Hematology Jan Bazner-Chandler CPNP, CNS, MSN, RN

Blood Blood is the fluid of life Blood is composed of:  Plasma  RBC  WBC  Platelets

Plasma Plasma consists of:  90% water.  10 % solutes: albumin, electrolytes and proteins.  Proteins consist of clotting factors, globulins, circulating antibodies and fibrinogen.

Red Blood Cells RBC’s travel through the body delivering oxygen and removing waste. RBC’s are red because they contain a protein chemical called hemoglobin which is bright red in color. Hemoglobin contains iron, making it an excellent vehicle for transporting oxygen and carbon dioxide.

RBC’s Average life cycle is 120 days. The bones are continually producing new cells.

White Blood Cells The battling blood cells. The white blood cells are continually on the look out for signs of disease. When a germ appears the WBC will:  Produce protective antibodies.  Surround it and devour the bacteria.

WBC’s WBC life span is from a few days to a few weeks. WBC’s will increase when fighting infection.

Platelets Platelets are irregularly- shaped, colorless bodies that are present in blood. Their sticky surface lets them form clots to stop bleeding.

Blood Values CBC with differential and platelet count.  Hgb: Normal levels are 11 to 16 g / dl Panic levels are:  Less than 5 g / dl  More than 20 g / dl

Hematocrit Normal hematocrit levels are 35 to 44%.  Panic levels: Hmct less than 15 % Hmct greater than 60%

Hemoglobin and Hematocrit Can be used as a simple blood test to screen for anemia. The CBC with differential would be used to help diagnose a specific disorder. A bone marrow aspiration would be the most conclusive in determining cause of anemia – aplastic / leukemia.

Bone Marrow Bone marrow is the spongy substance found in the center of the bones. It manufactures bone marrow stem cells, which in turn produce blood cells. Red blood cells – carry oxygen to tissue Platelets – help blood to clot White blood cells – fight infection

Bone Marrow Transplant Donor is placed under anesthesia. Marrow is aspirated out of the iliac crest. Marrow is filtered and treated to remove bits of bone and other unwanted cells and debris, transferred to a blood bag, and is infused into the patient’s blood just like at transfusion.

Bone Marrow Aspiration

Treatment Modalities Transfusion:  Packed red blood cells – anemia  Platelets – platelet dysfunction  Fresh frozen plasma – coagulation factors

Blood Transfusions 3 types of transfusion reactions  Hemolytic  Allergic  Febrile

Hemolytic Reaction Refers to an immune response against transfused blood cells. Antigens, on the surface of red blood cells, are recognized as “foreign proteins” and can stimulate B lymphocytes to produce antibodies to the red blood cell antigens.

Hemolytic reaction Flank pain Fever Chills Bloody urine Rash Low blood pressure Dizziness / fainting

Nursing Management Stop the blood transfusion. Start normal saline infusion. Take vital signs with blood pressure Call the MD Obtain blood sample and urine specimen. Return blood to blood bank. Document

Febrile Reaction Often occurs after multiple blood transfusions. Symptoms:fever, chills, and diaphoresis. Interventions:  Slow transfusion and administer antipyretic.  Administer antipyretic prior to administration.

Allergic Reaction Symptoms: rash, urticaria, respiratory distress, or anaphylaxis. Interventions:  administer antihistamine before transfusion  Physician may order washed rbc’s

Hematologic Conditions

Alteration in Hematologic Status Disorders of hemostasis or clotting factors Structural or quantitative abnormalities in the hemoglobin. Anemias Aplastic Anemia

Genetic Implications The following have a genetic link: implications for genetic screening and fetal diagnosis  Sickle cell anemia  Thalassemia  Hemophilia

Bleeding Disorders Three types Hemophilia: males only  Type A most common – factor VIII deficiency  Type B - lack of factor IX (Christmas Disease)  Type C – lack of factor XI Von Willebrand Disease – 1% of population – men or women – prolonged bleeding time

Hemophilia Type A Hemophilia type A is the deficiency of clotting factor VIII.  A serious blood disorder  Affects 1 in 10,000 males in the US  Autoimmune disorder with lowered level of clotting factor  All races and socio economic groups affected equally

Hemophilia Hemophilia is a sex-linked hereditary bleeding disorder Transmitted on the X chromosome Female is the carrier Women do not suffer from the disease itself

Historical Perspective First recorded case in Talmud Jewish text by an Arab physician – documentation of two brothers with bleeding after circumcision. Queen Victoria is carrier and spread the disease through the male English royalty.

Goals of Care Goals of care:  Provide factor VIII (IX) to aid blood in clotting.  To decrease transmission of infectious agents in blood products; hepatitis & AIDS.  Future: gene therapy to increase production of clotting factor.

Symptoms Circumcision may produce prolonged bleeding. As child matures and becomes more active the incidence of bleeding due to trauma increases

Symptoms May be mild, moderate or severe Bleeding into joint spaces, hemarthrosis Most dangerous bleed would be intracranial.

Diagnosis Presenting symptoms Prolonged activated aPTT and decreased levels of factor VIII or IX. Genetic testing to identify carriers

Treatment Products used to treat hemophilia are:  Fresh frozen plasma and cryoprecipitate which are from single blood donors and require special freezing.  Second generation of factor VIII are made with animal or human proteins.

Nursing Diagnoses Risk for injury Pain with bleed especially into a joint Impaired physical mobility Knowledge deficit regarding disease and management of disease

Nursing interventions No rectal temps. Replace the factor as ordered by physician. Manage pain utilizing analgesics as ordered. Maintaining joint integrity during acute phase: immobilization, elevation, ice. Physical therapy to prevent flexion contraction and to strengthen muscles and joints. Provide opportunities for normal growth and development.

Teaching Avoid aspirin which prolongs bleeding time in people with normal levels of factor VIII. A fresh bleeding episode can start if the clot becomes dislodged. Natural reactions in the body cause the clot that is no longer needed to “break down. This process occurs 5 days after the initial clot is formed.

Family Education Medic-Alert bracelet Injury prevention appropriate for age Signs and symptoms of internal bleeding or hemarthrosis Dental checkups Medication administration

Long Term Complications 20% develop neutralizing antibodies that make replacement products less effective. Gene therapy providing continuous production of the deficient clotting factor could be the next major advance in hemophilia treatment.

Disseminated Intravascular Coagulation or DIC DIC is an acquired coagulopathy that is characterized by both thrombosis and hemorrhage. DIC is not a primary disorder but occurs as a result of a variety of alterations in health.

Assessment The most obvious clinical feature of DIC is bleeding. Renal involvement = hematuria, oliguria, and anuria. Pulmonary involvement = hemoptysis, tachypnea, dyspnea and chest pain. Cutaneous involvement = petechiae, ecchymosis, jaundice, acrocyanosis and gangrene.

Management of DIC Treatment of the precipitating disorder. Supportive care with administration of platelet concentration and fresh frozen plasma and coagulation factors. Administration of heparin (controversial in children). Heparin potentates anti-thrombin III which inhibits thrombin and further development of thrombosis.

Nursing Diagnoses Altered tissue perfusion Risk for injury Anxiety

Nursing Interventions Rigorous ongoing assessment of all body systems Monitor bleeding No rectal temps Avoid trauma to delicate tissue areas All injections sites and IV sites need to be treated like an arterial stick.

Prognosis Depends on the underlying disorder and the severity of the DIC.

ITP Idiopathic thrombocytopenic purpura  Idiopathic = cause is unknown  Thrombocytopenic = blood does not have enough platelets  Purpura = excessive bleeding / bruising

Immune Thrombocytopenic Purpura Antibodies destroy platelets Antibodies see platelets as bacteria and work to eliminate them ITP is preceded by a viral illness  URI  Varicella / measles vaccine  Mononucleosis  Flu

Symptoms Random purpura Epistaxis, hematuria, hematemesis, and menorrhagia Petechiae and hemorrhagic bullae in mouth

Diagnostic Tests Low platelet count Peripheral blood smear Antiplatelet antibodies Normal platelet count: 150,000 to 400,000

Management IV gamma globulin to block antibody production, reduce autoimmune problem Corticosteroids to reduce inflammatory process IV anti-D to stimulate platelet production

Sickle Cell Anemia Autosomal recessive disorder Defect in hemoglobin molecule Cells become sickle shaped and rigid Lose ability to adapt shape to surroundings. Sickling may be triggered by fever and emotional or physical stress

Pathophysiology When exposed to diminished levels of oxygen, the hemoglobin in the RBC develops a sickle or crescent shape; the cells are rigid and obstruct capillary blood flow, leading to congestion and tissue hypoxia; clinically, this hypoxia causes additional sickling and extensive infarctions.

Whaley & Wong Text

Crescent Shaped Cells

Body Systems Affected by SS Brain: CVA – paralysis - death Eyes: retinopathy – blindness Lungs: pneumonia Abdomen: pain, hepatomegaly, splenomegaly (medical emergency due to possible rupture Skeletal: joint pain, bone pain – osteomyelitis Skin: chronic ulcers – poor wound healing

Vaso-occlusive Crisis Stasis of blood with clumping of cell in the microcirculation, ischemia, and infarction Most common type of crisis; painful Signs include fever, pain, tissue engorgement

Splenic Sequestration Life-threatening / death within hours Pooling of blood in the spleen Signs include profound anemia, hypovolemia, and shock Abdominal distention, pallor, dyspnea, tachycardia, and hypotension

Aplastic Crisis Diminished production and increased destruction of red blood cells Triggered by viral infection or depletion of folic acid Signs include profound anemia, pallor

Nursing Diagnoses Altered tissue perfusion Pain Risk for infection Knowledge deficit regarding disease process

Nursing Management - Hospital Increase tissue perfusion  Oxygen  Blood transfusion if ordered  Bed rest Pain management Hydration  IV fluids as ordered  Oral intake of fluids

Nursing Management Adequate nutrition Emotional Support Discharge instructions Information about disease management Daily folic acid Control of triggers Prophylactic antibiotics Immunizations / Pneumococcal

Patient Education Necessity of following plan of care Signs and symptoms of impending crisis. Signs and symptoms of infection Preventing hypoxia from physical and emotional stress Proving adequate rest

Beta-Thalassemia Hereditary / autosomal defect Genetic defect on chromosome 11 Mediterranean descent Defect in the beta globin gene Beta globin chains are required for synthesis of hemoglobin A

RBC Characteristics  Microcytosis = small in size  Hypochromia = decrease hemoglobin  Poikilocytosis = abnormal shape

Treatment / Prognosis Supportive Blood transfusions as needed Bone marrow transplant Poor prognosis / death within 1 st year due to septicemia or heart failure.

Iron Deficiency Anemia Most common nutritional deficiency Depletion of iron stores

Abnormal Laboratory Values Hemoglobin levels less than 8 g/dL Decreased levels of Serum Iron or Total Iron Binding or Serum Ferritin Microcytic and hypochromic red blood cells

IDA Occurs in children experiencing:  Rapid physical growth  Low iron intake  Inadequate iron absorption  Loss of blood

Symptoms Associated with low oxygenation of tissue:  Pallor  Fatigue  Shortness or breath  Irritability  Intolerance of physical work / exercise

Management Iron supplementation  Given in a.m. on an empty stomach  To avoid staining of teeth, give using a syringe, dropper or straw  Instruct caretaker that child may have dark- colored stools

Management Nutritional counseling Infants younger than 12 months should be on formula until around 12 months of age Infants 12 months or older  Decrease intake of milk  Introduce solid foods Children: iron fortified cereals, foods, meat, green leafy vegetables Teenagers: reduce junk food

Aplastic Anemia Acquired or inherited Normal production of blood cells in the bone marrow is absent or decreased. A marked decrease in RBC’s, WBC’s and platelets.

Causes Exposure to drugs Exposure to chemicals Exposure to toxins Infection Idiopathic in nature

Blood Characteristics Neutophil less than 500 Platelet less than 20,000 Hemoglobin less than 7 Reticulocytes 1% Nursing Diagnosis? Bone marrow reveals hypo-cellular and fatty marrow.

Management Immunosuppressive therapy  Antithymocyte globulin Administered IV over 4 days Response seen within 3 months Bone Marrow Transplant

Hyper-bilirubinemia

Hyperbilirubinemia Many babies have some jaundice. When they are a few days old, their skin slowly begins to turn yellow. The yellow color comes from the color of bilirubin. When red blood cells die, they break down and bilirubin is left. The red blood cells break down and make bilirubin. In newborns, the liver may not be developed enough to get rid of so much bilirubin at once. So, if too many red blood cells die at the same time, the baby can become very yellow or may even look orange. The yellow color does not hurt the baby's skin, but the bilirubin goes to the brain as well as to the skin. That can lead to brain damage.

Signs and symptoms Very yellow or orange skin tones (beginning at the head and spreading to the toes) Increased sleepiness, so much that it is hard to wake the baby High-pitched cry Poor sucking or nursing Weakness, limpness, or floppiness

Photo Therapy

Fiberoptic Blanket

Nursing Interventions Monitor bilirubin levels Assess activity level – muscle tone – infant reflexes Encourage po intake: May need to supplement with formula if inadequate breastfeeding Weight daily to assess hydration status Monitor stools – amount and number Cover eyes while under bili-lights Facilitate parent - infant bonding Loss of moro or startle reflex can indicate possible brain damage due to Kernicterus