CARE OF CLIENTS WITH HEMATOLYMPHATIC DISORDERS Charles Dominick C.

Bustamante Objectives Objectives (cont) REVIEW OF BASIC ANATOMY AND PHYSIOLOGY OF THE HEMATOLOGIC SYSTEM The Hematologic System Blood

Blood Cells BLOOD It is a specialized organ that differs from other organs in that it exist in a fluid state. Plasma- is the fluid portion of blood It contain CHONs, such as Albumin, globulin, fibrinogen, electrolytes, waste products and nutrients 55% of the blood volume is plasma BLOOD Cellular component of Blood: 40-45% RBC (Erythrocytes) WBC ( Leukocytes) Platelets (Thrombocytes) Hematopoiesis- the process to replenish its supply of blood Bone marrow- Primary site of HEMATOPOIESIS BLOOD Adult Bone marrow produces: 175 B RBCs 70 B Neutrophils 175 B platelets daily The marrow responds to increase demand and release adequate no. of cells into the circulation The volume of blood in humans is approx. 7-10% of the normal body weight and amounts to 5-6L. BLOOD Functions: Carries O2 absorbed from the lungs Carries nutrients from the GIT to the body cells for cellular metabolism Carries waste products from cellular metabolism to the lungs, skin, liver and kidneys Carries hormones, antibodies HEMOSTASIS- the balance between these two systems, clot (thrombus) formation and clot dissolution or fibrinolysis

BONE MARROW Site of hematopoiesis or blood cell formation Child- all site are involved Adult- in the pelvis, ribs, vertebrae and sternum Marrow- is one of the largest organs in the body, making up 4-5% KBW Red marrow- cellular component Yellow marrow- fats Stem cell RED BLOOD CELLS Is a biconcave disk that resembles a soft ball compressed between two fingers Disk shape provide a large surface area that facilitates the absorption and release of oxygen molecules Mature RBC Hemoglobin- contains Iron and 95% of the mass The transport of O2 between the lungs and tissues RED BLOOD CELLS Immature RBC Reticulocytes Occurs as a normal response to an increased demand for RBC or in some dse. State Iron is present in the heme component of the molecule An important property of HEME is its ability to bind to O2 loosely and reversibly. O2 readily binds with hemoglobin in the lungs and is carried as oxyhemoglobin in arterial blood OXYHEMOGLOBIN- is brighter red than hemoglobin that does not contain O2 (reduced O2) RED BLOOD CELLS Whole blood- normally contains about 15g of hemoglobin per 100 ml of blood ERYTHROPOIESIS- RBC production ERYTHROPOIETIN- a hormone produced primarily by the KIDNEY, If the it detects low O2 5days- the entire process RED BLOOD CELLS Iron stores and metabolism 10-15mg Fe- average daily diet Addition 2mg daily- must be absorbed by women to replace blood lost during menstruation 3g- total iron content in average adult Mostly absorbed in the small intestine as FERRITIN Transported by binding to TRANSFERRIN RED BLOOD CELLS Vit B12 and Folic acid metabolism Are required for the synthesis of DNA

Can only be found in diet Folic acid is absorbed in the proximal small intestine Vit. B12 is absorbed with the help of IF From animal origin Strict vegetarians are deficient of Vit. B12 RED BLOOD CELLS 120 days- normal life RBC SPLEEN- Graveyard of RBC WHITE BLOOD CELLS PLATELETS Also called thrombocytes Are granular fragments of giant cells in the bone marrow called megakaryocytes THROMBOPOIETIN- regulates Platelet production Stimulates the production and differentiation of megakaryocytes from the myeloid stem cell Play an essential role in the control of bleeding They adhere to the site of injury and each other, forming a PLATELET PLUG that temporarily stops bleeding 7-10 days- Normal life span PLASMA and PLASMA CHONs More than 90% of the plasma is water If the plasma is allowed to clot, the remaining fluid is SERUM Globulins and Albumin- example of Plamsa CHONs Globulins for transport Albumin for maintenance of fluid balance in the vascular system RETICULOENDOTHELIAL SYSTEM Is composed of special tissue macrophages, which is derived from the Monocytes. Macrophages: They defend the body against foreign invaders via phagocytosis They removed old or damaged cells from the circulation They stimulate the inflammatory process and present antigen to the immune system RETICULOENDOTHELIAL SYSTEM Histiocytes: Kuppfer cell in the liver Peritoneal macrophages Alveolar macrophages Spleen Site of activity of all macrophages Red pulp- 75% OF THE SPLEEN White pulp- consist B and T Lymphocytes The spleen sequestered newly released reticulocytes from the marrow, removing nuclear fragments and othe materials RETICULOENDOTHELIAL SYSTEM Spleen Major source of hematopoiesis in the fetal life

It forms substances that promotes phagocytosis of neutrophils Forms the antibody IgM after exposure to antigen HEMOSTASIS The process of preventing blood loss from intact vessels and of stopping bleeding from severed vessels. 2 process are involved in arresting bleeding: Primary hemostasis The severed blood vessels constrict Circulating platelets aggregates at the site and adhere to the vessel and to one another Unstable hemostatic plug formed HEMOSTASIS ASSESSMENT OF CLIENTS WITH HEMATOLOGIC PROBLEMS Complete Health (Nursing) History

Complete Blood Count

Blood Coagulation Studies Bone Marrow Aspiration and Biopsy COMMON HEMATOLOGIC PROBLEMS ANEMIA ANEMIA ANEMIA Nursing Process Nursing Diagnosis: Activity intolerance related to weakness, fatigue and general malaise Imbalanced nutrition, less than body requirements, related to inadequate intake of essential nutrients Ineffective tissue perfusion related to inadequate blood volume or hematocrit Noncompliance with prescribed therapy ANEMIA Major Goals: Increase tolerance of normal activity Attainment and maintenance of adequate nutrition Maintenance of adequate tissue perfusion Compliance to prescribed therapy Absence of complications ANEMIA Nursing Interventions: Managing of Fatigue

The most frequent symptom and complication of Anemia Maintaining adequate nutrition Maintaining adequate perfusion Monitoring and managing potential complications HYPOPROLIFERATIVE ANEMIAS IRON DEFICIENCY ANEMIA Results when the intake of dietary iron is inadequate for hemoglobin synthesis The most common type of anemia in all age group The most common anemia in the world BLEEDING- the most common cause of Iron deficiency in men and postmenopausal women The most common cause of iron deficiency in premenopausal womenMENORRHAGIA (excessive menstrual bleeding) and Pregnancy with inadequate Iron supplement IRON DEFICIENCY ANEMIA Clinical Manifestations: Smooth, sore tongue (Atrophic Glossitis); Stomatitis; Dysphagia Brittle, ridged nails; koilonychia Angular cheilosis Pica Tinnitus CV symptoms (if hgb=7.5g/dL): tachycardia, chestpain, SOB, CHF

Diagnostic findings: BM aspiration- the most definitive method of establishing the diagnosis in Iron deficiency anemia Ferritin and Hemoglobin values- the most reliable laboratory findings in evaluating Iron deficiency anemia IRON DEFICIENCY ANEMIA Medical management: It should be investigated 1st 6-12 months Iron supplement Iron Supplementation ORAL Ferrous Sulfate: pc Ferrous Gluconate: and Ferrous Fumarate: ac PARENTERAL Iron Dextran (Imferon) Oxygen Therapy as needed for SOB Blood Transfussion as necessary

IRON DEFICIENCY ANEMIA Nursing Interventions: Promote Rest to reduce oxygen demands of tissues (priority) Provide good oral care to prevent or relieve stomatitis

Provide good skin care to prevent pressure sores (especially among bedridden elderly patients) Diet Therapy: Iron Rich Foods Organ Meats, Lean Meats, Egg yolk Legumes Green Leafy Vegetables Raisins and other dried fruits

APLASTIC ANEMIA Disorder characterized by bone marrow hypoplasia or aplasia resulting in pancytopenia (dec. RBC, WBC, Plt.) Causes: Idiopatic Ionizing radiation Viral infection Chemical Toxins and Drugs (e.g.Chloramphenicol) APLASTIC ANEMIA APLASTIC ANEMIA APLASTIC ANEMIA MEGALOBLASTIC ANEMIA Types FOLATE DEFICIENCY ANEMIA PERNICIOUS ANEMIA Assessment: (BM Analysis) Megaloblastic RBCs- characteristic feature Hyperlasia- abnormal increase in the no. of cells Pancytopenia- decrease in all myeloid derived cells Poikilocytosis- abnormal shape which widely varied MEGALOBLASTIC ANEMIA Folic Acid/Folate Deficiency Anemia: Folic acid- a vitamin that is necessary for normal RBC production, is stored as compound referred to as folates. Folate- found in green leafy vegetable and liver Causes: Poor dietary intake, Chronic Malnutrition, Anorexia Nervosa Alcoholism Pregnancy Malabsorption Malignancy Prolonged TPN Chronic Hemodialysis Folate Deficiency Anemia Folate Deficiency Anemia Pernicious Anemia Megaloblastic anemia associated with Vit. B12 deficiency

AKA Macrocytic, Hyperchromic anemia Causes: Inadequate dietary intake Absence of IF Ileal resection and Gastrectomy GI disease- ie Crohns dse. Pernicious Anemia Decreased intrinsic factor by parietal cells of the stomach causes dec. B12 absorption. Vit. B12 has to bind with IF to be absorbed in the small intestines Decreased B12 absorption reults to Dec. RBC production Dec. DNA synthesis in maturing RBC (RBCs do not divide normally so they grow bigmegaloblastic cells) Impaired integrity of cells in the GI tact (mouth, stomach, anus), vagina, and axon of neurons Pernicious Anemia Pernicious Anemia Collaborative Management: Monthly vitamin B12 IM for life Folic Acid and Ferrous Sulfate Hydrochloric Acid (HCl), 4-10mL PO well diliuted in water TID with meals during first week of B12 therapy BT as necessary PE every 6 mos. For follow up Higher risk for Gastric Ca, Throid dysfunctions: ANEMIAS IN RENAL DISEASE Patients with ESRD Patients undergoing long term hemodialysis HEMOLYTIC ANEMIAS SICKLE CELL ANEMIA Results from inheritance of the Sickle hemoglobin gene (hemoglobin molecule to be defective) The Sickle hemoglobin (HbS) acquires a crystal-like formation when exposed to low oxygen tension. RBC wirh HbS loses its round, very pliable, biconcave disk shape and becomes deformed, rigid and sickle shaped The long rigid RBCs can adhere to the endothelium of small vessels, when they pile up against each other, blood low to a region or an organ may be reduced

Common on African descent and also people in the Middle east, Mediterraneans and Aboriginal tribes of India SICKLE CELL ANEMIA Complications: Infection Stroke

Renal failure Impotence Heart failure Pulmonary HPN SICKLE CELL ANEMIA Types Sickle cell Crisis: Sickle crisis- the most common and very painful Results to tissue hypoxia and necrosis due to inadequate blood supply Aplastic crisis- results from infection with the human parvovirus Sequestration crisis- results when other organs pool the sickled cells SICKLE CELL ANEMIA Acute Chest Syndrome Manifested by a rapidly falling hemoglobin level, tachycardia, fever and bilateral infiltrates seen on the chest x-ray Infection- major cause SICKLE CELL ANEMIA Medical treatment: 3 Primary treatment modalities: BMT- offers a potential cure Hydroxyurea IncreasesHemoglobin F levels in patient with sickle cell anemia, decreasing the permanent formation of Sickle cell Long term BT: PRBC Prevent complication Prevent acute exacerbation of anemia Improving the response to infection SICKLE CELL ANEMIA Supportive therapy: Aspirin- very useful in diminishing mild to moderate pain NSAIDS Morphine- DOC for pain THALASSEMIA A group of hereditary disorders associated with defective hemoglobin chain synthesis High prevalence in people of Mediterranean, African and Southeast Asians Hypochromic, microcytic anemia The production of one or more globulin chains within the hemoglobin is reduced. This increases rigidity of the RBC and the premature destruction of this cells THALASSEMIA Classification: Alpha Thalassemia Mainly for people in Asia and the Middles East Milder form than Beta The RBC is extremely microcytic Beta Thalassemia Other name thalassemia major or Cooleys anemia

Most prevalent in the Mediterranean population

THALASSEMIA Beta Thalassemia Characterized by severe anemia, marked hemolysis and ineffective erythropoiesis Early transfusion therapy, growth and development through childhood is facilitated Organ dysfunction due to Iron overload Regular chelation therapy (subcutaneous Deferoxamine) BMT- possible cure THALASSEMIA PROLIFERATIVE DISORDERS Proliferative Disorders Erythrocyte Proliferative Disorders Polycythemia Vera: Assessment Polycythemia Vera: Mangement Polycythemia Vera: Nursing Mx The Leukemias The Leukemias The Lymphomas The Lymphomas The Lymphomas The Lymphomas: Chemotherapy Multiple Myeloma Multiple Myeloma BLEEDING DISORDERS Bleeding Disorders Throbocytopenia Thrombocytopenia Thrombocytopenia Autoimmune Idiopathic Thrombocytopenic Purpura Disseminated Intravascular Coagulation Purpura DIC Manifestations DIC Mangement HEMOPHILIA Hemophilia A Caused by a genetic defect that results in deficient or defective factor VIII Occurs in 1:10,000 3X more common Hemophilia B (Christmas defect) Stems from a genetic defect that causes deficient or defective factor IX HEMOPHILIA Both types are inherited as X-linked traits Most affected people are males

Females are carriers The disease are recognized early childhood, usually in the toddler age group Hemophilia occurs in all ethnic group

HEMOPHILIA Clinical Manifestations: Hemorrhages into various parts of the body Hemarthroses and hematoma- particularly severe factor VIII deficiency Most commonly affected joints: Knees Elbows Ankles Shoulders Wrist Hips Spontaneous Hematuria Decreased sensation and weakness Hemophilia HEMOPHILIA Medical Management: Infusion of Fresh Frozen Plasma- the only treatment for Hemophilia (Past) Factor VIII and IX concentrates available to all blood banks. Aminocaproic acid (Amicar)- is a fibrinolytic enzyme inhibitor that can slow the dissolution of blood clots that do form it is effective as an adjunctive measure after oral surgery Desmopressin (DDAVP)- induces a transient rise in Factor VIII levels. Very effective in mild form of Hemophilia A VON WILLEBRANDS DISEASE A common bleeding disorder affecting the males and females equally Usually inherited as a dominant trait Caused by a deficiency on Von Willebrand factor necessary for Factor VIII activity also necessary for platelet adhesion at the site of vascular injury The synthesis of Factor VIII is normal, its half-life is shortened VON WILLEBRANDS DISEASE Clinical Manifestations: Nosebleed Excessive heavy menses Bleeding from cuts Postoperative bleeding Soft tissue or joint hemorrhages VON WILLEBRANDS DISEASE Assessment Findings: Prolonged bleeding time Prolonged PTT VON WILLEBRANDS DISEASE Medical Management:

Administer Cryoprecipitate (contains factor VIII, fibrinogen and Factor IX) Administer Desmopressin Von Willebrands Disease