Management of Clients with Hematologic Disorders

Alapan, Nikki Bermudez, Igi Buenaventura, Hayzel Gomez, Lyle

Hematologic Disorders
1.Disorders affecting Red Blood Cells 2.Disorders affecting White Blood Cells 3.Disorders of Platelets and Clotting Factors

Disorders Affecting Red Blood Cells

1. Anemia
A. Anemia caused by Decreased Erythrocyte Production B. Anemia caused by Increased Erythrocyte Production

Anemia
Anemia - a clinical condition that results from an insufficient supply of healthy red blood cells (RBCs), the volume of packed RBCs, and/or the quantity of hemoglobin. Hypoxia - results when the bodys tissues are not adequately oxygenated.

Etiologic Categories of Anemia

1. Decreased RBC Production 2. Increased RBC Desruction (Hemolysis) 3. Blood Loss

Decreased RBC Production

Defective DNA Synthesis Cobalamin/Vitamin B12 Deficiency Folic Acid Deficiency Decreased Hemoglobin Synthesis Iron Deficiency Thalassemia (Decreased Globin Synthesis) Sideroblastic Anemia (Failure To Completely Form Heme) Decreased Number of Erythrocyte Precursors Aplastic Anemia Anemia of Leukumia and Myelodysplasia Chronic Diseases

Increased RBC Destruction

Intrinsic
Abnormal Hemoglobin (Sickle Cell Anemia) Enzyme Deficiency Membrane Abnormalities

Extrinsic
Physical Trauma Antibodies (Autoimmune and Isoimmune) Infectious Agents Toxins (Snake Venom; Chemotherapy)

Blood Loss
Acute
Trauma Blood Vessel Rupture

Chronic
Gastritis Hemorrhiods Menstruation

Anemia
Pathophysiology

Transport of oxygen is impaired Hemoglobin is lacking or the number of RBCs is too low to carry adequate oxygen to tissues Hypoxia develops Increasing the rate of RBC production

Anemia
Pathophysiology

Increasing cardiac output by increasing stroke volume or heart rate Redistributing blood from tissues of low oxygen needs to tissues with high oxygen needs Shifting the oxygen-hemoglobin dissociation curve to the right

Anemia caused by Decreased Erythrocyte Production

1. Iron Deficiency Anemia 2. Thalassemia 3. Megaloblastic Anemia A. Pernicious Anemia or B12 Deficiency B. Folic Acid Deficiency Anemia

Iron Deficiency Anemia

chronic, hypochromic, microcytic anemia results from insufficient supply of iron in the body without iron, hemoglobin concentration in the RBCs is reduced the cells are unable to oxygenate the body tissues adequately

Iron Deficiency Anemia

Pathophysiology Iron is present is in all RBCs as heme in Hb Iron concentration in the body is regulated by the absorptive cells in the proximal small intestines Errors in this balance leads to anemia

Iron Deficiency Anemia

Diagnostic Test

CBC Radiographic Studies Stool examination Esophagoscopy Gastroscopy Sigmoidoscopy

Iron Deficiency Anemia

Clinical Manifestation MILD CASE OF IDA Asymptomatic SEVERE CASE OF IDA Fatigue Headache Dyspnea Palpitations

Iron Deficiency Anemia

Clinical Manifestation SEVERE CASE OF IDA Pallor in the face, palm of hand, nail bed and mucous membranes of the mouth and conjuctiva Angular Stomatitis inflammaton of the mucosa of the mouth Glossititis inflammation of the tongue

Iron Deficiency Anemia

Clinical Manifestation SEVERE CASE OF IDA Cheilitis inflammation of the lips Brittle nails

Iron Deficiency Anemia

Nursing Intervention Monitor the client to ensure compliance in taking supplemental iron Diets high in iron should e planned with the client and family members.

Thalassemia

an autosomal-recessive genetic disorder results in inadequate normal Hb production disrupts the synthesis of globin

Thalassemia
Pathophysiology ALPHA-THALASSEMIA mutation of alpha-globin gene(s) THALASSEMIA MINOR 1 beta-globin gene is mutated minor disruptions in beta-globin synthesis

Thalassemia
Pathophysiology THALASSEMIA MAJOR mutation exists in both beta-genes significant impairment of beta-globin synthesis marked reduction in hemoglobin production profound anemia

Thalassemia
Pathophysiology HEMOLYSIS from an imbalance in the alpha- and betaglobin chains which are normally paired excess unpaired alpha- or beta-globin chains aggrevate and form a precipate damages RBC membranes intravascular hemolysis

Thalassemias

Diagnostic Test
CBC Amniocentesis

Thalassemia

Clinical Manifestation ALPHA-THALASSEMIA Asymptomatic THALASSEMIA MINOR Symptoms of mild to moderate Anemia

Thalassemia
Clinical Manifestation THALASSEMIA MAJOR pain failure to thrive diarrhea splenomegaly hepatomegaly Jaundice Bone marrow hyperplasia

Thalassemia Nursing Intervention Educate the patient about the disease Give the patient a list of medications to avoid

Megaloblastic Anemia
1. Pernicious Anemia or B12 Deficiency 2. Folic Acid Deficiency Anemia

Pernicious Anemia Cobalamin/B12 Deficiency

autoimmune disorder Absence of intrinsic factor in gastric secretions malabsorption of Cobalamin

Pernicious Anemia B12 Deficiency

Diagnostic Test Schilling Test- patient receives small oral dose of radioactive Vitamin B12 followed in a few hours by a large nonradioactive Vitamin B12 . If the Vitamin B12 is absorbed more than 8% will be excreted in the urine within 24 hours.

Pernicious Anemia B12 Deficiency

Pathophysiology Faulty absorption from the GI tract Absence of Intrinsic Factor No Vitamin B12 absorption Erythrocyte production is eventually diminished

Pernicious Anemia B12 Deficiency

Clinical Manifestation

Classic signs of anemia Neurologic manifestations with severe anemia (<7 g/dl)
Vitamin B12 is necessary for the synthesis of myelin Parasthesias of feet and fingers Ataxia Loss of position and vibration sense Spasticity

Pernicious Anemia B12 Deficiency

Clinical Manifestation

Sore tongue, beefy red (unknown cause) Hyperbilirubinemia

Jaundice/icterus Lemon yellow skin combination of pallor and jaundice

Hepatosplenomegaly enlarged liver and spleen

Pernicious Anemia B12 Deficiency

Nursing Intervention Monitor B12 levels Monitor Hemogram and reticulocytes Give Folic acid with B12 for severe anemia Folic acid caution

Folic Acid Deficiency Anemia

Folic acid deficiency anemia happens when your body does not get enough folic acid. Folic acid is one of the B vitamins, and it helps your body make new cells, including new red blood cells

Folic Acid Deficiency Anemia

Pathophysiology Malaabsorptive diseases of the small bowel, such as sprue, may not absorb folic acid normally

Folic Acid Deficiency Anemia

Diagnostic Test

CBC Clinical Manifestations Folic acid levels Bone marrow aspiration and analysis

Folic Acid Deficiency Anemia

Clinical Manifestation Classic signs of anemia No neurologic manifestations Manifestations related to malnourishment
painful ulcerations of cheeks and tongue fissuring of lips and mouth

Gastrointestinal symptoms
Dysphagia Flatulence Watery diarrhea

Folic Acid Deficiency Anemia

Nursing Intervention
Monitor CBC and reticulocytes If malabsorption is cause, diet alone will not be sufficient Oral is preferred

Anemia caused by Increased Erythrocyte Production

1. Hemolytic Anemia 2. Sickle Cell Disease 3. Polycythemia Vera 4. Hemochromatosis

Hemolytic Anemia
An end result of condition that lead to hemolysis Hemolysis- premature destruction of erythrocytes Anemia occurs when the bone marrow fails to replace RBCs at the rate they are destroyed

Hemolytic Anemia

Diagnostic Test

CBC Peripheral smear Morphologic examination RBC indices Increased RBC distribution width (RDW) Reticulocyte count

Hemolytic Anemia
Diagnostic Test Lactic acid dehydrogenase level Serum haptoglobin level Indirect bilirubin level

Hemolytic Anemia
Clinical Manifestation Weakness Fatigue Hemochromatosis-Bronze skin tone and diabetes Hemoglobinuria dark urine Jaundice Elevation in Bilirubin levels Enlargement of spleen and liver

Sickle Cell Disease

Group of inherited disorders of mutant hemoglobin Sickling of RBCs Low oxygenation Abnormally shaped RBCs Sickle Cell Anemia autosomal-recessive disorder Sickle Cell Trait much milder form

Sickle Cell Disease

Pathophysiology Defective hemoglobin exposed to a decrease 02 Decreased solubility Sickle Cell becomes rigid, sticky and fragile Sickle cell agglutinate and impede circulation in the capillaries Microinfarction tissue hypoxia and further sickling

Sickle Cell Disease

Diagnostic Test Low Hematocrit Sickled Cells on Smear Hemoglobin Electrophoresis - a blood test done to evaluate the different types of hemoglobin in the bloodstream.

Sickle Cell Disease

Clinical Manifestation Jaundice is characteristic and is usually obvious in the sclerae Enlargement of the face and skull Tachycardia Cardiac murmurs Cardiomegaly enlarged heart Dysrhythmias Heart Failure

Sickle Cell Disease

Nursing Intervention Manage Pain Prevent/Manage Infection Promote Coping Skills Minimize Deficient Knowledge

Polycythemia Vera
Primary polycythemia Profilerative disorder Myeloid stem cells seem to have escaped normal control mechanisms

Polycythemia Vera
Pathophysiology The bone marrow is hypercellular, and the erythrocyte, leukocyte, and plateple counts in the peripheral blood are elevated Spleen resumes its embryonic function of hematopoiesis and enlarges The bone marrow becomes fibrotic, with a resultant inability to produce as many cells

Polycythemia Vera
Diagnostic Test CBC Elevated erythrocyte mass Leukocyte and Platelet count Erythropoietin level Elevated hematocrit

Polycythemia Vera
Clinical Manifestation Ruddy complexion Splenomegaly Increased Blood Volume
Headache Dizziness Tinnitus Fatigue Paresthesias Blurred Vision

Polycythemia Vera
Clinical Manifestation Increased Blood Viscosity
Angina Claudication Dyspnea Thrombophlebitis

Elevated Blood pressure

Polycythemia Vera
Nursing Intervention The nurses role primarily that of educator.
Risk factors for thrombotic complications Avoid tight and restrictive clothing Avoid crossing the legs Sedentary behavior should be encouraged Minimize alcoholism Proper Diet

Hemochromatosis
Iron overload disease Inherited metabolic disorder Increased absorption of iron that is deposited in the body tissues and organs particularly the liver, heart and pancreas

Hemochromatosis
Diagnostic Test Laboratory Blood Test Liver Biopsy Transferring Saturation Test calculate how much iron is bound to the protein that carries iron to the blood Serum Ferratin Test shows the level of iron in the liver

Hemochromatosis
Clinical Manifestation

Joint Pain Fatigue Lack of energy Irritability Depression Loss of body hair Abdominal pain Lose of sexual drive Heart Problems

Hemochromatosis
Nursing Intervention Should not take Iron supplements Avoid Alcoholic Beverages Dietary intake of iron is limited

Disorders Affecting White Blood Cells

1. Agranulocytosis 2. Multiple Myeloma 3. Infectious Mononucleosis 4. Splenic Rupture and Hypersplenism

Agranulocytosis
(granulocytopenia, malignant neutropenia) is an acute, potentially fatal blood dyscrasia profound neutropenia (a reduced number of circulating neutrophils) Because neutrophils make up roughly 93% of all granulocytes, the terms neutrophenia and agranulocytosis are often used interchangeably. Agranulocytosis is a fairly rare condition. For unknown reasons, females are much more susceptible to this condition than males, although even among females, its relatively rare.

Agranulocytosis
Pathophysiology Failure to produce adequate numbers of WBC prevents normal surveillance and phagocytosis; infection from the neutropenia is a common sequel. Neutrophils constitute a swift and powerful defense against invading microorganisms. Consequently, decreases in their number result in a greater susceptibility to bacterial invation, especially when the clients absolute neutrophil count drops below 500/mm3.

Diagnostic Test Complete blood count- leucopenia, evidenced by WBC counts of 500 to 3000/mm3 with extreme reduction in polymorphonuclear cells (0 to 2%) Bone marrow examination Urine culture, blood and ulcerative lesions in the throat and mouth that are positive for bacteria, usually gram-positive cocci. A history of exposure to an offending agent as well as all the aforementioned findings

Agranulocytosis

Agranulocytosis
Clinical Manifestation Fatigue and weakness Sore throat Ulcerations of the pharyngeal and buccal mucosa, Dysphagia High fever Weak and rapid pulse, and sever chills

a B- cell neoplastic condition abnormal malignant proliferation of plasma cells secreting a monoclonal paraprotein, accumulation of mature plasma cells in the bone marrow, Risk factors include an increase incidence in some families, ionizing radiation and occupational chemical exposure.

Multiple Myeloma

Pathophysiology an abnormal proliferation of plasma cells. With this overproduction of plasma cells, bone destruction also occurs. disruption of RBC, leukocyte and platelet production, with result from plasma cells crowding the bone marrow. Impaired production of these cells forms causes anemia, increased vulnerability to infection and bleeding tendencies, respectively.

Multiple Myeloma

Diagnostic Test Radiographic Studies- reveal diffuse lesions in the bone, widespread demineralization and osteoporosis. Urine examination- Bence- Jones protein in the urine, consisting of monoclonal immunoglobulin light chains. Bone marrow biopsy- the bone marrow contains large numbers of immature plasma cells. Normally, plasma cells constitute 5% of the bone marrow cell population. Blood examination- blood samples sent for plasma electrophoresis reveal a large amount of abnormal immunoglobulin.

Multiple Myeloma

Multiple Myeloma
Clinical Manifestation Back pain or bone pain that worsen with movement. Pathologic fractures accompanied by severe pain. Sternum and rib cage deformities. Diffuse osteoporosis.

Multiple Myeloma
Nursing Intervention Patients also need to be instructed about the signs and symptoms of hypercalcemia. Maintaining mobility and hydration is important to diminish exacerbations. Renal function should also be monitored closely. Maintaining high urine output (3 L/day) can be very useful in preventing this complication. Patients need to be educated about activity restrictions (e. g., lifting no more than 10 pounds, use of proper body mechanics). Pain management. NSAIDs can be very useful for mild pain or in combination with opioid analgesics.

Infectious Mononucleosis
Infectious mononucleosis glandular disease or the kissing disease, a self- limiting condition characterized by painful enlargement of the lymph nodes, lymphocytosis, sore throat and fever. Primarily a disease of the young, it usually strikes children between the ages of 3 and 5 years and young adults between the ages of 15 and 25 years old.

Infectious Mononucleosis
The greatest incidence occurs among college students, medical students and nurses. Although this disease usually occurs sporadically, epidemic forms may sweep through colleges and childrens homes.

Infectious Mononucleosis
Pathophysiology

The cause of infectious mononucleosis is Epstein- Barr virus (EBV), a herpes virus. mode of transmission remains unknown, the disease may be transmitted through the oropharyngeal route during close contact, as with kissing.

Infectious Mononucleosis
Diagnostic Test Paul- Bannel test Complete Blood Count- WBC count usually ranges from 12,000 to 20,000/mm3, of which 50% are lymphocytes and monocytes and 10% to 20% are large, atypical lymphocyte. The mono spot test is also performed with a throat swab- it detects anti- EBV antibodies and is positive in 50% of cases within the first week and 90% of cases in the fourth week.

Infectious Mononucleosis
Clinical Manifestation Fatigue Headaches Malaise Myalgias Temperature up to 39 C (102.2F) Pharyngitis

Infectious Mononucleosis
Nursing Intervention Bed rest is recommended until fever is resolved. Acetaminophen, cool sponge bath, and a large fluid intake help control fever. Warm saline throat irrigation may relieve the sore throat Aspirin is avoided because of risk of Reyes syndrome Contact sports must be avoided to reduce the risk of splenic rupture.

Splenic Rupture and Hypersplenism

The most frequent indication for splenectomy is rupture of the spleen complicated by severe hemorrhage. Causes of splenic rupture include:
Trauma Accidental tearing of the splenic capsule during surgery on neighboring organs. Disease of the spleen that causes softening or damage

hypersplenism, a second important indication for splenectomy, the spleen destroys, in excessive numbers, one of the blood cell types Primary hypersplenism occurs in idiopathic thrombocytopenic purpura and congenital spherocytosis.

Splenic Rupture and Hypersplenism

Diagnostic Test Laboratory indication for splenectomy include granulocytopenia of less than 500/mm3 and thrombocytopenia of less than 20,000/mm3.

Splenic Rupture and Hypersplenism

Clinical Manifestations Moderate to massive splenomegaly Anemia Leucopenia or thrombocytopenia A compensatory increase in the production of the affected cell line by the bone marrow.

Disorders of Platelets and Clotting Factors

Disorders of hemostasis that affect platelets and clotting factors 1. Hemorrhagic Disorders 2. Immune Thrombocytopenic Purpura 3. Coagulation Disorders 4. Disseminated Intravascular Coagulation 5. Hemophilia

Hemorrhagic Disorders
Normal clot formation and lysis depend on 1. Intact blood vessels 2. An adequate number of functioning platelets 3. Sufficient amounts of the 12 clotting factors 4. A well-controlled fibrinolytic system

Hemorrhagic Disorders
Four basic problems underlying hemorrhagic disorders: 1. weak, damaged vessels 2. Platelet deficiency result from hypoprofileration, excessive pooling of platelet in spleen, excessive platelet destruction 3. Deficiency or total lack of one of the clotting factors 4. Excessive or insufficient fibrinolysis

Immune Thrombocytopenic Purpura

Most commmon thrombocytopenic disorder Idiopahitc thrombocytopenic purpura Cause was unknown Hemorrhagic autoimmune disease Destruction of platelets

Immune Thrombocytopenic Purpura Diagnostic Test

A platelet count below 100,000/mm Prolonged bleeding time with normal coagulation time Increased capillary fragility as demonstrated by the torniquet test Positive platelet anitbody screening Bone marrow aspirate containing normal or increased numbers of megakaryocytes

Coagulation Disorders
defect in clotting mechanism one or more of the clotting factors is depleted or absent 1. Disseminated intravascular coagulation 2. Hemophilia

Disseminated Intravascular Coagulation

loss of balance between clotting and lysing systems caused by the simultaneous presence of thrombin and plasmin Fibrinolysis too much plasmin Thrombosis too much thrombin Consumptive Coagulopathy- clotting factors are consumed to such an extent that generalized bleeding occurs

Disseminated Intravascular Coagulation

Pathophysiology Excessive clotting can be precipitated through intrinsic or extrinsic coagulation pathways

Disseminated Intravascular Coagulation

Clinical Manifestation Bleeding from mucous membranes venipuncture sites and GI and urinary tracts. Organ dysfunction
Renal failure

Hemorrhages

Disseminated Intravascular Coagulation

Nursing Intervention Monitor and manage potential complications
Target potiential sites of end-organ damage
Kidneys Lungs Brain Skin

Hemophilia
X-linked genetic disorder A deficiency of coagulation factors HEMOPHILIA A Deficiency of Factor VIII HEMOPHILIA B Christmas Disease Deficiency of factor IX

Hemophilia
Assessment Hemorrhage Bleeding into joints
Knees Elbows Ankles Shoulders Wrist Hips

Ankylosis (fixation) of the joint

Hemophilia
Nursing Intervention Assist in coping Teach about activity restrictions Avoid any agents that interfere with platelet aggregation Dental hygiene is important Nasal packing should be avoided All injections should be avoided