Zoya Minasyan, RN-MSN- Edu

 A deficiency in the  Number of erythrocytes (red blood cells [RBCs])  Quantity of hemoglobin  Volume of packed RBCs (hematocrit)  Diverse causes such as  Blood loss  Impaired production of erythrocytes  Increased destruction of erythrocytes

 RBC function  Transport oxygen (O 2 ) from lungs to systemic tissues  Carry carbon dioxide from the tissues to the lungs

 Classified as  Morphologic  Cellular characteristics (erythrocyte size and color )  Descriptive, objective laboratory information  Etiologic  Underlying cause (clinical conditions)

 Caused by the body’s response to tissue hypoxia  Hemoglobin (Hb) levels are used to determine the severity of anemia. Mild states of anemia (Hb 10 to 14 g/dL ) may exist without causing symptoms. Symptoms include palpitations, dyspnea, and mild fatigue. In cases of moderate anemia (Hb 6 to 10 g/dL ), cardiopulmonary symptoms are increased and the patient may experience them while resting, as well as with activity. The patient with severe anemia (Hb less than 6 g/dL ) has many clinical manifestations involving multiple body systems.

 Pallor  ↓ Hemoglobin  ↓ Blood flow to the skin  Jaundice  ↑ Concentration of serum bilirubin  Pruritus (Itching)  ↑ Serum and skin bile salt concentrations  Additional attempts by the heart and lungs to provide adequate O 2 to the tissues  Cardiac output maintained by increasing the heart rate and stroke volume 7

 Group of disorders caused by impaired DNA synthesis  Characterized by the presence of large RBCs (megaloblasts)  Easily destroyed because of fragile cell membrane  Majority result from deficiency in  Cobalamin (vitamin B 12 )  Folic acid

 Classification  Cobalamin (vitamin B 12 ) deficiency  Folic acid deficiency  Drug-induced suppression of DNA synthesis  Inborn errors  Erythroleukemia

 Intrinsic factor (IF)  Protein secreted by the parietal cells of the gastric mucosa  IF is required for cobalamin absorption in the small intestine.  Causes  Pernicious anemia  Gastrectomy  Nutritional deficiencies  Chronic alcoholism  Hereditary enzymatic defects

 GI surgery  Chronic diseases of the GI tract  Long-term users of H 2 -histamine receptor blockers  Absence of IF

General symptoms of anemia  Sore tongue  Anorexia  Nausea/Vomiting  Abdominal pain  Neuromuscular manifestations  Weakness  Paresthesias of the feet and hands  ↓ Vibratory and position senses  Ataxia (lack of muscle coordination)  Muscle weakness  Impaired thought processes

 Parenteral or intra-nasal administration of cobalamin  Important to emphasize adequate dietary intake The dosage and frequency of cobalamin administration may vary. A typical treatment schedule consists of 1000 mg of cobalamin IM daily for 2 weeks and then weekly until the hematocrit is normal, then monthly for life. High-dose oral cobalamin and sublingual cobalamin are also available for those in whom GI absorption is intact. As long as supplemental cobalamin is used, the anemia can be reversed. However, if the person has had long- standing neuromuscular complications, they may not be reversible.

Early detection and treatment.  Ensure that injuries are not sustained because of the patient’s diminished sensation to heat and pain.  C ompliance with treatment.  Evaluate patient for gastric carcinoma.

 Also a cause of megaloblastic anemia  Folic acid is required for DNA synthesis.  RBC formation and maturation  Common causes  Dietary deficiency  Malabsorption syndromes  Drugs  Increased requirement  Alcohol abuse and anorexia  Loss during hemodialysis

 Clinical manifestations are similar to those of cobalamine deficiency.  Absence of neurologic problems  Treated by replacement therapy  Encourage patient to eat foods with large amounts of folic acid. GI disturbances include dyspepsia (upset stomach or indigestion) and a smooth, beefy red tongue. During diagnostic studies, the serum folate level is low (normal is 3 to 25 mg/mL) Replacement therapy: The usual dose is 1 mg per day by mouth. In malabsorption states, up to 5 mg per day may be required.

 Underproduction of RBCs  Mild shortening of RBC survival  Causes  End-stage renal disease  Primary factor: ↓ Erythropoietin  Chronic liver disease  Chronic inflammation  Malignant tumors  Chronic endocrine diseases

 Treating underlying cause.  Rarely blood transfusions.  Erythropoietin therapy  Erythropoietin therapy ( Epogen, Darbepoetin) is used for anemia related to renal disease and may be used for anemia related to cancer and its therapies. However, it is used conservatively because the risk of thromboembolism and mortality is increased in some patients.

 Pancytopenia  Decrease in all blood cell types  RBCs  White blood cells (WBCs)  Platelets  Hypocellular bone marrow  Types  Congenital  Chromosomal alterations  Acquired  Results from exposure to ionizing radiation, chemical agents, viral and bacterial infections

 Abrupt or gradual development  Symptoms caused by suppression of any or all bone marrow elements  General manifestations of anemia  Fatigue, dyspnea  Cardiovascular and cerebral responses  Neutropenia-low neutrophil count (WBC, fighting disease)  The patient with neutropenia is susceptible to infection and is at risk for septic shock and death. Even a low-grade temperature (>100.4 o F) should be considered a medical emergency.  Treatment options  Hematopoietic stem cell transplantation  Immunosuppressive therapy

 Result of sudden hemorrhage  Trauma  Complications of surgery  Disrupted vascular integrity  Concerns  Hypovolemic shock  Reduced plasma volume  Diminished O 2 because fewer RBCs are available 21

 Cause  Body’s attempt to maintain adequate blood volume and O 2  Pain  Internal hemorrhage  Tissue distention, organ displacement, nerve compression  Retroperitoneal bleeding  Numbness  Pain in the lower extremities  Shock is the major complication 22

 Replacing blood volume to prevent shock  Identifying the source of the hemorrhage  Stopping blood loss  Correcting RBC loss  Providing supplemental iron 23

 May be impossible to prevent if caused by trauma  Postoperative patients  Monitor blood loss.  No need for long-term treatment 24

 Reduced iron stores  Bleeding ulcer  Hemorrhoids  Menstrual and postmenopausal blood loss  Management  Identify source.  Stop bleeding.  Use supplemental iron if needed. 25

 Destruction or hemolysis of RBCs at a rate that exceeds production  Intrinsic hemolytic anemia  Abnormal hemoglobin  Enzyme deficiencies  RBC membrane abnormalities 26

 Extrinsic hemolytic anemia  More common than intrinsic  Acquired  Sites of hemolysis  Intravascular  Extravascular 27

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 Jaundice  Destroyed RBCs cause increased bilirubin.  Enlarged spleen and liver  Hyperactive with macrophage phagocytosis of defective RBCs  Accumulation of hemoglobin molecules can obstruct renal tubules.  Tubular necrosis 29

 Group of inherited, autosomal recessive disorders  Presence of an abnormal form of hemoglobin in the erythrocyte  Hemoglobin S (HbS), abnormal  HbS causes the RBC to stiffen and elongate.  Sickle shape in response to ↓ O 2 levels  Substitution of valine for glutamic acid on the β - globin chain of hemoglobin  Genetic disorder  Incurable disease, often fatal 30

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 Typical patient is asymptomatic, except during sickling episodes.  Symptoms may include  Pain and swelling  Pallor of mucous membranes  Jaundice 32

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 Gradual involvement of all body systems  Usually fatal by middle age from renal and pulmonary failure  Prone to infection  Pneumonia, most common infection  Acute chest syndrome 34

 Peripheral blood smear  Sickling test  Electrophoresis of hemoglobin(the movement of charged particles when an electric field is applied to them)  Skeletal x-rays  Magnetic resonance imaging (MRI) 35

 Alleviate symptoms of disease complications.  Minimize end target-organ damage.  Avoid high altitudes, maintain fluid intake, treat infections, help with pain control.  O 2 for hypoxia and to control sickling  Pain management  Acute chest syndrome  Antibiotics  O 2 therapy  Fluid therapy  Transfusions, if needed 36

 Blood transfusions in crisis  Hydroxyurea: Antisickling agent  Erythropoietin in patients unresponsive to hydroxyurea  Hematopoietic stem cell transplant  Can cure some patients with SCD 37

Three extrinsic categories 1. Physical factors  Physical destruction of RBCs results from extreme force on the cells. Hemodialysis, prosthetic heart valves 2. Immune reactions  Antigen-antibody reactions destroy RBCs.  Isoimmune reactions  Antibodies develop against antigens; blood transfusions  Autoimmune reactions Develop antibodies against their own RBCs 3. Infectious agents and toxins  Foster hemolysis in four ways  Invading RBCs and destroying contents  Releasing hemolysis substances  Generating an antigen-antibody reaction  Contributing to splenomegaly 38

 Iron overload disorder  Increased intestinal iron absorption and increased tissue iron deposition  Autosomal recessive, C282Y and H63D  Early symptoms-fatigue, abdominal pain, weight loss.  Later symptoms-liver enlargement, cirrhosis, skin pigment change (bronzing), cardio-myopathy, arthritis, testicular atrophy.  Treatment- to remove excess iron from the body(remove 500ml blood each week for 2-3 years until iron stores are normal in body)  Avoid vitamin C and iron supplements, uncooked seafood.

 Involves the vascular endothelium, platelets, and coagulation factors, which helps to stop hemorrhage and repair vascular injury.  Three major disorders  Thrombocytopenia (low platelet count)  Hemophilia (disorder of clotting factors)  Disseminated intravascular coagulation (DIC)

 Reduction of platelets  Prolonged bleeding from minor trauma  Nursing  Monitor for s/s of bleeding  Monitor for PT, PTT, fibrinogen, platelets count  Avoid injections (IV, IM, SubQ) to prevent bleeding  Protect from trauma,  Administer blood products  Stop anticoagulants  Avoid high impact activity (aerobics, contact sports)

 X-linked recessive genetic disorder  Deficient coagulation factor  Hemophilia A caused by Factor VIII deficiency  Hemophilia B factor IX deficiency  Complications related to prolonged bleeding, uncontrolled hemorrhage after dental extractions, GI bleeding from gastritis or ulcers, ecchymosis or hematomas; pain, paralysis from compression of nerves  Replacement of clotting factors

 Decrease in clotting factor and platelets, which may lead to hemorrhage.  Is always caused by and underlining disease or condition. The underlining cause must be treated for the DIC to resolve.  Assess for petechiae, oozing at IV site, internal bleeding, heart rate, increasing in abd girth, change in mental status, pain, decrease in urine output.  Administer blood product.