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Medical- Surgical Nursing

Hematologic Disorders

HEMATOLOGIC DISORDERS
I. Disorders of the RBC A. Anemias A.1 Hypoproliferative - Caused by decreased RBC production. This type of anemia can result from the inability of the bone marrow to properly produce RBCs or the failure of the body to make or absorb substances needed for PBC production A.1.1 Iron Deficiency Anemia - Results when the intake of dietary iron is inadequate for hemoglobin synthesis. - Microcytic, hypochromic

PATHOPHYSIOLOGY

ASSESSMENT FINDINGS

INTERVENTIONS

MEDICATIONS

Blood loss, poor intestinal absorption, inadequate diet iron stores become depleted RBCs become microcytic manifestation s of anemia

y Decreased levels of hemoglobin and MCV, which measures the size of RBC y Hematocrit and RBC levels are also low y Vinson- Plummers Syndrome y Stomatitis y Dysphagia y Atrophic glossitis (small, sore tongue) y Cheilosis- cracks at the side of the lips y Koilonychia- spoon- shaped or concave finger nails y Pica- craving of non-edible substances. y Tinnitus- ringing of the ear y If HGB= 7.5g/dL or below- increased PR, chest pain, SOB, CHF

Medical Management 1. Colonoscopy, endoscopy or x-ray examination of GIT to detect ulcerations, gastritis, polyps or cancer 2. Oxygen therapy 3. Blood transfusion as needed Nursing interventions 1. Preventive education 2. Promote healthy diet. Iron-rich foods like organ meat, lean meat, egg yolk, beans, GLV, raisins, dried fruits 3. Promote rest 4. Provide good skin and oral care.

y Oral iron preparations y Ferrous sulfate y Ferrous gluconate y Ferrous fumarate y IV or IM dextran

A.1.2 Vit B12 Deficiency Anemia - Caused by faulty absorption of Vitamin B12 in the GIT. May be caused by Chrons disease, gastric surgery, autoimmune gastric mucosal atrophy Decreased intrinsic factor production - Pernicious Anemia - Megaloblastic anemia - Macrocytic, Hyperchromic A.1.3 Folic Acid Deficiency Anemia - May be caused by inadequate dietary intake of folic acid, malabsorption, chronic alcoholism and drugs

Decreased production of INTRINSIC FACTOR Decreased absorption of Vitamin B12 Decreased DNA synthesis in maturing RBC (megaloblastic cells) Decreased RBC production

y Signs of anemia y Neurologic manifestations such as paresthesia (numbness and tingling in the feet and lower legs), uncoordinated balance, poor sense of position y Jaundice y Beefy red tongue y Mild diarrhea

Collaborative Management 1. Encourage increased intake of foods rich in vitamin B12 2. Blood transfusion as needed 3. Hematologic and GI evaluation every 6 months 4. Schilling test

y y y y

Monthly vitamin B12 IM for life Folic acid Ferrous sulfate Diluted HCl

Decreased availability of folic acid Impaired DNA synthesis in maturing RBC (megaloblastic cells) Decreased RBC production

y y y y

Cracked lips, sore tongue Decreased RBC, HGB, HCT Increased MCV and MCHC Absence of neurologic problems unlike in pernicious anemia

Collaborative Management 1. Promote healthy diet. Encourage increased intake of GLV, fruits and meat 2. Tell patients to avoid over- cooking of vegetables

y Folic acid per orem

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- Megaloblastic anemia - Macrocytic, Hyperchromic

A.1.4 Aplastic Anemia -Deficiency of the circulating RBC because of the failure of the bone marrow to produce these cells. - Can be caused by a decrease in or damage to marrow stem cells, damage to the microenvironment within the marrow, and replacement of the marrow with fat - Results in bone marrow aplasia, markedly reduced hematopoeisis - nuetropenia and thrombocytopenia are also seen

- Can be congenital or acquired, but most cases are idiopathic - May also be triggered by medications, chemicals, or radiation damage.

y Bone Marrow Aspiration: extremely hypoplastic or even aplastic (very few to no cells) marrow replaced with fat. y Pancytopenia y Anemia y Infection y Bleeding

Medical Management 1. Bone Marrow Transplantation (BMT) 2. Peripheral Blood Stem Cell Transplantation (PBSCT) 3. Immunosuppressive therapy 4. Blood transfusion as needed Nursing Intervention 1. Assess carefully for signs of tissue hypoxia, infection and bleeding 2. Promote rest 3. Minimize risk for infection 4. Minimize risk for bleeding

y y y y y y

Corticosteroids Clyclosporine Cyclophosphamide Antithymocyte globulin Antilymphovyte globulin Azathioprine

A.2 Bleeding - RBC loss due to bleeding from GIT, menorrhagia (excessive menstrual flow), epistaxis (nose bleed), or wound

Bleeding Blood loss Excessive RBC loss Manifestations of anemia

y y y y y y y

Pallor Weakness Anorexia Weight loss Headache/ Dizziness Tachycardia/ Palpitations Syncope

Collaborative Management 1. Treat the cause of bleeding 2. Hold drugs that may further cause bleeding 3. Blood transfusion as needed 4. Cryoprecipitate or platelet transfusion

y Erythropoetin

A.3 Hemolytic A.3.1 Sickle Cell Anemia - a severe hemolytic anemia that results from inheritance of the sickle cell hemoglobin gene

Low oxygen tension p Crystal like formation of RBCs p erythrocyte containing HbS loses its round, pliable, biconcave disk shape p adhere to each other p decreased blood flow

y y y y y y y y y

Pain Swelling Fever Anemia Jaundice Expansion of bone marrows Enlarged heart Dysrythmias Heart failure

Medical Management 1. Pharmacologic Therapy 2. Transfusion Therapy 3. Supportive Therapy Nursing Interventions 1. Manage pain 2. Monitor for signs and symptoms of infection 3. Promote coping 4. minimizing deficient knowledge 5. Monitoring and managing potential complications

y Analgesics y Antibiotics

B. Polycythemias B.1 Polycytemia vera - Hyperplasia of the bone marrow - compensatory response to chronic hypoxia Hypoxia increased erythropoietin production stimulation of the bone marrow Increased RBC production y y y y y y y increased RBC, WBC, platelet Ruddy complexion Headache Dizziness Fatigue Blurred vision Hepatosplenomegaly Collaborative Management 1. Increase fluid intake 2. Monitor for signs and symptoms of bleeding and thromboembolism 3. Therapeutic phlebotomy 4. Chemotherapy 5. Radiation therapy y Analgesics y Antihistamine

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II.

Disorders of the WBC A. Leukopenia and Nuetropenia y A condition in which there are fewer than normal WBCs.

y This may be due to a decrease in production or an increase in destruction. Patients with this condition are at greater risk for infections.

y There are no definite symptoms until the patient becomes infected y CBC can reveal neutropenia before the onset of infection

Medical Management 1. If neutropenia is medication induced, the offending agent must be stopped 2. Bone marrow recovery treatment 3. Withholding or reducing the dose of chemotherapy or radiation may be required 4. Blood, urine and sputum cultures Nursing Interventions 1. Assess for the severity of the neutropenia 2. Preventing and managing infections 3. Early detection of infection must be done

y Granulocyte/ Macrophage stimulating factor y Corticosteroids

B. Luekocytosis and the Leukemias B.1 Acute Lymphocytic Leukemia - results from an uncontrolled proliferation of immature cells (lymphoblast) derived from the lymphoid stem cell y Immature lymphocytes proliferate crowd the normal development of myeloid cells normal hematopoeisis is inhibited Decreased numbers of leukocytes, erythrocytes and thrombocytes y Increased number of lymphocytes y Decreased numbers of leukocytes, erythrocytes and thrombocytes y High proportion of immature cells y Pain y Enlarged liver or spleen y Bone pain y Headache y vomiting Medical Management 1. Goal: complete remission 2. Prophylaxis with cranial irradiation and intrathecal chemotherapy 3. Bone Marrow transplantation (BMT) 4. Peripheral Blood Stem Cell Transplantation (PBSCT) y Wide variety of chemotherapeutic agents

B.2 Chronic Lymphocytic Leukemia

y Derives From a malignant clone of B lymphocytes

y Most leukemia cells are fully mature y Increased lymphocyte count (lymphocytosis) is always present y Lymphadenopathy y Pain y Splenomegaly y B symptoms y Fever y Drenching sweating y Unintentional weight loss

Medical Management 1. In early stages, CLL does not require any treatment 2. When symptoms become severe or progress to later stages, chemotherapy with corticosteroids and chlorambucil is often used.

y y y y

Chlorambucil Corticosteroids Fludarabine Monoclonal Antibodies

B.3 Acute Myeloid Leukemia - Results from a defect in the hematopoietic stem cell that differentiates into myeloid cells: monocytes, granulocytes, erythrocytes and platelets

y Defect in the hematopoietic stem cell that differentiates into myeloid cells will result into pancytopenia

y Insufficient production of normal blood cells y CBC results may show decrease in erythrocytes and thrombocytes; although leukocyte count can be low, normal or high y Bone marrow analysis may show

Medical Management 1. Goal: achieve complete remission in which there is no detectable evidence of residual leukemia remaining in the bone marrow 2. Aggressive administration of chemotherapeutic agents

y y y y y y

Cytarabine Daunorubicin Mitoxantrone Idarubicin G- CSF GM- CSF

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increased numbers of blast cells y Fever and infection that results from neutropenia y Bleeding tendencies from thrombocytopenia y Enlarged liver and spleen y Hyperplasia of gums y Bone pain B.4 Chronic Myeloid Leukemia - Arises from a mutation in the myeloid stem cell. Normal myeloid cells continue to be produced, but there is a preference for immature (blast) forms.

3. Bone Marrow transplantation (BMT) 4. Peripheral Blood Stem Cell Transplantation (PBSCT)

Mutation in the myeloid stem cell p overproduction in blast cells p expansion of marrow into the cavities of long bones p formation of cells in the liver and spleen p enlargement of these organs

y WBC count commonly exceeds 3 100,000/mm y Shortness of breath y Decreased capillary perfusion to the lungs and brain from leukostasis y Enlarged and tender spleen y Enlargement of the liver y Malaise, anorexia and weight loss

Medical Management 1. Goal: Less aggressive therapeutic approach that focuses on reducing the WBC count to normal level, but does not alter cytogenetic changes 2. Correction of the chromosomal abnormality (conversion of the malignant stem cell population back to normal) 3. Chemotherapy 4. Bone Marrow transplantation (BMT) 5. Peripheral Blood Stem Cell Transplantation (PBSCT)

y y y y

Imatinib Interferon alpha Busulfan Hydroxyurea

Nursing Interventions (for all types of Leukemia) 1. Preventing and managing infection and bleeding 2. Provide good skin and oral care 3. Improving nutritional intake 4. Easing pain and discomfort 5. Decreasing fatigue and deconditioning 6. Maintaining fluid and electrolyte imbalance 7. Improving self- care 8. Managing anxiety and grief 9. Encouraging spiritual well- being 10. Monitoring and managing potential complications III. The Lymphomas A. Hodgkins Disease - neoplastic disorder affecting the lymphnodes Reed-Sternberg cells p Initiates in single node p contiguous extension along the lymphatic system y y y y y y y y y y Reed- Sternberg cells Fever, chills, malaise Night sweats Weight loss Pruritus Edema Abdominal pain Splenomegaly Hepatomegaly Cough and dyspnea Medical Management 1. Radiation therapy 2. Chemotherapy 3. Bone Marrow Transplantation Nursing Interventions 1. Prevention of infection 2. Small frequent feeding 3. Encourage patient to avoid alcohol, tobacco, spices, and extreme food temperatures y y y y y y y y Mustargen Oncovin Procarbazine Prednisone Adriamycin Bleomycin Vinblastine Dacarbazine

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B. Non- Hodgkins Lymphomas (NHL) y Lymphosarcoma or Burkitts sarcoma

Neoplastic growth of lymphoid tissue p spread of these malignant lymphoid cells

y Lymphadenopathy y B symptoms (recurrent fever, drenching night sweats, unintentional weight loss of 10% or more)

Medical Management 1. Radiation therapy 2. Chemotherapy Nursing Interventions 1. Prevention of infection

y y y y y

Cytoxan Adriamycin Oncovin Prednisone Blenoxane

C. Multiple Myeloma y Proliferation of abnormal plasma cells in the bone marrow

Malignant plasma cells produce an increased amount of a specific immunoglobulin that is nonfunctional They also stimulate the creation of new blood vessels to enhance the growth of these clusters of plasma cells

y y y y y y y y

Elevated monoclonal protein spike in the serum or urine- Bence- Jones Protein Bone lesions in x-ray Severe bone pain Fatigue and weakness Hyperviscosity of the blood Bleeding Proteinuria Hypercalcemia and hyperuricemia

Collaborative Management 1. Chemotherapy 2. Plasmapheresis

y y y y

Cytoxan Alkeran Corticosteroids Alpha interferon

IV.

The Bleeding Disorders

A. Thrombocythemia A.1 Primary - Also called Essential Thrombocythemia - stem cell disorder within the marrow - A marked increase in platelet production occurs, with platelet count consistently 3 greater than 600,000/mm

Exact cause is unknown

y y y

Asymptomatic CBC reveals elevated levels of platelet Bane Marrow (by aspiration or biopsy) shows a marked increase in megakaryocytes (platelet precursors)

Medical Management 1. Platelet pheresis 2. Lower platelet production Nursing Interventions 1. Instruct the patient about the risk of hemorrhage and thrombosis 2. Inform patient about signs and symptoms of thrombosis, particularly the neurologic manifestations, such as visual changes, numbness, tingling, and weakness 3. Reduce risk factors for bleeding and thrombosis

A.2 Secondary - Reactive thrombocytosis B. Thrombocytopenia B.1 Primary - Decreased production of platelet within the bone marrow, increased destruction of platelets, or increased consumption of y Bone marrow aspiration may show increased megakaryocytes and normal or even increased platelet production as the body attempt to compensate for the

Collaborative Management 1. Treatment is aimed at the underlying disorder

Medical Management 1. Treatment of the underlying disease 2. Platelet transfusion

y Steroids y IV immunoglobulin y Avoid use of ASA and NSAIDs

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platelets y

decreased platelet in the circulation Splenomegaly

Nursing Intervention 1. Prevent and control bleeding 2. Prevent complications 3. Monitor for signs of bleeding

B.2 ITP - platelets clump together abnormally in the capillaries and too few platelets remain in the circulation

y y

Autoimmune disorder Sulfa drugs, pregnancy and SLE may iduce ITP

y y y y

Isolated decrease in platelet, less tham 3 20,00/ mm They may also have an increase in megakaryocytes within the marrow Easy bruising Hemoptysis

Medical Management 1. Goal: safe platelet count 2. Immunosuppressive agents 3. Platelet transfusion 4. Splenectomy Nursing Interventions 1. Bleeding precautions

y Corticosteroids y IV immunoglobulin y Danazol y Immuran y Oncovin y Velban

C. Platelet Defects y Platelet level may be normal but nonfunctional

y y y

Prolonged bleeding time Increased PT and PTT Ecchymoses

Collaborative Managemant 1. Platelet transfusion 2. Bleeding precautions 3. Control and management of bleeding

y Aminocaproic acid

D. Hemophilia - Inherited bleeding disorder - Absence of a specific clotting factor, either factor VIII or factor IX

Autosomal recessive disorder wherein there is a lack of a clotting factor that is essential for the clotting cascade to pursue

y y y

Bleeding Hematomas Joint pain

Collaborative Management 1. Administration of factor VIII or factor IX concentrates 2. Bleeding precautions 3. Instruct the patient to avoid contact sports 4. Avoid medications that may further aggravate the patients condition

y Aminocaproic acid y Desmopressin

E. Von Willebrands Disease y It is an inherited, autosomal dominant, disorder characterized by a decrease in the Von Willebrand factor and prolonged bleeding time V. The Coagulation Disorders A. Vitamin K Deficiency y Vitamin K is essential in the formation of some clotting factors. Absence of this vitamin will interfere the normal clotting cascade

y Epistaxis y Normal platelet count but slightly prolonged PTT y Heavy menses

Collaborative Management 1. Factor VII replacement via infusions of cryoprecipitate 2. Bleeding precautions

y Aminocaproic acid y Desmopressin

y Prolonged PT y Prolonged bleeding time

Collaborative Management 1. Administration of Vitamin K 2. Bleeding precaution

y Vitamin K

B. Dissemintaed Intravascular Coaggulation - Acquired thrombolytic and hemorrhagic syndrome

Widespread bleeding clotting in small vessels of the body with consumption of clotting factors and platelets, so that bleeding and thrombosis occurs simultaneously

y y

Signs of abnormal clotting y Coolness and mottling of extremities y Acrocyanosis y Hematuria y Altered mental status y Pain Signs of abnormal bleeding ]oozing, bleeding from sites of

Collaborative Management 1. Bleeding precautions 2. Replacement therapy (fresh frozen plasma, platelet transfusion, cryoprecipitate) 3. Fluid replacement 4. Oxygen therapy 5. Maintenance of BP

y Heparin

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y y y y

procedures Internal bleeding Low platelet count Prolonged PT, PTT Decreased fibrinogen level

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