Disorders of the Hematological System

Blood and Lymph Two of the body’s most important functions are: Transportation Protection-from foreign invaders Achieved through the circulatory and lymph systems Blood is the primary transportation fluid

Blood Cells RBC’s, WBC’s, and Platelets Blood cells are made in bone marrow Other structures involved in the blood cell production are: Kidneys (Erythropoietin) Liver (stores iron) Lymph system (breaks down blood cells, produces WBC)

Red Blood Cell Production (RBC) Erythropoiesis is the process of making red blood cells Stimulated by decreased PO2 The kidneys releases Erythropoietin Reticulocytes are immature RBC’s Iron, folic acid, and Vitamin B12 are needed for RBC production Iron is needed for Hemoglobin production

Red Blood Cells (RBC) Normal Values Males 4.7-6.1 Females 4.2-5.4 Increased: Dehydration, Polycythemia Decreased: Anemia, Leukemia, Post-hemorrhage

Hemoglobin (Hgb) Contained within RBC’s – red pigment Oxygen carrying compound of the RBC Normal Values Males 14-18 g/dL Females 12-16 g/dL Increased: Dehydration, COPD, Polycythemia Decreased: Anemia, leukemia, Post-hemorrhage

Hemoglobin Erythrocytes are classified according to: To measure Hgb, RBC’s are broken and Hgb is released and counted Hemoglobin content is expressed as normochromic or hypochromic anemia Erythrocytes are classified according to: Size, shape and color RBCs size is usually expressed as: macrocytic, microcytic, or normocytic Average life span of an RBC = 120 days

Hematocrit A measure of the packed cell volume of RBCs Expressed as a % of the total blood volume Normal Values Males 42-52% Females 37-47%

Hematocrit Increased: Polycythemia, dehydration Decreased: Anemia, leukemia, hemorrhage, fluid overload

White Blood Cells Have nuclei, are colorless, live from a few days  several years Primarily involved in body defenses such as destruction of bacteria and viruses “Differential” – blood count in which the different kinds of WBCs are counted and reported  can discriminate between virus and backteria

Platelets Also called “thrombocytes Normal Values 150,000-400,000 cells/microliter – involved with clotting 5-9 day life span Increased- Polycythemia, granulocytic leukemia Decreased- Bleeding, thrombocytopenia, Chemotherapy

Erythrocyte Indices MCV, MCH, MCHC Will help determine type of anemia Iron Deficiency Folic Acid Deficiency B12 Deficiency Definition of Corpuscular: A living cell

Mean Corpuscular Volume (MCV) Volume (size) of the RBC Hematocrit divided by RBC Count Low RBC = Microcytic (small) Iron Deficiency Anemia High RBC = Macrocytic (large) B12 Folic Acid Deficiency Anemia

Mean Corpuscular Hemoglobin (MCH) Weight of hemoglobin in a RBC Hemoglobin divided by the total RBC count Low Hypochromic (Deficient of hemoglobin) Iron Deficient Anemia High Hyperchromic- Lab error

Mean Corpuscular Hemoglobin Concentration (MCHC) Concentration of hemoglobin in the RBC The hemoglobin is divided by the RBC Low Hypochromic (deficient of hemoglobin) Iron Deficiency Anemia High Hyperchromic Lab Error

Blood Types Genetically determined Determined by the presence or absence of specific antigens on the outer surface of the RBC In certain blood types, the antigens on the RBC are accompanied by antibodies in the plasma.

Blood Types O – Good Donor, Bad Receiver Contains A and B antibodies at birth A- Very selective- receives A only Contains B antibodies at birth B- Very selective- receives B only Contains A antibodies at birth AB – Good Receiver, Bad Donor Does not contain A or B antibodies

Rh Factor Located on the surface of RBCs “positive” = have the factor “negative” = do not have the factor When an Rh- person has been exposed to Rh+ blood, antibodies develop within 2 weeks No reaction with this first exposure.

Rh Factor On any exposure thereafter, the Antibodies will attack Rh positive blood. “Rh incompatibility” = mother’s antibodies hemolyze the fetal RBCs  rupture and loss of contents of cells

Rh Factor RhoGAM – intramuscular (or IV) injection Action: desensitization antibodies Next baby’s intrautero development without the potential complications associated with Rh incompatibility Who (include blood type and Rh factor) will need to receive Rhogam? A women who is Rh Negative and is pregnant (prophylaxis) or has just given birth (baby is Rh positive)

Rh Factor When is RhoGAM given? 2 injections 1. between 20-28wks pregnancy 2. within 72 hrs. post partum

Who’s the Best? Which blood type (including Rh factor) is the universal donor? O negative Which blood type is the universal recipient? AB positive

Lymphatic System

LYMPHATIC SYSTEM A subdivision of the cardiovascular system Consists of: Lymph vessels Lymph fluid Lymph tissue Basic Functions: Maintenance of fluid balance Production of lymphocytes Absorption and transportation of lipids from intestine to bloodstream

LYMPHATIC SYSTEM Lymph is a specialized fluid formed in the tissue spaces and transported by way of lymphatic vessels to eventually  reenter the circulatory system. Lymphatic Tissue Lymph nodes Tonsils Spleen Thymus

DISORDERS OF THE HEMATOLOGIC AND LYMPHATIC SYSTEMS

Anemia Characterized by low RBC count, ↓ hematocrit and hemoglobin Look at CBC indices to determine RBC health Anemia causes delivery of an insufficient supply of oxygen AND nutrients to the cells Loss of the O2-carrying element in the blood results in a supply/demand imbalance in vital organs.

Anemia Causes Hemorrhage Impaired production of RBC’s Increased destruction of RBC’s (hemolysis) Nutritional deficiencies

Diagnostic Tests: Coombs’ Test Hemoglobin Electrophoresis Schilling Sickledex TIBC Gastric Analysis

Coombs Test Direct Antiglobulin test – identifies autoantibodies against RBCs Coombs + = autoimmune hemolytic anemia, hemolytic disease of the newborn, lymphomas, LE, mycoplasma infection, and infectious mononucleosis

General Signs and Symptoms of Anemia Big five DOE Chest pain Palpitations(Tachycardia) Dizziness (Vertigo) Pallor Anorexia Disorientation Dyspepsia Fatigue Headache Insominia

Hypovolemic Shock May be caused by severe dehydration or extreme blood loss May require 1000 mL loss before severe s/s Initial s/s: Thirst Weakness Restlessness Later s/s Hypotension Tachycardia and weak, thready pulse Pallor and cold, clammy skin Oliguria Disorientation Prostration

Hypovolemic Shock H&H will not decrease until ~6-8 hours Treatment If bleeding source obvious: direct pressure If not, intervention ASAP IV fluids (Isotonic/Volume expanders: NS) O2 O negative blood transfusion Platelets Plasma Factor 7

Nursing Interventions Monitor blood and fluid restoration Identify blood loss sites to control bleeding VS frequently I/O – particularly careful monitoring of urine output Patient teaching

Pernicious Anemia Caused by autoimmune disease: There is an absence of glycoprotein intrinsic factor that is usually secreted by the stomach and assists with the absorption of Vitamin B12. Without this factor, Vitamin B12 is unavailable for the maturation of the Red Blood Cell Production.

Signs and Symptoms Subjective Objective Palpitations Nausea Flatulence Indigestion Soreness and burning of the tongue Tingling of the hands and feet Impaired proprioception Objective Smooth and erythematous tongue Gum infection Mental disorientation Personality changes Behavior problems Severe neurological impair from destroyed Spinal Cord Tracts

Diagnostic Tests Gastric Analysis – determine pH Schilling test Subject will get two doses of vitamin B-12 (cobalamin). The first dose is taken by mouth. The second dose is radioactive and is given as a shot 2-6 hours later. Urine is tested for radioactive B12 Reveals malabsorption of Vit B12 Serum Megaloblastic Anemia Profile (serum level of B12) soon to replace Schilling test Will reveal serum levels of Vit B12 + more Gastric Analysis – determine pH

Medical Management Medication B 12 injections Folic acid supplement Iron replacement Transfused with Packed RBC if severe anemia CBC every 3-6 months

Iron-deficiency Anemia Plenty of RBC, normal H &H sometimes, but hypochromic Causes Diet Usually chronic bleeding Body’s demand exceeds its absorption Pregnancy Infants Young adolescents Malabsorption of iron as in celiac disease or sprue Subtotal gastrectomy because iron needs an acid environment to be absorbed.

Iron Deficiency Anemia Signs and Symptoms Sore, Swollen Tongue Cracks in the side of the mouth Brittle nails Increased frequency of infections PICA- Ice, dirt, paint etc. Restless Leg Syndrome

Iron Deficiency Anemia Medical Management Because less than 10% of iron is absorbed in the duodenum, high doses of Iron supplement are needed Additional Vitamin C has shown to enhance iron absorption See p. 277 Box 7-2 for food sources of nutrients needed for Erythropoiesis

Z-track Because some patients cannot tolerate oral preparations of iron, Iron can be given IV or IM. The z-track method of giving iron dextran is preferred to prevent staining

Sickle Cell Anemia EMcrit.org Genetic --Recessive homozygous Predominately found in the African and African-American Population First crisis usually occurs at 10-12 weeks of age Pathophysiology: After the hemoglobin molecules give up their oxygen, some of them may cluster together and form long, rod-like structures.

Sickle Cell Anemia A sickle cell is an abnormal, crescent-shaped RBC containing hemoglobin S (a defective hemoglobin molecule) Sickle Cell crisis – an episode of acute “sickling” of RBCs which causes occlusion and ischemia in distal blood vessels Sickling  clumping or aggregation of these misshapen RBCs which lodge in small vessels

Health maintenance for patients with sickle cell disease starts with early diagnosis, preferably in the newborn period and includes penicillin prophylaxis, vaccination against pneumococcus bacteria and folic acid supplementation. Pulmonary Hypertension

Sickle Cell Maintenance Health maintenance for patients with sickle cell disease Starts with early diagnosis, preferably in the newborn period Vaccination against pneumococcus bacteria (infections are a major complication of Sickle-Cell anemia) Folic acid supplementation Prevention of infection and dehydration

Medical Management Supportive Pain is biggest problem Rehydration PCA pump is indicated Long-term MS therapy with breakthrough pain meds Rehydration Packed cells for anemia but clients have problems with iron overload Probable treatment/cure is stem cell transplant

Aplastic Anemia aka Hypoplastic Anemia Congenital Aplastic Anemia is caused by a chromosomal alteration Acquired Aplastic Anemia related to chemical exposures. Cause of 70% of acquired aplastic anemias remains unknown (idiopathic)

Aplastic Anemia People are pancytopenic when: Reduced or absent red blood cells Reduced or absent white blood cells Reduced or absent platelets

Aplastic Anemia Signs/Symptoms: Pallor Bleeding tendencies Dyspnea Palpitations Repeated infection with high fevers Fatigue, weakness General malaise

Diagnostic Tests Diagnostic tests Bone Marrow Aspiration Hypoplastic or aplastic fatty deposits CBC may show normocytic and normochromic blood cells

Medical Management Need to know cause! Blood transfusion with caution (need to preserve immune system for bone marrow transplant) Spleenectomy if spleen is enlarged Steroids and/or androgens used to stimulate bone marrow Immunosuppressive therapy or cyclosporine

Bone Marrow Transplant Involves taking cells that are normally found in the bone marrow (stem cells), filtering those cells, and giving them back either to the patient or to another person. The goal of BMT is to transfuse healthy bone marrow cells into a person after their own unhealthy bone marrow has been eliminated. Stem cells, when transplanted, find their way to the recipient's marrow and begin to differentiate and produce all types of blood cells that are needed by the body.

Polycythemia Abnormal ↑ in RBC’s Two types Primary Secondary Myeloproliferative disease: overproduction by the bone marrow Related to a Stem Cell abnormalityIncreased WBC, RBC and platelets Secondary Usually related to chronic hypoxia – a physiologic response to compensate for hypoxia Increased RBC Chronic hypoxia maybe d/t: pulmonary disease, CV disease, high altitude, tissue hypoxia

Polycythemia Vera Signs and Symptoms: ↑ blood volume and viscosity  HTN, angina, HF, thrombophlebitis Venous distention GI bleeding Hepatomegaly and splenomegaly from organ engorgement

Polycythemia Vera Diagnostic Tests: H and H (Plasma and RBC volume ↑) Reticulocyte and Erythrocyte counts Platelets and RBC’s Bone Marrow exam

Polycythemia Vera

Polycythemia Vera Medical Management Repeated plebotomy Myelosuppressive agents Allopurinol for gout True Chronic Disease requires lifetime treatment Population can be non-compliant until very ill Death by thrombosis

Disorders of Leukocytes

Agranulocytosis Severe reduction of Granulocytes Basophils Eosinophils Neutrophils Low WBC count (leukopenia) Usually caused by medication and/or toxic substances(includes herbal substances!)

Agranulocytosis Signs and Symptoms: Fever and chills Headache and fatigue Infections Ulceration of the mucous membranes Pneumonia and UTIs

Medical Management Need to know the cause Transfusion of Packed RBC G-CSF (filgrastim or Neupogen) Neutropenic precautions (Reverse Isolation) Death by Infection

Nursing Interventions A patient with a compromised WBC system is highly susceptible to life-threatening infections. List nursing interventions directed toward protecting the patient from potential sources of infection.

Leukemia Malignancy involving the hematopoetic system. Characterized by rapidly developing white cells that overwhelm the bone marrow, lymph system, spleen and liver Complications include infiltration and damage to organs as well as the central nervous system Increased susceptibility to infections

Leukemia Four Major Types: Acute lymphocytic leukemia (ALL) Acute Myelogenous leukemia (AML) Chronic Myelogenous leukemia (CML) Chronic Lymphocytic leukemia (CLL)

Leukemia S/SX Irrelevant complaints at first Petechiae/Ecchymosis Malaise Fatigue Irritability Petechiae/Ecchymosis Mucous membrane bleeding Bone pain Meningial irritation Enlarged lymph nodes

Leukemia Diagnostic Tests WBC ↓, ↑, or excessively ↑ Bone Marrow Bx. CXR Lymph Node Bx. LP, CT scan

Medical Management Chemotherapy – interrupt cell growth at multiple points in the cell cycle Bone marrow transplant and stem cell therapy Death by infection, hemorrhage and/or Graft-Verses-Host Disease if BMT treated More to follow in Oncolgy Nursing

Nursing Interventions Prevention of Infection Patient education Pain Control Psycho-social support for coping

Coagulation Disorders

Disorders of Coagulation Release of blood from the vascular sys. results from: trauma or vessel damage vessel inadequacy disturbance of the function of platelets or clotting factors, or liver disease ( impairment of clotting mechanism) Disorders can be congenital, acquired, or 2nd to disease or medication toxicity

Thrombocytopenia “Who Ate my Platelets?” Idiopathic Thrombocytopenia (auto-immune disease) Aplastic anemia Leukemia Tumors Chemotherapy Drugs Alcohol Infections/Sepsis

Thrombocytopenia Platelets < 150,000 (nml = 150,000-400,000/mmᶟ) S/SX Petechiae and eccymosis on the skin Frank Bleeding Low Platelet count on CBC With extremely low platelet counts, fatal central nervous system or gastrointestinal hemorrhage can occur

Thrombocytopenia Management Corticosteroid therapy Splenectomy if needed IVIG Immunosuppresive drug therapy Platelets to increase count to 20,000 Plasmapheresis Plasmapheresis involves selective removal of the patient's plasma, which is then replaced by albumin solution. Time time needed to complete a treatment usually ranges from 1 1/2 - 3 hours. 

Hemophilia Disease of the royal family (don’t marry your cousin!) X-linked hereditary trait Hemophilia

Hemophilia Clinical Manifestations: Diagnostic Tests Internal or external hemorrhage with large ecchymoses into tissue Hemarthrosis = bleeding into a joint Swelling, fever, pain Diagnostic Tests Blood work for Factors VIII or IX absent or deficient

Medical Management Replacement therapy—giving or replacing the clotting factor that’s too low or missing. Most common: antihemophilic factor VIII is missing Hemophilia B is missing factor IX Check out the National Hemophilia Society website-www.hemophilia.org

Hemophilia Crisis Usually involves trauma After an IM injection Usually involves trauma Ice and elevate above heart Apply pressure to bleeding Immobilization of limb Pain Management Can be treated with cryoprecipitate (FFP) Medic alert bracelet should be worn at all times

HIV and Hemophilia Usually associated with infusions prior to 1984 Heat inactivation of the virus has significantly reduced the client’s exposure to HIV

vonWillebrand’s Disease Mild form of Hemophilia S/Sx Slow coagulation of blood (as opposed to none) Spontaneous GI Bleeds Epistaxis Gingival Bleeding Common occurrences: post partum, menorrhagia, post op

vonWillebrand’s Disease Medical Management: Usually treated with Cyroprecipitate (containing Factor VIII) Fibrinogen Fresh plasma Desmopressin (DDAVP): Synthetic ADH (vasopressin)

Disseminated Intravascular Coagulation A disorder in which the proteins that control blood clotting become abnormally active  Small blood clots form in blood vessels Plasma clotting factors are depleted during widespread clotting within small vessels  bleeding disorder and thrombosis. Primary disorder initiates a generalized intravascular clotting

High Risk Diagnosis pg. 290, AHN

Medical Management Supportive in nature Heparin therapy to calm the hypercoagulopathy Lab tests confirm dx Serum fibrinogen - low (PT) - high (PTT) - high Platelets: depressed D-Dimer(fibrin breakdown): elevated

Multiple Myeloma A malignant neoplastic immunodeficiency disease of the bone marrow Neoplastic plasma cells infiltrate the bone marrow  destruction of osseous tissue, esp. flat bones  pain, fractures, and skeletal deformities “Monoclonal Protein” – the specific immunoglobulin produced by the myeloma cells

Disrupts erythrocyte, platelet and leukocyte production Electrolyte disturbance from release of Calcium and Phosphorus from deteriorating bone cells Gradual onset so it slips right by practitioners Could present itself as a spinal fracture with cord compression

Signs and Symptoms Bone pain Fatigue Feeling ill Fever Night sweats Weight loss is not common in the early stages.

Multiple Myeloma Physically, patients are pale with diffuse bone tenderness, especially around the sternum (breastbone) and pelvis (hips). Pathologic fractures (fractures caused by tumors) occur frequently. The spine is the most common location for a pathological fracture. It can also happen in the ribs and pelvis.

Diagnostics Radiographic skeletal studies Bone marrow aspiration Labs Pancytopenia (low cells) Hypercalcemia Hyperuricemia Elevated creatinine Urinalysis High protein

Multiple Myeloma Medical Management Supportive Radiation and chemotherapy to reduce or impede tumor growth Hypercalcemia tx. Pain management

Multiple Myeloma Nursing Interventions: Pain management Prevention infection Prevention bone injury Administration Chemo Maintain hydration I/O Weight-bearing Patient Education

Hodgkin’s Disease Enlargement of lymphoid tissue Affects men 2:1 Look! It’s Math! ’m so happy! Enlargement of lymphoid tissue Affects men 2:1 Bimodal peak (2 separate populations) Early 20-30 Late 60-70 Accounts for <1% of all cancer cases Differentiated by Lymphocyte cytology, Reed-Sternberg cell

Hodgkin’s Lymphoma Hodgkin's disease may occur in a single lymph node, a group of lymph nodes, or, sometimes, in other parts of the lymphatic system such as the bone marrow and spleen. This type of cancer tends to spread in a fairly orderly way from one group of lymph nodes to the next group.

Hodgkin’s Lymphoma Risk factors associated with this disease: Age/Sex -- Hodgkin's disease occurs most often in people between 15 and 34 and in people over the age of 55. It is more common in men than in women. Family History -- Brothers and sisters of those with Hodgkin's disease have a higher-than-average chance of developing this disease. Viruses – Epstein-Barr virus is an infectious agent that may be associated with an increased chance of getting Hodgkin's disease.

Hodgkin’s Lymphoma Signs/Symptoms A painless swelling in the lymph nodes in the neck, underarm, or groin Unexplained recurrent fevers Night sweats Unexplained weight loss Itchy skin

Hodgkin’s Lymphoma Diagnostic studies CBC: elevated WBC ESR X-rays CT MRI Presence of Reed-Sternberg cell Reed-Sternberg cell: characterized by mirror image nuclei

Medical Management Radiation against localized tumors Chemotherapy for early and late stages Candidates for Neupogen because of suppressed neutrophil production High risk for secondary malignancies especially leukemia

Non-Hodgkins Lymphoma Absence of the Reed-Sternberg Cell Usually described as “all other lymphomas” More likely to spread beyond the lymph nodes Comprised of approximately 10 different subtypes and 20 different disease entities These subtypes are grouped into 3 biologic states- low grade, intermediate grade, and high grade lymphomas

Non-Hodgkins Lymphoma S/Sx Fatigue Malaise Anorexia

Non-Hodgkins Lymphoma Diagnostic Tests: Anemia Increased platelets Leukocytosis(increased and abnormal WBCs) ESR-elevated Hypercalcemia and elevated Alkaline Phosphatase Positive Coombs test Chest Xray CT scan

Non-Hodgkins Lymphoma Medical Management Therapy is determined by several factors: biologic state of the lymphoma stage of lymphoma presence or absence of symptoms (e.g., weight loss, night sweats, organ dysfunction) overall general health of the patient Chemo and radiation

Non-Hodgkin’s Lymphoma Nursing Interventions Supportive care during radiation and chemo Observation for complications Patient education Participate in care planning