HEMATOLOGY 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

Hemolytic Anemia: Sickle Cell Medical MGMT Supportive Therapy

**Adequate hydration** **IV hydration w/ D5W or D5 in 0.25 normal saline solution is usually required for sickle cell crisis** O2 therapy may also be needed **Aspirin may be needed for mild to moderate pain**

Alcohol's relationship with folic acid

**Alcohol increases folic acid requirements, and at the same time, patients with alcoholism usually have a diet that is deficient in the vitamin.**

MDS: Medical Management

**Allogenic BMT** CURE RBC transfusions required to control the anemia and symptoms {**watch for iron overload}** EPO reduces the need for transfusions Platelet transfusions for bleeding **Manage infections** **G-CSF, EPO, or both has been successful in increasing neutrophils and diminishing anemia in certain patients.**

MDS: Assessment and Diagnostic Findings

**CBC reveals macrocytic anemia** **WBC and platelets diminished** **EPO may be low**

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).**

Anemias in Renal Disease: The availability of Epogen and Procrit

**Dramatically altered the management of anemia in end-stage renal disease by decreasing the need for RBC transfusion with associated risks. ** **Erythropoietin in combination with oral iron supplements can raise HCT levels to between 33-38%. This treatment is successful with dialysis patients. ** Hypertension can appear when HCT levels raise too high (most serious side effect). Therefore, HCT should be checked frequently when a patient begins erythropoietin therapy.

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.**

Assessment of Neurological function in Anemia

**Important because the effect of pernicious anemia on the central and peripheral nervous systems. ** **Assessment should include the extent of peripheral numbness, paresteshia, ataxia ( loss of bodily movements), poor coordination, and confusion. ** Finally it is important to monitor relevant laboratory test results and to note any changes overtime.

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)**

Assessment: The tongue may be smooth and red in what type of anemia?

**Iron deficiency anemia**<< IRON IS CONVERTED INT HEMOGLOBIN!!!

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***

Nursing Interventions: Managing Fatigue

**Most frequent symptom and complication* Assisting the patient to prioritize activities and to establish a balance b/w activity and rest that is realistic and feasible from the patient's perspective. Patients with chronic anemia need to maintain some physical activity and exercise to prevent the deconditioning that results from inactivity.

Assessment of GI system in Anemia:

**Nausea and vomiting Find out appearance of emesis[looks like coffee grounds] Melena/dark stools, diarrhea, anorexia, and glossitis (inflammation of the tongue). **Stools should be tested for occult blood.** **Women should be questioned about their menstrual periods (excessive menstrual flow, other vaginal bleeding)** and the use of iron supplements during pregnancy.***

Nursing Interventions: Maintaining Adequate Perfusion

**Patients with acute blood loss or severe hemolysis may have decreased tissue perfusion from decreased blood volume or reduced circulating RBC's (decreased hematocrit) ** **Lost volume is replaced with transfusions or IV fluids based on the symptoms of lab findings. Supplemental Oxygen may be necessary, but it is rarely needed on a long-term basis.** Nurse monitors vital signs closely, other medications such as antihypertensive agents may need to be adjusted or withheld.

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.**

Nursing Process: Sickle Cell Crisis: Planning and Goals

**Relief of pain** decreased incidence of crisis enhanced self-esteem and power absence of complications

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

A conclusion as to whether the anemia is caused by destruction or by inadequate production of RBC's usually can be reached on the basis of what factors?

**The marrow's ability to respond to decreased RBC's (as evidenced by an increased reticulocyte count in the circulating blood)** **The degree to which young RBC's proliferate in the bone marrow and the manner in which they mature (as observed on the bone marrow biopsy)** **The presence or absence of end products of RBC destruction within the circulation (ex: increased bilirubin level, decreased haptoglobin level)**

Anemias In Renal Disease:

**Usually anemic when serum creatinine levels exceed 3mg/100mL** HCT: falls between 20-30% RBC's appear normal on peripheral smear. **This anemia is caused by mild shortening of RBC life span and a defeiciency of erythropoieitin [ necessary for erythropoiesis] As renal function decreases, erythropoietin which is produced by the kidneys also decreases. ** **Patients undergoing long-term hemodialysis lose blood into the dialyzer and therefore may become iron deficient. ** **Folic acid deficiency develops because this vitamin passes into the dialysate. Therefore, patients who are receiving hemodialysis and who are anemic should be evaluated for iron and folate deficiency and treated appropriately.**

How else can you lose blood besides hemolytic and hypoproliferative classifications?

**You can lose blood by the loss of RBC's that occurs with bleeding, potentially from any major source such as the GI tract, the uterus, the nose, or a wound.** VERY IMPORTANT!!!!

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.**

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.**

Monitoring and Managing Potential Complications:

A **significant complication** of anemia is heart failure from chronic *diminished blood volume* and the heart's compensatory effort to increased cardiac output. **Patients with anemia should be assessed for signs and symptoms of heart failure. ** A serial record of body weights can be more useful then I&O. Diuretics may be required in case of fluid retention resulting from congestive heart failure.

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***

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

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]

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

Objective 1: Describe process of Hematopiesis:

Cellular components of blood consists of 3 primary cell types: RBC's WBC's and Platelets; look at chart you printed out in notes. **Because most blood cells have a short life span, the need for the body to replenish its supply of cells is continuous; this process is termed hematopiesis. ** Blood carries oxygen absorbed from the lungs and nutrients absorbed in the GI tract to the body cells for metabolism. Blood also carries waste products produced by cellular metabolism to the lungs, skin, liver, and kidneys, where they are transformed and eliminated from the body. Blood also carries hormones, antibodies, and other substances to their sites of action or use.

Nursing Management Sickle Cell:

Chronic Pain and Substance Abuse: Best managed by prevention Receiving care by a single provider is beneficial than rotating physicians

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

Anemia of Chronic Disease

Disorders include rheumatoid arthritis; severe/chronic infections, and many cancers. Usually mild to moderate and nonprogressive. Develops gradually over 6-8 weeks and then stabilizes at a hematocrit seldom less than 25%. Hemoglobin rarely falls below 9 g/dL Bone marrow has normal cellularity with increased stores of iron as the iron is diverted from the serum. EPO levels are low, perhaps because of decreased production and iron use is blocked. Most of these patients have few symptoms and do not require treatment for anemia. With successful treatment of the underlying disorder, the bone marrow iron is used to make RBC's and the hemoglobin level rises.

***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*********

MDS: Nursing Management

Extensive instruction of infection risk Avoid infection S/s of developing infection Instruction for risk of bleeding **Neutropenic precautions** Monitor lab values **Chelation [ used to remove excess iron acquired from chronic transfusions; most effective in SUB-Q infusion over 8-12 hrs]** ***Local erythema requires no intervention it is a common reaction***<< in regards to chelation

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.

Folic acid requirements with hemolytic anemias & pregnancy

Folic acid requirements are usually increased in patients with chronic hemolytic anemias and in woman who are pregnant because the need for RBC production is increased in these conditions. Some patients with malabsorptive diseases of the small bowel, such as sprue, may not absorb folic acid normally.

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

Hypoproliferative Anemia: What does it result from?

Hypoproliferative anemia results from *DEFECTIVE RBC PRODUCTION***

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

In order to function, how must blood remain ?

In order to function blood must remain in its normally fluid state. Because blood is a fluid, the danger always exists that trauma can lead to blood loss from the vascular system. To prevent this, an intricate clotting mechanism is activated when necessary to seal any leak in the blood vessels. Excessive clotting is equally dangerous because it can obstruct blood flow to VITAL TISSUES. To prevent this, the body has a fibrinolytic mechanism that eventually dissolves clots (thrombi) formed within the blood vessels. The balance between these two systems, clot (thrombus) formation and clot (thrombus) dissolution or fibrinolysis is called HEMOSTASIS!!

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.**

Nursing Interventions: Maintaining Adequate Nutrition

Inadequate intake of essential nutrients, such as iron, vitamin B12, folic acid, and protein can cause some anemias. Healthy diet should be encouraged Alcohol should be avoided or limited Dietary teaching sessions should be individualized, including cultural aspects related to food prefences and or preparation **Many forms of anemia are not the result of nutritional deficiency!!!!! So patients must avoid taking excess iron supplements**

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

Why is it more reliable to use ferritin and hemoglobin rather than iron and hemoglobin to check for Iron deficiency anemia?

It is more reliable to use ferritin rather than iron because when a person has an infection, and inflammatory conditions they can cause low serum iron level and TIBC with an elevated ferritin level.

LOOK AT TABLE 33-4 ON PAGE 887

LOOK AT TABLE 33-4 ON PAGE 887 DONT FORGET!!!

Nursing Management: Sickle Cell

Leg Ulcers: Require careful management and protection from trauma and contamination Referral to a wound care specialist If leg ulcers fail to heal, grafting may be neccessary Aseptic technique is warranted to prevent nosocmial infections

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

MDS: Clinical Manifestations

Many patients asymptomatic Fatigue often present Neutrophil dysfunction= ^ risk for infection Platelet function altered= risk for bleeding

Nursing Management: Sickle Cell

Minimizing Deficient Knowledge: Keeping warm and maintaining adequate hydration Avoid stressful situations= more challening Group education may be effective

Hemolytic Anemia: What can patients develop from repeated blood transfusions: Medical MGMT

Multiple autoantibodies making cross-matching difficult. **Classic distinguishing factor with a hemolytic transfusion the patient becomes more anemic after being transfused. ** Patient supported with corticosteroids (prednisone) & IV immunoglobulin

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

Megaloblastic Anemia: Promoting Home and Community- Based Care:

Nurse needs to pay particular attention to ambulation and should assess the patient's gait, and stability as well as the need for assistive devices (canes, walkers) and for assistance in managing daily activities. If sensation is altered, patients needs to be instructed to avoid excessive heat and cold **Because mouth and tongue soreness may restrict nutritional intake, the nurse can advise patients and families to prepare bland, soft foods, and eat small amounts frequently. Nurse also can explain that alcohol induced anemia can induce neurological problems** Patients also must be taught the chronicity of their disorder and the necessity for monthly vitamin B12 injection even in the absence of symptoms. Many patients can be instructed to self-administer their injections. **Gastric atrophy associated with pernicious anemia increases the risk for gastric carcinoma.**

Assessment of Anemia: Nutritional assessment

Nutritional assessment is important, because it may indicate deficiencies in essential nutrients such as iron, vitamin B12, and folic acid. **Strict vegetarians are also at risk for megaloblastic types of anemia if they do not supplement their diet with vitamin B12.** <<Vitamin B12 are in MEATS!!

Nursing Interventions: Promoting Compliance to Prescribed Therapy:

Patient's with anemia, medications or nutritional supplements are often prescribed to alleviate or correct the condition. To enhance compliance, the nurse can assist patients in developing ways to incorporate the therapeutic plan into their lives, rather than merely giving the patient a list of instructions.

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 Management: Sickle Cell

Priapism leading to impotence: Male patients develop sudden painful episodes of priapism (persistent penile erection) Patient is taught to empty his bladder @ onset of the attack, exercise, and take a warm bath If episode persists > 3 hours, medical attention is recommended.

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

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

Megaloblastic: Assessment and Diagnostic Findings of Vitamin B12 deficiency

Schilling Test: P/t receives small oral dose of radioactive Vitamin B12, followed in a few hours by a large nonradioactive parenteral dose of vitamin B12 (aids in renal excretion of radioactive dose). If oral vitamin is absorbed, more than 8% will be excreted in the urine within 24 hours; therefore if no radioactivity is present in the urine (radioactive B12 stays in GI tract), the cause is GI malabsorption of Vitamin B12. If urine is radioactive, the cause of deficiency is not ileal disease or pernicious anemia. Later, same procedure is repeated, but this time intrinsic factor is added to oral radioactive vitamin B12. If radioactivity is now detected in the urine (B12 absorbed from GI tract in presence of intrinsic factor), diagnosis of pernicious anemia can be made. Schilling test only useful if urine collections are complete. Intrinsic factor antibody test: (+) test indicates the presence of antibodies that bind with vitamin B12- intrinsic factor complex and prevent it from binding to receptors in the ileum, thus preventing absorption. Not specific for pernicious anemia alone but it can aid in the diagnosis.

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**

Assessment of Cardiac Status:

Should be carefully assessed. When hemoglobin level is low, the heart attempts to compensate by pumping faster and harder in an effort to deliver more blood to hypoxic tissues. **Increased cardiac workload can result in symptoms such as tachycardia, palpitations, dyspnea, dizziness, orthopnea, and exertional dyspnea. ** **Heart failure may eventually develop as evidenced by an enlarged heart (cardiomegaly) and liver (hepatomegaly) and by peripheral edema.**!!!!

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.**

Assessment: What should the health history include?

The health history should include a medication history, because some medications can depress bone marrow activity or interfere with folate metabolism. An accurate history of alcohol intake, including the amount and duration should be obtained. Family history is important, because certain anemias are inherited. Athletic endeavors should be assessed, because extreme exercise can decrease erythropoiesis and RBC survival in some athletes.

Planning and Goals of Anemia:

The major goals for the patient may include increased tolerance of normal activity attainment or maintenance of adequate nutrition maintenance of adequate tissue perfusion Compliance with prescribed therapy Absence of complications

What factors influence the development of anemia-associated symptoms?

The speed in which the anemia has developed The duration of the anemia (chronicity) The metabolic requirements of the individual Other concurrent disorders or disabilities (cardio-pulmonary disease) Special complications or concomitant features of the condition that produced the anemia

A person who has been anemic for a very long time with hemoglobin levels between 9-11 g/dL usually has??

few or no symptoms other than slight tachycardia on exertion and fatigue.

Myelodysplastic Syndrome (MDS) [bone marrow failure]

group of disorders of the myeloid stem cell that causes dysplasia (abnormal development) in one ore more types of cell lines. **MOST COMMON FEATURE**>> dysplasia of the RBC's --- is manifested as a macrocytic anemia; however WBC's (myeloid cells, paricularly neutrophils) and platelets can also be affected. **[PANCYTOPENIA]** Although bone marrow is actually hypercellular, many of the cells die within it before being released into circulation. Therefore, the number of affected cells in circulation is typically lower than normal. Neutrophils have diminished ability to destroy bacteria by phagocytosis Platelets are less able to aggregate and are less adhesive than usual **RESULT***>> increased risk for infection and bleeding, even when the actual # of circulating cells may not be excessively low. **Significant portion of MDS cases evolve into ACUTE MYELOID LEUKEMIA (AML); THIS TYPE OF THERAPY TENDS TO BE NONRESPONSIVE TO STANDARD THERAPY!!!**

Assessment: What type of anemia is jaundice present in?

jaundice is present in hemolytic anemia/megaloblastic anemia

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)