Nursing Mgmt:
**Should be assessed carefully for signs of infection and bleeding. Interventions are delineated in the sections on neutropenia and thrombocytopenia.**
Aplastic Anemia: Medical Mgmt:
**T-lymphocytes of patients with aplastic anemia destroy the stem cells and consequently impair the production of RBC, WBC's and platelets. ** **Can be treated with BMT Managed with immunosuppressive therapy [ prevents the patient's lymphocytes from destroying the stem cells]** Corticosteroids are not very useful as an immunosuppresive agent
Megaloblastic: Patients with pernicious anemia develop:
**a smooth, sore, red tongue, and mild diarrhea. Extremely pale especially in the mucous membranes Confusion Paresthesia in extremities (in lower legs & feet) **Difficulty with balance because of damage to spinal cord** Lose position sense (proprioception) Symptoms are progressive, although the course of illness may be marked by spontaneous partial remissions and exacerbations. Without treatment, patients can die after several years, usually from heart failure secondary to anemia.**
Chronic Myeloid Leukemia (CML):
**arises from mutation in the myeloid stem cell. Normal myeloid cells continue to be produced, but there is a preference for immature (blast forms). Therefore, a wide spectrum of cell types exist within the blood, from blast forms through mature neutrophils. ** **"PHILDAELPHIA CHROMOSOME"** **Because there is an uncontrolled proliferation of cells, the marrow expands into the cavities of long bones (ex: femur) and cells are also formed in the liver and spleen (extramedullary hematopiesis) resulting in enlargement of these organs that is sometimes painful. ** **Patients diagnosed with CML in the chronic phase have an overall median life expectancy of 3-5 yrs. During that time, they have very few symptoms and complications from the disease itself. Problems with infections and bleeding are rare. Once the disease transforms to the acute phase (blast crisis), the overall survival time rarely exceeds several months.**
Hemolytic Anemias: Thalassemia:
**characterized by hypochromia: (abnormal decrease in hemoglobin content of RBC's); extreme microcytosis ( smaller-than-normal RBC's), destruction of blood elements (hemolysis) ** **Production of one or more gloublin chains within the hemoglobin molecule is reduced. When this occurs, the imbalance of configuration of the hemoglobin causes it to precipitate in erythroid precursors. This increases the rigidity of the RBC's and thus premature destruction of these cells.**
Acute Myeloid Leukemia (AML): Clinical Manifestations
**most signs and symptoms evolve from insufficient production of normal blood cells. Fever and infection result from neutropenia, weakness and fatigue from anemia, and bleeding tendencies from thrombocytopenia.** **Proliferation of leukemic cells within organs leads to a variety of additional symptoms: pain from an enlarged liver or spleen, hyperplasia of the gums, and bone pain from expansion of marrow.**
Megaloblastic: Even if adequate vitamin B12 and intrinsic factor is present, how else can a deficiency occur?
A deficiency may occur if disease involving the ileum or pancreas impairs absorption. Pernicious anemia which tends to run in families, is primarily a disorder of adults, particularly the elderly. **ELDERLY*** >> the abnormality in the gastric mucosa; the stomach wall atrophies and fails to secrete intrinsic factor. Therefore the absorption of vitamin B12 is significantly impaired. ** patients with pernicious anemia have a higher incidence of gastric cancer***
Acute Lymphocytic Leukemia: ALL
ALL results from an uncontrolled proliferation of immature cells (lymphoblasts) derived from the myeloid stem cell. **The cell of origin is the precursor to the B-lymphocyte in approx 75% of ALL cases. ** **ALL is most common in young children w/ boys affected more than girls; the peak incidence is 4 yrs of age** If relapse occurs, resumption of induction therapy can often achieve second complete remission. Moreover, BMT may be successful even after a second relapse.
Diagnosis for Patients with Anemia:
Activity Intolerance r/t weakness, fatigue, and generalized malaise Imbalanced nutrition, less than body requirements, r/t inadequate intake of essential nutrients Ineffective tissue perfusion r/t inadequate blood volume or HCT Noncompliance with prescribed therapy.
Nursing Process: Sickle Cell Crisis: Diagnosis
Acute pain r/t tissue hypoxia due to agglutination of sickled cells within blood vessels Risk for infection Risk for powerlessness r/t illness-induced helplessness Deficient knowledge r/t sickle cell crisis prevention
AML: Supporting Care consists of:
Administering blood products (RBCs and platelets) and promptly treating infections. **Use of G-CSF [neupogen ] can shorten the period of significant neutropenia by stimulating the bone marrow to produce leukocytes more quickly; these agents do not appear to increase the risk of producing more leukemic cells.** BMT or peripheral blood stem cell transplantation (PBSCT) .
Hypoproliferative : Iron Deficiency: Medical Mgmt
Anemia may be a sign of a curable GI cancer or of uterine fibroid tumors. **Stool specimens should be tested for occult blood. ** **People 50+ should have colonoscopy, endoscopy, or other examinations of GI tract to detect ulcerations, gastritis, polyps, or cancer. ** **Ferrous sulfate, gluconate, and fumarate are available for treating iron deficiency anemia. ** Dextran may be administered IV if iron is needed in larger amounts or if oral iron is poorly. Small doses should be administered to avoid the risk of anaphylaxis. [ Use Z-track method--> inject in buttock]
***NURSING ALERT*****
Any elderly patient whose chief complaint is **back pain**, and who has an elevated protein level, should be evaluated for possible myeloma.
Aplastic Anemia: [bone marrow failure]
Aplastic Anemia is rather a rare disease caused by a decrease in or damage to marrow stem cells, damage to the microenvironment within the marrow, and replace of the marrow with fat. Result and bone marrow aplasia (reduced hematopiesis). Therefore, in addition to severe anemia, significant neutropenia and thrombocytopenia are also seen. [**PANCYTOPENIA**]!!!
Hemolytic Anemias: Thalassemia: Clinical Manifestations
Asymptomatic Normal Hemoglobin levels most of the time Several days after exposure to offending medication, may develop pallor, jaundice, and hemoglobinuria (hemoglobin in the urine)
Hemolytic Anemia: Sickle Cell Anemia: Medical Mgmt
BMT Hyroxyurea Long-term RBC transfusion
Hodgkin's and Non-Hodgkin's: Nursing Management
Chemotherapy or radiation Regardless of all treatment, patient may endure fatigue Risk for infection is significant in these patients, because of defective immune response. Patient's should be screened regularly for second malignancies.
What are the 3 phases of CML:
Chronic Transformation Accelerated (blast crisis)
Multiple Myeloma: Clinical Manifestations
Classic presenting symptom = **BONE PAIN** **Bone pain increased with movement, decreases with rest. Less pain during awakening, but worse during day** **Myeloma secretes a substance out of plasma cells called osteoclast factor that is involved in bone breakdown. ** **Osteoporosis may be seen on bone X-rays. ** **Bone destruction can be severe enough to cause fractures, including spinal fractures, which can impinge on the spinal cord and result in spinal cord compression.**
Chronic Lymphocytic Leukemia (CLL):
Common malignancy of older aduts; two thirds of all patients are older than 60 years of age at diagnosis. **MOST COMMON FORM OF LEUKEMIA**** in US and Europe **Average survival time is 14 years (early stage) and 2.5 years (late stage)**
Aplastic Anemia: Pathophysiology
Congenital (from birth) or acquired, but most cases are idiopathic (without apparent cause). Infections and pregnancy can trigger it, or may be caused by certain medications, chemicals or radiation damage. Agents that regularly produce marrow aplasia include benzene derivatives. Certain toxic materials such as inorganic arsenic and several pesticides have also been implicated for potential causes.
Hypoprofliferative : Vitamin B12 deficiency Laboratory Findings
Decreased Vitamin B12 level; increased MCV
Hypoproliferative: Decreased erythropoietin production (renal dysfunction) lab findings
Decreased erythropoietin level, normal MCV, and MCH; increased creatinine level
Hypoproliferative : Folate deficiency laboratory findings:
Decreased folate level; increased MCV
Hypoproliferative: Iron deficiency laboratory findings
Decreased reticulocytes, iron, ferritin, iron saturation, MCV; increased TIBC
Chronic Lymphocytic Leukemia (CLL): Pathophysiology
Derives from malignant B lymphocytes (T lymphocyte CLL is rare). **In contrast to acute forms of leukemia, most of the leukemia cells in CLL are fully mature. ** **It appears that these cells can escape apoptosis (programmed cell death) with the result of being an excessive accumulation of the cells in the marrow and circulation.** **Antigen CD52 is prevelant on the surface of many of these leukemic B cells.** Early stage- elevated lymphocyte count is seen and can exceed 100,000. Because lymphocytes are small, they can easily travel through small capillaries within the circulation, and the pulmonary and cerebral complications of leukocytosis (as seen with myeloid leukemia) typically are not found in CLL. **(Early Stage)Lymphadenopathy ( disease affecting lymph nodes) occurs as the lymphocytes are trapped within the lymph nodes. Nodes become very large are sometimes painful. Hepatomegaly and Splenomegaly may develop.** **Later stages- anemia and thrombocytopenia may develop. Treatment is typically initiated in the later stages; earlier treatment does not appear to increase survival. Autoimmune complications can also occur at any stage as either autoimmune hemolytic anemia or idiopathic thrombocytopenic purpura (ITP). In the autoimmune process, RES destroys the body's own RBC's or platelets.**
Acute Myeloid Leukemia (AML): Assessment and Diagnostic Findings
Develops without warning with symptoms occurring over a period of weeks or months. **CBC results show a decrease in both erythrocytes and platelets. Although the total leukocyte count can be low, normal or high, the percentage of normal cells is usually vastly decreased. ** **A bone marrow analysis shows an excess of immature blast cells** **Patients with this leukemia often have significant more problems with BLEEDING***, in that they have underlying coagulopathy and higher incidence of Disseminated Intravascular Coagulation (DIC)**
Non-Hodgkin's Lymphomas: Clinical Manifestations
Early stage- virtually absent/minor Late stage- lymphadenopathy is noticeable.
***NURSING ALERT***
Even when the anemia is severe, RBC transfusions should not be used because the patient's body has compensated over time by expanding the total blood volume. Administration of blood transfusions to such patients, particularly those who are elderly or who have cardiac dysfunction, can precipitate pulmonary edema. If transfusions are required, RBC's should be transfused slowly, with careful attention to signs and symptoms of fluid overload*********
Acute Lymphocytic Leukemia (ALL): Medical Management
Expected outcome is complete remission Lymphoid blast cells are typically very sensitive to corticosteroids and to vinca alkaloids; therefore these medications are integral part of the initial induction therapy. **Because ALL frequently invades the CNS, prophylaxis with cranial irradiation or intrathecal chemo [ methotrexate] or both is an integral part of treatment plan.** Treatment protocols for ALL tend to be complex, using a wide variety of chemo agents. **Infections are common, especially viral infections. The use of corticosteroids to treat ALL increases the patient's susceptibility to infection. Patients with ALL tend to have a better response to treatment than patients with AML do. BMT or PBSCT offers a chance for prolonged remission or even cure if the illness recurs after therapy.**
Megaloblastic: Medical Management
Folate deficiency is treated by increasing the amount of folic acid in the diet and administering 1mg of folic acid daily. Folic acid is administered IM ONLY for people with malabsorption problems; After hemoglobin levels return to normal, folic acid replacement can be stopped! Patients with alcoholism should continue to take folic acid as long as they continue alcohol consumption. Vitamin B12 deficiency: Treated by vitamin B12 replacement **more common** due to ineffective absorption or absence of intrinsic factor, replacement is by monthly IM injections of Vitamin B12 usually at a dose of 1000mg. To prevent recurrence of pernicious anemia, Vitamin B12 therapy must be continued for life.
Megalosblastic Anemias: Pathophysiology: Folic Acid Deficiency
Folic acid, a vitamin necessary for normal RBC production, is stored as compounds referred to as folates. Folates are quickly depleted when the dietary intake of folate is deficient (within 4 months) **Folate is found in green veggies and liver.**
Complications of Anemia :
General complications of anemia include **Heart failure Paresthesias<< Pernicious Anemia Confusion**
Hemolytic Anemia: Thalassemia: Nursing Management
Given a list of medications to avoid Splenectomy
Nursing Process: Assessment of Anemia
Health History and physical examination provide important data about the type of anemia involved, the extent and type of symptoms it produces. Weakness, fatigue, and general malaise are common Pallor of the skin and mucous membranes (sclera, oral mucosa)
Potential Complications of Anemia:
Heart failure Paresthesias Confusion
Nursing Process: Sickle Cell Crisis: Collaborative problems and Potential Complications:
Hypoxia, ischemia, infection, poor wound healing leading to skin breakdown and ulcers Dehydration Cerebrovascular Accident (stroke, brain attack) Anemia Renal dysfunction Heart failure, pulmonary hypertension, and acute chest syndrome Impotence Poor Compliance Substance abuse r/t poorly managed chronic pain
Neutropenia: Medical Management
If medication induced, med needs to be stopped!!! Corticosteroids if it is a immunologic disorder **G-CSF can be effective in increasing neutrophil count when cause is decreased production** **Withholding or reducing dose of chemotherapy or radiation may be required if neutropenia is caused by these treatments** **Should the neutropenia be accompanied by a fever, the patient is automatically considered to be infected and usually admitted to the hospital** Cultures of blood, urine, sputum, should be obtained as well as chest radiograph. Broad spectrum antibiotics are initiated as soon as the samples for culture are obtained.
Hodgkin's Disease: Long Term Complications of Therapy
Immune dysfunction Herpes infection Penumococcal sepsis AML MDS Non-Hodgkin's lymphoma Solid tumors
Acute Lymphocytic Leukemia: ALL: Nursing Mgmt
Improving Nutritional Intake: Often reduced because of pain and discomfort associated with stomatitis **Mouth care before and after meals Analgesics before food ** Small frequent feedings of foods that are soft in texture **Low microbial diets are typically prescribed (avoiding uncooked fruits, or vegetables and those without peelable skin)**
Megaloblastic Anemias: Pathophysiology: Vitamin B12 deficiency
Inadequate dietary intake ***Faulty absorption from the GI tract***[ most common] Occurs in conditions such as **Chron's disease**, or after ileal resection or gastrectomy. ** Absence of intrinsic factor as in pernicious anemia. **Without intrinsic factor, orally consumed vitamin B12 cannot be absorbed and RBC production is eventually diminished.**
Polycythemia Vera: Complications
Increased risk for thombosis resulting in CVA (brain attack, stroke) Heart Attack (MI) **thrombotic complications most frequent cause of death** **Bleeding is a complication possibly due to fact that platelets (very large) are somewhat dysfunctional** **Bleeding can occur in the form of nosebleeds, ulcers, and frank GI bleeding**
Aplastic Anemia: Clinical Manifestations
Infection, fatigue, pallor, dyspnea. Purpura (bruising) may develop later and should trigger a CBC and hematologic evaluation if these were not performed initially.
Nursing Management of Megaloblastic Anemia:
Inspection of the skin and mucous membranes. Mild jaundice may be present and is best seen in the sclera without using flourescent lights. Vitiligo (patchy loss of skin pigmentation) and premature graying of the hair are often seen in patients with pernicious anemia. The tongue is smooth, red, and sore. A careful neurologic assessment is important, including tests of position and vibration sense.
Hypoproliferative: Types of anemia
Iron deficiency Vitamin B12 deficiency Folate Deficiency Decreased erythropoietin production (eg, from renal dysfunction) **bone marrow failure** Cancer/Inflammation & Aplastic Anemia
Hodgkin's Disease: Assessment and Diagnostic Findings
Made by means of excisional lymph node biopsy and finding of the **Reed-Sternberg** cell because this disease mocks infection Assess extent of disease (STAGING) Size of spleen and liver Chest X-ray and a CT scan of the chest, abdomen, and pelvis are crucial to identify the extent of lymphadenopathy within these regions Labs: CBC, platelet count, EST, and liver and renal studies Bone marrow biopsy is performed if suspected bone marrow involvement
Multiple Myeloma:
Malignant disease of most mature form of B-lymphocyte (plasma cell) **Plasma cells secrete immunoglobulins, proteins necessary for antibody production to fight infection.**
Multiple Myeloma: Patho
Malignant plasma cells produce increased amount of specific immunoglobulin that is non-functional. **Immunoglobulin secreted by myeloma cells is detectable in the blood/urine and is referred to as the **monclonal protein**. Monoclonial protein- useful marker to monitor extent of disease. Malignant plasma cells secrete certain substances to stimulate the creation of new blood vessels to enhance the growth of these clusters of plasma cells; (angiogenesis)
Acute Lymphocytic Leukemia: ALL: Nursing Mgmt
Managing Mucositis: Maintain moisture in oral cavity Provide comfort Soft toothbrush, or if counts are low, sponge-tipped applicators Rinse only with NS, NS and baking soda, or prescribed solutions. Perineal and Rectal care to prevent skin impairment Stool softeners to increase moisture of bowel movements
Nursing Process: Sickle Cell Crisis: Nursing Interventions
Managing Pain: Analgesics **Joint swollen you want to support and elevate until swelling diminishes** Relaxation techniques; breathing exercises and distraction
Hemolytic Anemias: Clinical Manifestations: Acute Chest Syndrome
Manifested by rapidly falling hemoglobin level, tachycardia, fever, and bilateral infiltrates seen on the chest x-ray. Signs often mimic infection Pulmonary Fat Embolism>> increased secretory phospholipase A2 concentration has been identified as a predictor of impending acute chest syndrome. Managed by: Antibiotic therapy Incentive spirometry Fluid Restriction Corticosteroids Pulmonary Function Monitoring
Chronic Myeloid Leukemia (CML): Clinical Manifestations
Many patients asymptomatic **Leukocytosis is detected by a CBC WBC commonly exceeds 100,000** **High WBC patients may be SOB or slightly confused due to decreased capillary perfusion to the lungs and brain from leukostasis ( excessive amount of WBC's inhibits the blood flow through capillaries)** Patients may complain of enlarged, tender spleen. Some patients may have insidious symptoms: Malaise, anorexia, and weight loss.
Lymphomas
Neoplasms of cells of lymphoid origin. Tumor starts in lymph nodes, but can involve lymphoid tissue in the spleen, GI tract, liver, or bone marrow. Hodgkins and Non-Hodgkins Lymphomas
Neutropenia: Clinical Manifestations
No definite symptoms of neutropenia until the patient becomes infected. **Routine CBC with differential tests, such as those obtained after chemotherapy treatment, can reveal neutropenia before the onset of infection.**
Hypoproliferative: Cancer inflammation lab findings
Normal MCV, MCH, normal or decreased erythropoietin level; increased % of iron saturation, ferritin level, decreased iron, TIBC
Hemolytic Anemia: Sickle cell Anemia Assessment and Diagnostic Findings
Normal hemoglobin= sickle cell trait Normal Hematocrit=sickle cell trait Normal Blood Smear= sickle cell trait Low Hematocrit= sickle cell anemia Sickled cell Blood smear= sickle cell anemia
Non-Hodgkin's Disease: Medical Management
Not aggressive and truly localized- Radiation therapy Aggressive- combination of chemotherapeutic agents given even in early stages Interferon drug of choice - **Rituximab** for low grade lymphomas Central Nervous system involvement- cranial radiation.
Non-Hodgkin's Lymphomas
Originate from neoplastic growth of lymphoid tissue. **Most NHL's involve malignant B-lymphocytes. ** **In contrast to Hodgkin's Disease, the lymphoid tissues involved are largely infiltrated with malignant cells**
Neutropenia Nursing Management
Patient teaching extremely important!!! Patient's at risk for neutropenia should have blood drawn for CBC's **NURSES NEED TO BE ABLE TO CALCULATE ANC!!!!**
Hypoproliferative : Iron deficiency clinical manifestations
Patients with iron deficiency primarily have the symptoms of anemia. If the deficiency is severe or prolonged, they may also have **a smooth, sore tongue, brittle and ridged nails, and **angular cheilosis** ( an ulceration of the corner of the mouth) **Health histories may be significant for multiple pregnancies, GI bleeding, and pica (craving for unusual substances, such as ice, plaster, or craving chalk)**
Nursing Intervention: Sickle Cell
Preventing and Managing Infection: Prescribed antibiotic [ ADHERE TO THERAPY!!!]
Nursing Interventions: Sickle cell
Promoting Coping Skills: Enhance pain management Nursing care that focuses on patient strenghts Providing patient opportunities to make decisions about daily care may increase the patient's feelings of control
Classification of anemias: Hypoproliferative
RBC's usually survive normally, but the bone marrow cannot produce adequate numbers of these cells. The decreased production is reflected in a low reticulocyte count. **Inadequate RBC's may result from marrow damage due to medications or chemicals or from a lack of factors necessary for RBC formation (iron, Vitmain B12, folic acid, erythropoietin)** FOLIC ACID IS A B VITAMIN
Hodgkin's Disease
Rare malignancy More common in men- Early 20, and 50+ **initiates in a single node; spreads by continguous extension along with lymphatic system** Cause known, but viral etiology suspected. **Malignant cell of Hodgkin's disease is the Reed-Sternberg cell, a gigantic tumor that is morphologically unique and is thought to be of immature lymphoid origin. **Pathologic hallmark** and essential diagnostic criterion for Hodgkins Disease** Repeated Biopsies may be required to establish the diagnosis
Polycythemia Vera: Medical Mgmt
Reduce high blood cell mass **Phlebotomy is important part of therapy and can be performed repeatedly to keep the HCT within normal range.** (Removal of 500mL once or twice weekly), to deplete the patient's iron stores, and to disable the RBC manufacturers excessively. **Avoid iron supplements** **^ Uric acid= allopurinol to prevent gout attacks ( defective metabolism of uric acid causes arthritis) ** **Hydroxyurea can be used to suppress bone marrow function, but may increase risk for leukemia**
Polycythemia Vera: Nursing Management
Risk factors for thrombotic complications should be assessed **Patients with history of bleeding to avoid aspirin and aspirin containing meds because they alter platelet function Minimize alcohol intake** For pruritus, the nurse may recommend bathing in tepid or cool water, along with application of cocoa butter- based lotions and bath products
Hemolytic Anemia: Sickle Cell: Medical Mgmt: Transfusion Therapy
Risk include: Iron overload Poor venous access infection ^ viscosity of blood Require daily folic acid replacements Infections must be treated promptly
Nursing Process: Sickle Cell Crisis: Assessment:
Should be assessed for factors that could have precipitated the crisis, such as symptoms of infection or dehydration, or situations that promote fatigue, or emotional stress **Pain levels should always be monitored Pain rating scale Quality of pain (sharp, dull, burning) Frequency of pain Factors that alleviate or aggravate the pain**
Hemolytic Anemias: Clinical Manifestations: Sickle Cell Crisis
Sickle crisis- very painful; results from tissue hypoxia and necrosis due to inadequate blood flow to a specific region or organ Aplastic crisis- results from infection with human parvovirus. Hemoglobin levels fall rapidly and the marrow cannot compensate Sequestration crisis- results when other organs pool the sickled cells.
Megaloblastic: Clinical Manifestations b/w Folic acid and Vitamin B12 deficiency:
Similar deficiencies However, neurologic manifestations of vitamin B12 deficiency do not occur with folic acid deficiency, and they persist if B12 is not replaced. Folic acid: Measured through Red Cell Folate for true folate deficiency. **B12 deficiency: Because the body can compensate very well until the anemia is severe, the typical manifestations of anemia ( weakness, listlessness, and fatigue) may not appear initially. Hematologic effects of deficiency are accompanied by effects on the other organs systems, particularly the GI tract and nervous system.**
Non-Hodgkin's Disease: Assessment And diagnostic Findings
Staging important from CT scan Bone marrow biopsies and cerebrospinal fluid analysis.
NEUTROPENIC PRECUATIONS!!!! HAVE TO KNOW
THOROUGH HAND-HYGIENE MUST BE DONE BY EVERYONE BEFORE ENTERING THE PATIENTS ROOM EACH AND EVERYTIME AND AFTER!!! ALLOW NO ONE WITH A COLD OR SORE THROAT TO CARE FOR THE PATIENT OR TO ENTER THE ROOM, OR COME IN CONTACT WITH PATIENT AT HOME CARE FOR NEUTROPENIC PATIENTS BEFORE CARING FOR OTHER PATIENTS AS MUCH AS POSSIBLE **USE PRIVATE ROOM IF ANC IS < 1000** **ALLOW NO FRESH FLOWERS** **CHANGE WATER IN CONTAINERS EVERY SHIFT (INCLUDE O2 HUMIDIFICATION SYSTEMS Q24HRS)** **MAKE SURE ROOM IS CLEANSED DAILY**
Classifcation of hemolytic anemias:
stem from premature **destruction** of RBC's which results in liberation of hemoglobin from the RBC into the plasma. The increased RBC destruction results in **TISSUE HYPOXIA** which in turn **STIMULATES ERYTHROPOIETIN**. The increased production is reflected in an increased reticulocyte count, as the bone marrow responds to the loss of RBC's. **Released hemoglobin is converted into bilirubin ; which cause bilirubin concentration to rise. ** Hemolysis can result from an abnormality within the RBC itself (sick cell anemia)
Hodgkin's Disease: Clinical Manifestations
usually begins as painless enlargement of one or more lymph nodes on one side of the neck. Painless and firm, but not hard **Most common sites of lymphadenopathy are cervical, supraclavicular, mediastinal nodes involvement of the iliac or inguinal nodes or spleen is much less common** **All organs are vulnerable to invasion by Hodgkin's Disease. Symptoms result from compression of organs by the tumor, such as cough and pulmonary effusion, jaundice, abdominal pain, bone pain. Herpes Zoster infections are common. ** **Fever without chills, drenching sweats, and unintential weight loss ** These are called "B symptoms" ***Mild anemia is THE MOST COMMON HEMATOLOGIC FINDING. *** WBC's may be elevated or decreased. Platelet count is typically normal, unless the tumor has invaded the bone marrow suppressing hematopiesis. Patient's with Hodgkin's disease have impaired cellular immunity as evidenced by an absent or decreased reaction to skin sensitivity.