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Chapters 41 & 42

Hematologic Problems

Anemia
Reduction in either the number of red blood cells, the amount of hemoglobin, or the hematocrit Clinical sign (not a specific disease); a manifestation of several abnormal conditions Average number of RBCs per cubic millimeter is 4,400,000 to 5,200,000 Each RBC contains nearly 300 million hemoglobin molecules. Each can carry 4 O2 molecules When, for any reason, the hemoglobin content in the bloodstream dips below the minimum for body needs, the result is anemia, meaning (although not literally) "no blood." Anemia is a reduction in the number or volume of RBCs. This reduction in RBCs results, obviously, in a reduction in the amount of hemoglobin and, therefore, in a reduction in the body's oxygen-carrying capacity. Another cause of anemia could be a diet deficient in iron-rich foods. Explain Hematocrit method to include Buffy Coat Layer. Spin 3000rpm, 3 min (Hemoglobin is generally the Hematocrit divided by 3) Hct (%)= (0.0485 x ctHb (mmol/L) + 0.0083 x 100

Cardiovascular Effects of Anemia


Desaturation, Dyspnea, Fatigue, Hypotension, Headache, Pallor & Tachycardia. (Dizzy, Devious Fat Harold Had Peaches & Tomatoes.)

Anatomy and Physiology Review

Bone marrow Red blood cells (erythrocytes) White blood cells Platelets

Accessory Organs of Blood Formation


RBCs are terminally differentiated; that is, they can never divide. RBCs live about 120 days and then are ingested by phagocytic cells in the liver and spleen. Most of the iron in their hemoglobin is reclaimed for reuse. The remainder of the heme portion of the molecule is degraded into bile pigments and excreted by the liver. Some 3 million RBCs die and are scavenged by the liver each second. Erythropoietin (EPO), produced by the kidneys, enhances the production of red blood cells (RBCs).

Blood Cell Differentiation

Review of RBC Growth Cycle

Hemostasis/Blood Clotting
o o o o o Platelet aggregation Blood clotting cascade Intrinsic factors Extrinsic factors Fibrin clot formation

Review of Platlet Growth Cycle

Platelets and Blood Clotting

Hageman (Intrinsic Pathway)(12)-- D-Dimer (Extrinsic Pathway) VonWillebrand (Platelet Adherence to wall) (8) Heterodimer (Tf)

Alterations to Clotting Cascade

Intrinsic Factors = Within the blood Extrinsic Factors = Outside the blood (trauma, shape & smoothness of capillary Hemophilia Factor IX Coumadin works on Factor X HeparinFactor Xa---> Factor V

Anticlotting Forces

Assessment Critical Foci


Patient history Functional Patterns Activity Intolerance Nutrition-Metabolic Pattern Types of food Medications (see Table 41-3) Respiratory & Cardiac Assessment

Drugs
Anticoagulantsinterfere with steps in blood clotting; limit or prevent extension of clots and prevent new clots Fibrinolyticsselectively degrade fibrin threads in the formed blood clot Platelet Inhibitorsprevent platelets from becoming active or activated platelets from clumping together Aspirin & Plavix

Physical Assessment
Skin Head and neck Respiratory Cardiovascular Renal and urinary bleeding, ESRD & EPO Musculoskeletal Abdominal Spleen size, GI Bleed Central nervous system Restlessness, Headache Psychosocial Coping, Support Nutritional status Family history and genetic risk Current health problems Co-Morbidities Anemia as a Sign & Symptom of a Disease

Diagnostic Assessment
Tests of cell number and function: Complete blood count Reticulocyte count Nucleated RBC Hemoglobin electrophoresis Leukocyte alkaline phosphatase Coombs test Serum ferritin, transferrin, and total iron-binding capacity

Tests Measuring Bleeding and Coagulation


Capillary fragility test Bleeding time test Prothrombin time International normalized ratio (INR) Partial thromboplastin time Platelet agglutination/aggregation

Other Tests
Radiographic examinations Radioisotope Scans of Bone Marrow Bone marrow aspiration and biopsy Direct examination of stem cells

Sickle Cell Disease


Genetic disorder resulting in chronic anemia, pain, disability, organ damage, increased risk for infection, and early death Sickle cell disease state and sickle cell trait Variation in severity & complications

Sickle Cell Anemia

Story of Sickle Cell Crisis at High Altitude Bolivia or anesthesia

Sickle Cell Disease: Pathophysiology

Normally, Sickled cells return to normal shape when low O2 condition is reversed
Main problem of the disorder is formation of abnormal hemoglobin chains Response to local or systemic hypoxia RBCs sickle, decrease o2 carrying capacity & clog capillaries Sickle cell crisis Symptomatic after enough capillaries shut down Incidence/prevalence In the Unites States, around 72,000 people are symptomatic with the disease The disease occurs in about 1 in every 500 African-American births and 1 in every 1000 to 1400 Hispanic-American births 2 million Americans, or 1 in 12 African Americans, carry the sickle cell trait.

Sickle Cell Disease: Clinical Manifestations


Cardiovascular changes Heart Failure SOB & Fatigue

Skin changes Cool, Pallor or Cyanosis, Clubbing Abdominal changes Pain, firm large lumpy liver & spleen Renal and urinary changes Priapism Chronic Kidney Disease Musculoskeletal changes Pain & Ulcers CNS changes Fever, Infarcts, Seizures & Gait Disturbances

Sickle Cell Disease: Assessment


Besides assessing for the system changes common to SCD Assess Level, Location, Character and Duration of Pain Assess mental status changes O2 Saturation Levels Family and Patient Coping Mechanism and Support Systems

SCD: Laboratory Assessment


Hemoglobin S (HbS) < 1% for those without SCD 5% - 50% for those with SCD Trait May exceed 90% for those with SCD Number of RBCs with permanent sickling Hematocrit Low Reticulocyte count Elevated anemia of long duration Total bilirubin Elevated Release from damaged RBCs Total white blood cells Elevated Imaging assessment Crew-cut skull

Sickle Cell Disease: Interventions


Pain is the most common problem: Drug therapy48 hours of IV analgesics

O2 Therapy Hypotonic IV & Oral hydration Complementary and alternative therapies (Warm & Calm) Transfusions Avoided Fe Overload

Goals of Treatment--- Chart 42-3 Pg. 898


Management of vaso-occlusive crisis Management of chronic pain syndromes Management of chronic hemolytic anemia Prevention and treatment of infections Management of the complications and the various organ damage syndromes associated with the disease Prevention of stroke Detection and treatment of pulmonary hypertension

Sickle Cell Interventions: Potential for Sepsis


Interventions include: Prevention and early detection strategies spleen often non-functional Excellent Infection control practices (Handwashing) for all family & care-givers Drug therapy Prophylactic BID PCN Acute chest syndrome. Similar to pneumonia, this life-threatening complication is caused by infection or trapped sickled cells in the lung. It is characterized by chest pain, fever, and an abnormal chest X ray.

Sickle Cell Disease Interventions: Potential for Multiple Organ Dysfunction


Interventions include: Hydration (200ml/hr) Oxygen therapy (Monitor ABGs) Transfusion therapy (Cautiously) Goal to dilute the HbS levels Monitor patients FE levels closely Desfereral drug of choice

Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency Anemia

Most common type of inherited hemolytic anemia G6PD necessary for the RBC to produce energy to survive normal life expectancy Effect of exposure to some drugs, benzene and other toxins Screening for this deficiency necessary before donating blood, because cells deficient in G6PD can be hazardous Hydration Osmotic diuretics Transfusions ------RBCs break

RBC Indices
MCV Mean Corpuscular Volume MCH Mean Corpuscular Hemoglobin MCHC Mean Corpuscular Hemoglobin Concentration RDW Relative Distribution Width

Iron Deficiency Anemia (Microcytic)


This common type of anemia can result from blood loss, poor intestinal absorption, or inadequate diet. If mildsymptoms of weakness and pallor. Evaluate adult patients for abnormal bleeding, especially from the GI tract. Treatmentincreasing oral intake of iron from food sources, oral iron supplements, or IM iron solutions.--- Z-track

Factors Influencing Fe Absorption


Calcium competes with iron for absorption Substances that diminish the absorption of ferrous and ferric iron include phytates, oxalates, phosphates, carbonates, and tannates Starch and clay eating produce malabsorption of iron and iron deficiency anemia (Pica) Low gastric acid conditions whether naturally occurring (Celiac Disease), surgically or medication induced. Extensive surgical removal of the proximal small bowel or chronic diseases (eg, untreated sprue or celiac syndrome) can diminish iron absorption

Vitamin B12 Deficiency Anemia (Macrocytic)


Lack of vitamin B12 causes improper DNA synthesis of RBCs. Poor intake of foods containing vitamin B12, small bowel resection, tapeworm, overgrowth of intestinal bacteria. Glossitis smooth beefy tongue

Fatigue & wt loss Paresthesias hands & feet, poor balance Weight loss surgery

Pernicious Anemia
Same as pervious slide except due to specific cause Anemia resulting from failure to absorb vitamin B12 Caused by a deficiency of intrinsic factor

Folic Acid Deficiency Anemia


Manifestations similar to those of vitamin B12 deficiency, but nervous system functions remain normal Common causespoor nutrition, malabsorption (Crohns), alcoholism and drugs Green Leafy, Liver, Beans & Nuts

Aplastic Anemia
Deficiency of circulating RBCs because of failure of the bone marrow to produce these cells Injury to the pluripotent stem cell Pancytopenia common Treatment: Blood transfusions Immunosuppressive therapy Splenectomy

Polycythemia VeraCancer of the RBCs


Disease with a sustained increase in blood hemoglobin or hematocrit Massive production of red blood cells Excessive leukocyte production Excessive production of platelets Treatment: Phlebotomy Hydration Anticoagulants

Myelodysplastic Syndromes

Group of disorders caused by the formation of abnormal cells in the bone marrow Blood and platelet transfusions Erythropoietin Chelation therapy Drugs for iron overloaddeferasirox and deferoxamine mesylate

Transfusion Therapy
Pretransfusion responsibilities to prevent adverse transfusion reactions: Verify prescription. Test donors and recipients blood for compatibility. Examine blood bag for identification. Check expiration date. Inspect blood for discoloration, gas bubbles, or cloudiness.

Transfusion Responsibilities
Provide patient education. Assess vital signs. Begin transfusion slowly, and stay with patient first 15 to 30 minutes. Ask patient to report unusual sensations such as chills, shortness of breath, hives, or itching. Administer blood product per protocol. Assess for hyperkalemia.

Blood Typing
Allele from Allele from Parent 1 Parent 2 Genotype of offspring Blood types of offspring

A A A B B B

A B O A B O

AA AB* AO AB* BB BO

A AB A AB B B

OO

Human blood type is determined by co-dominant alleles This makes six different genotypes. Rh Pos or Neg makes 12 different blood types

Types of Transfusions
Red blood cell Platelet Plasmafresh frozen plasma Cryoprecipitate Granulocyte (white cell)

Transfusion Reactions
Febrile transfusion reactions Hemolytic transfusion reactions Allergic transfusion reactions Bacterial transfusion reactions Circulatory overload Transfusion-associated graft-versus-host disease

Autologous Blood Transfusion


Collection and infusion of patients own blood Eliminates compatibility problems; reduces risk for transmission of bloodborne disease Types: Preoperative Acute normovolemic hemodilution Intraoperative autologous transfusion Postoperative blood salvage