The Child with Hematologic or Immunologic Dysfunction
Chapter 26
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Assessment of Hematologic Function
• Complete blood count
• History and assessment findings • Child’s energy and activity level
• Growth patterns
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Anemia
• The most common hematologic disorder of childhood
• Decrease in number of RBCs and/or hemoglobin concentration below normal
• Decreased oxygen-carrying capacity of blood
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Classification of Anemias
• Etiology and physiology
– RBC and/or Hgb depletion • Morphology
– Characteristic changes in RBC size, shape, and/or color
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Consequences of Anemia
• Decrease in oxygen-carrying capacity of blood and decreased amount of oxygen available to tissues
• When anemia develops slowly, child adapts
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Effects of Anemia on Circulatory System
• Hemodilution
• Decreased peripheral resistance • Increased cardiac circulation and turbulence
– May have murmur
– May lead to cardiac failure • Cyanosis • Growth retardation
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Diagnostic Evaluation
• CBC
– Decreased RBCs – Decreased Hbg and Hct
• Other tests for particular type of anemia
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Therapeutic Management
• Treat underlying cause
– Transfusion after hemorrhage if needed – Nutritional intervention for deficiency anemias
• Supportive care
– IV fluids to replace intravascular volume – Oxygen
– Bed rest
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Nursing Considerations
• Prepare child and family for laboratory tests
• Decrease oxygen demands • Prevent complications • Support family
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Iron Deficiency Anemia
• Caused by inadequate supply of dietary iron
• Generally preventable – Iron-fortified cereals and formulas for infants – Special needs of premature infants – Adolescents at risk due to rapid growth and poor eating habits
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Iron Deficiency Anemia (cont’d)
• Pathophysiology
• Therapeutic management • Prognosis
• Nursing considerations
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Sickle Cell Anemia
• A hereditary hemoglobinopathy
• Ethnicity – Occurs primarily in blacks
• • •
Occurrence 1 in 375 infants born in US 1 in 12 have sickle cell trait Occasionally also in persons of Mediterranean descent
•
Also seen in South American, Arabian, and East Indian descent
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Etiology of Sickle Cell
• In areas of world where malaria is common, individuals with sickle cell trait tend to have survival advantage over those without trait
• Autosomal recessive disorder – 1 in 12 blacks are carriers (have sickle cell trait) – (If both parents have trait, each offspring will have 1 in 4 likelihood of having disease)
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Pathophysiology
• Partial or complete replacement of normal Hgb with abnormal hemoglobin S (Hgb S)
• Hemoglobin in the RBCs takes on an elongated ―sickle‖ shape
• Sickled cells are rigid and obstruct capillary blood flow
• Microscopic obstructions lead to engorgement and tissue ischemia • Hypoxia occurs and causes sickling
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Sickled Hemoglobin
• (Note to instructor: Image available in Electronic Image Collection)
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Pathophysiology
• Large tissue infarctions occur
• Damaged tissues in organs; impaired function – Splenic sequestration
• •
May require splenectomy at early age Results in immunity
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Prognosis
• No cure (except possibly bone marrow transplants)
• Supportive care/prevent sickling episodes • Frequent bacterial infections may occur due to immunocompromise • Bacterial infection is leading cause of death in young children with sickle cell disease • Strokes in 5%-10% of children with disease – Result in neurodevelopmental delay, mental retardation
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Systems Affected
• (Note to instructor: Image available in Electronic Image Collection)
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Sickle Cell Crisis
• Precipitating factors
– Anything that increases body’s need for oxygen or alters transport of oxygen
– Trauma – Infection, fever
– Physical and emotional stress
– Increased blood viscosity due to dehydration – Hypoxia
•
From high altitude, poorly pressurized airplanes, hypoventilation,vasoconstriction due to hypothermia
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Sickle Cell Crisis (cont’d)
• Acute exacerbations that vary in severity and frequency
• Types – Vaso-occlusive [VOC] thrombotic
•
• •
Most common type of crisis—very painful
Stasis of blood with clumping of cells in microcirculation→ischemia→infarction Signs: fever, pain, tissue engorgement
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Sickle Cell Crisis (cont’d)
• Types (cont’d)
– Splenic sequestration
• • •
Life threatening—death can occur within hours Blood pools in the spleen Signs
– Profound
anemia, hypovolemia, and shock
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Sickle Cell Crisis (cont’d)
• Types (cont’d)
– Aplastic crises
• • •
Diminished production and increased destruction of RBCs Triggered by viral infection or depletion of folic acid Signs include profound anemia, pallor
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Diagnosis of Sickle Cell
• Cord blood in newborns
• Newborn screening done in 43 states • Genetic testing to identify carriers and children who have disease • Sickle-turbidity test – Quick screening purposes in children >6 mos of age
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Medical Management
• Aggressive treatment of infection
• Possible prophylactic antibiotics from 2 mos-5 yrs
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Medical Management (cont’d)
• Monitor reticulocyte count regularly to evaluate bone marrow function
• Blood transfusion, if given early in crisis, may reduce ischemia
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Medical Management (cont’d)
• Frequent transfusion decreases hemosiderosis (iron in tissues)
– Treat with iron-chelation such as feroxamine + vitamin C to promote iron excretion • Rx—Hydronurea (Cytotoxic) decreases production of abnormal blood cells and decreases pain
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Nursing Management
• Monitor child’s growth—watch for failure to thrive
• Careful multi-system assessment • Assess pain • Observe for presence of inflammation or possible infection • Carefully monitor for signs of shock
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Psychosocial Needs
• Coping mechanisms
• Support with genetic counseling • Financial needs
• Caregiver role strain
• Living with chronic illness in the family
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Thalassemia
• Inherited blood disorders of hemoglobin synthesis
• Classified by Hgb chain affected and by amount of effect
• Autosomal recessive with varying expessivity
– Both parents must be carriers to have offspring with disease
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Pathophysiology
• Anemia results from defective synthesis of Hgb, structurally impaired RBCs, and shortened life of RBCs
• Chronic hypoxia – Headache, irritability, precordial and bone pain, exercise intolerance, anorexia, epistaxis • Detected in infancy or toddlerhood
– Pallor, FTT, hepatosplenomegaly, severe anemia (Hgb <6)
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Thalassemia
• -Thalassemia
– Alpha chains affected – Occurs in Chinese, Thai, African, and Mediterranean people • -Thalassemia – Occurs in Greeks, Italians, and Syrians – is most common and has four forms
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-Thalassemia
• Four types
– Thalassemia minor—asymptomatic silent carrier – Thalassemia trait—mild microcytic anemia
– Thalassemia intermediate—moderate to severe anemia + splenomegaly
– Thalassemia major (―Cooley’s anemia‖) — severe anemia requiring transfusions to survive
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Diagnosis
• By hemoglobin electrophoresis
• RBC changes often seen by 6 weeks of age • Child presents with severe anemia, FTT
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Medical Management
• Blood transfusion to maintain normal Hgb levels
• Side effect—hemosiderosis
•
Treat with iron chelating drugs such as deferoxamine (Desferal)
– Binds
excess iron for excretion by kidney
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Nursing Management
• Observe for complications of transfusion
• Emotional support to family • Encourage genetic counseling
• Parent and patient teaching for self-care
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Prognosis
• Retarded growth
• Delayed or absent secondary sex characteristics • Expect to live well into adulthood with proper clinical management • Bone marrow transplant is potential cure
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Aplastic Anemia
• All formed elements of the blood are simultaneously depressed: ―pancytopenia‖
• Hypoplastic anemia: profound depression of RBCs but normal WBCs and platelets
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Aplastic Anemia (cont’d)
• Etiology
– Primary (congenital) – Secondary (acquired)
• Diagnostic evaluation
• Therapeutic management • Nursing consideration
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Hemophilia
• A group of hereditary bleeding disorders that result from deficiencies of specific clotting factors
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Types of Hemophilia
• Hemophilia A
– ―Classic hemophilia‖ – Deficiency of factor VIII
– Accounts for 80% of cases of hemophilia
– Occurrence: 1 in 5000 males
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Types of Hemophilia (cont’d)
• Hemophilia B
– Also known as Christmas disease – Caused by deficiency of factor IX
– Accounts for 15% of cases of hemophilia
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Etiology of Hemophilia A
• X-linked recessive trait
• Males are affected • Females may be carriers • Degree of bleeding depends on amount of clotting factor and severity of a given injury • Up to ⅓ of cases have no known family history – In these cases disease is caused by a NEW mutation
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Manifestations of Hemophilia
• Bleeding tendencies range from mild to severe
• Symptoms may not occur until 6 mos of age – Mobility leads to injuries from falls and accidents
• Hemarthrosis
– Bleeding into joint spaces of knee, ankle, elbow leading to impaired mobility • Ecchymosis
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Manifestations (cont’d)
• Epistaxis
• Bleeding after procedures – Minor trauma, tooth extraction, minor surgeries – Large subcutaneous and intramuscular hemorrhages may occur – Bleeding into neck, chest, mouth may compromise airway
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Clinical Therapy
• Can be diagnosed through amniocentesis
• Genetic testing of family members to identify carriers • Diagnosis on basis of hx, labs, and exam – *Labs: Low levels of factor VIII or IX, prolonged PTT – *Normal: platelet count, PT, and fibrinogen
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Medical Management
• DDAVP
– IV – Causes 2-4 X increase in factor VIII activity
– Used for mild hemophilia
• Replace missing clotting factors • Transfusions – At home with prompt intervention to reduce complications – Following major or minor hemorrhages
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Prognosis
• Historically, most died by 5 yrs age
• Now mild to moderate hemophilia patients live near normal lives • Gene therapy for future – Infused carrier organisms act on target cells to promote manufacture of deficient clotting factor
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Interventions
• Close supervision and safe environment
• Dental procedures in controlled situation • Shave only with electric razor
• Superficial bleeding—apply pressure for at least 15 minutes + ice to vasoconstrict
• If significant bleeding occurs, transfuse for factor replacement
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Managing Hemarthrosis
• During bleeding episodes, elevate and immobilize joint
• Ice • Analgesics
• ROM after bleeding stops to prevent contractures
• PT
• Avoid obesity to minimize joint stress
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Von Willebrand Disease
• A hereditary bleeding disorder, involving deficiency of Von Willebrand factor, (a plasma protein, and the carrier for factor VIII)
• Von Willebrand factor is necessary for platelet adhesion • Transmitted as autosomal dominant trait • Occurs in males and females • Gene for disease is located on chromosome 12
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Manifestations of Von Willebrand Disease
• Easy bruising
• Epistaxis • Gingival bleeding
• Excessive bleeding with lacerations or surgeries
• Menorrhagia
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Diagnosis Laboratory Findings
• Decreased Von Willebrand factor levels
• Von Willebrand antigen levels • Decreased platelet agglutination
• Prolonged bleeding time
• PTT may be normal or prolonged
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Treatment of Von Willebrand Disease
• Infusion of Von Willebrand’s protein concentrate
• DDAVP infusion prior to surgery or to treat bleeding episode
• Aminocaproic acid to treat bleeding in mucous membranes (in some cases)
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Interventions
• Avoid aspirin or NSAIDs (increased bleeding time and inhibit platelet function)
• Manage bleeding episodes with prompt infusion therapy
• Children with Von Willebrand’s have normal life expectancy if well managed
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Idiopathic Thrombocytopenic Purpura (ITP)
• An acquired hemorrhagic disorder characterized by
– Thrombocytopenia: excessive destruction of platelets
– Purpura: discoloration caused by petechiae beneath the skin
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ITP Forms
• Acute self-limiting
– Often follows URI or other infection • Chronic (>6 months duration)
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ITP
• Diagnostic evaluation
• Therapeutic management • Prognosis
• Nursing considerations
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Disseminated Intravascular Coagulation (DIC)
• Disorder of coagulation that occurs as complication of numerous pathologic processes
– Hypoxia
– Acidosis
– Shock – Endothelial damage
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Pathophysiology of DIC
• Coagulation process is abnormally stimulated
• Excessive amounts of thrombin are generated
• Fibrinolytic mechanisms are activated and cause extensive destruction of clotting factors
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DIC
• Diagnostic evaluation
• Therapeutic management • Nursing considerations
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Epistaxis (Nosebleeding)
• Isolated and transient epistaxis is common in childhood
• Recurrent or severe episodes may indicate underlying disease
– Vascular abnormalities, leukemia, thrombocytopenia, clotting factor deficiency diseases (Von Willebrand disease and hemophilia)
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Nursing Considerations— Epistaxis
• Remain calm, keep child calm
• Have child sit up and lean forward • Pressure to nose
• Further evaluation if bleeding continues
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Neoplastic Disorders
• Leading cause of death from disease in children past infancy
• Almost half of all childhood cancers involve blood or blood-forming organs
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Leukemias
• Most common form of childhood cancer
• 3-4 cases per 100,000 Caucasian children <15 yrs old • More frequent in males >1 yr old • Peak onset between 2 and 6 yrs of age • Survivability
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Classification of Leukemias
• Leukemia: A broad group of malignant disease of bone marrow and lymphatic system
• Complex disease with varying heterogeneity • Classifications are increasingly complex
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Morphology
• Acute lymphoid leukemia (ALL)
• Acute non-lymphoid (myelogenous) leukemia (ANLL or AML) • Stem cell or blast cell leukemia
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Symptoms
• ALL: lymphatic, lymphocytic, lymphoblastic, and lymphoblastoid leukemia
• AML: granulocytic, myelocytic, monocytic, myelogenous, monoblastic, and nonmyeloblastic leukemia
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Chromosome Studies
• Children with Trisomy 21:20X greater risk for developing ALL
• Children with more than 50 chromosomes on the leukemic cells have the best prognosis • Specific translocations of chromosomes on leukemic cells can affect prognosis
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Pathophysiology
• Leukemia is an unrestricted proliferation of immature WBCs in the blood-forming tissues of the body
• Liver and spleen are the most severely affected organs
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Pathophysiology (cont’d)
• Although leukemia is an overproduction of WBCs, often acute form causes low leukocyte count
• Cellular destruction takes place by infiltration and subsequent competition for metabolic elements
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Consequences of Leukemia
• Anemia from decreased RBCs
• Infection from neutropenia • Bleeding tendencies from decreased platelet production • Spleen, liver, and lymph glands show marked infiltration, enlargement, and fibrosis
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Diagnostic Evaluation
• Based on history, physical manifestations
• Peripheral blood smear – Immature leukocytes
– Frequently low blood counts
• LP to evaluate CNS involvement • Bone marrow aspiration or biopsy
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Therapeutic Management
• Chemotherapeutic agents
• Cranial irradiation (in some cases)
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Four Phases of Therapy
• Induction therapy: 4-6 weeks
• CNS prophylactic therapy: intrathecal chemotherapy • Intensification (consolidation) therapy: To eradicate residual leukemic cells and prevent resistant leukemic clones • Maintenance therapy: to preserve remission
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Hematopoietic Stem Cell Transplantation (HSCT)
• Donors may be relatives or non-relatives
• Antigen-matched or mismatched • Peripheral stem cells may be used
• Stem cells from umbilical cord blood
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Risks of HSCT
• Significant risk of morbidity and mortality
• Graft vs. host disease (GVHD) • Overwhelming infection
• Severe organ damage
• Cure after HSCT: up to 60%-70%
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Prognosis
• If relapse after HSCT: dismal prognosis
• Identified factors for determining prognosis – Initial WBC count
– Age at time of diagnosis
– Type of cell involved – Gender – Karyotype analysis
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Nursing Considerations
• Assessment
• Nursing diagnosis • Planning • Implementation – Prepare child and family for procedures – Pain management – Prevent complication of myelosuppression
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Increased Susceptibility to Infection
• At time of diagnosis and relapse
• During immunosuppressive therapy • After prolonged antibiotic therapy that predisposes to the growth of resistant organism
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Infection Control
• Environment
• Hand hygiene • Visitor restriction
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Managing Chemotherapeutic Agents
• ―Vesicants‖—sclerosing agents even in minute amounts
• Interventions for extravasation • Risk for anaphylaxis
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Managing Problems of Drug Toxicity
– Nausea/vomiting
– Anorexia – Mucosal ulceration – Neuropathy – Hemorrhagic cystitis – Alopecia – Mood changes – Moon face
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Nursing Diagnoses
• Risk for injury related to malignant process, treatment
• Risk for fluid volume deficit related to nausea, vomiting • Altered nutrition • Impaired skin integrity • Altered family processes • Fear related to diagnosis, procedures, treatments
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Lymphomas
• Hodgkin disease
– More prevalent in 15-19 yrs of age • Non-Hodgkin lymphoma (NHL)
– More prevalent in children <14 yrs of age
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Hodgkin Disease
• Neoplastic disease originating in lymphoid system
• Often metastasizes to spleen, liver, bone marrow, lungs, and other tissues
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Four Types of Hodgkin Disease
• Lymphocytic predominance
• Nodular sclerosis • Mixed cellularity
• Lymphocytic depletion
• Accurate staging is basis for treatment and prognosis
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Classifications of Hodgkin Disease
• Classification A: asymptomatic
• Classification B: temperature of 38°C or higher for three consecutive days, night sweats, unexplained wt loss of 10% or more over previous 6 mos
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Diagnostics
• Clinical manifestations of Hodgkin disease
• Lymph node biopsy for diagnosis and staging
• Presence of Reed-Sternberg cells is characteristic of Hodgkin disease
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Therapeutic Management
• Radiation
• Chemotherapy (alone or with radiation) • Prognosis
• Nursing considerations
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Non-Hodgkin Lymphoma
• Approximately 60% of pediatric lymphomas are NHL
• Clinical appearance – Disease usually diffuse rather than nodular
– Cell type undifferentiated or poorly differentiated
– Dissemination occurs early, often, and rapidly – Mediastinal involvement and invasion of meninges
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NHL (cont’d)
• Diagnostic evaluation
• Therapeutic management • Prognosis
• Nursing consideration
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Immunologic Deficiency Disorders
• HIV/AIDS
– Epidemiology – Etiology and pathophysiology
– Clinical manifestations
– Diagnostics – Therapeutic management
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Severe Combined Immunodeficiency Disease (SCID)
• Therapeutic management
• Nursing considerations
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Wiskott-Aldrich Syndrome
• Thrombocytopenia
• Eczema • Immunodeficiency of selective functions of B and T lymphocytes • X-linked recessive
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Blood Transfusion Therapy
• Verify identity of recipient and verification of donor’s blood group
• Monitor VS • Use appropriate filter • Use blood within 30 minutes of arrival • Infuse over 4 hours maximum
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Transfusion Reactions
• Hemolytic: the most severe, but rare
• Febrile reactions: fever, chills • Allergic reaction: urticaria, pruritis, laryngeal edema
• Air emboli: may occur when blood is transfused under pressure
• Hypothermia • Electrolyte disturbances: hyperkalemia from massive transfusions or pt with renal problems
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Nursing Responsibilities
• Identify donor and recipient blood types and groups before transfusing
• Transfuse slowly for first 15-20 minutes • Observe carefully for patient response • Stop transfusion immediately if signs/symptoms of transfusion reaction; notify practitioner
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Delayed Reactions to Blood Transfusion
• Transmission of infection
– Hepatitis, HIV, malaria, syphilis, other – Blood banks test vigorously and discard units of infected blood • Delayed hemolytic reaction – Destruction of RBCs and fever 5-10 days after transfusion – Observe for posttransfusion anemia
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Family Concerns
• Difficult decision for HTSC transplant
– Child faces death without transplant – Preparing child for transplant places child at great risk • No ―rescue‖ procedure if complications follow HTSC transplants • Nursing considerations
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Apheresis
• Definition: removal of blood from an individual; separation of the blood into its components
• Nursing considerations
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Hematopoietic Stem Cell Transplantation (HSCT)
• Used to establish healthy cells in both malignant and nonmalignant disease
• Ablative therapy: high-dose combination chemo (with or without radiation) to eradicate unhealthy cells and suppress immune system to prevent rejection of transplanted marrow
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HSCT (cont’d)
• Stem cells harvested from bone marrow, peripheral blood, or umbilical vein of placenta
• Stem cells given to patient by IV transfusion • Newly transfused stem cells repopulate ablative bone marrow
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Allogeneic Bone Marrow Transplant (BMT)
• Involves matching histocompatible donor with recipient
• Limited by presence of suitable marrow donor
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Umbilical Cord Blood Stem Cell Transplantation
• Rich source of hematopoietc stem cells for use in children with cancers
• Stem cells found with high frequency in circulation of newborns
• Benefit of umbilical cord blood is blood’s relative immunodeficiency at birth, allowing for partially matched unrelated cord blood transplants to be successful
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Autologous BMT
• Uses patient’s own marrow collected from disease-free tissue, frozen and sometimes treated to remove malignant cells • Has been used to treat neuroblastoma, Hodgkin disease, NHL, Wilms' tumor, rhabdomyosarcoma, and Ewing sarcoma
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Peripheral Stem Cell Transplants (PSCT)
• A type of autologous transplant
• Different type of collection from patient • Stimulate production of high number of stem cells then collect stem cells by an ―apheresis‖ machine • Stem cells separated from whole blood • Remaining blood cells and plasma returned to patient after apheresis
• Stem cells frozen for later transfusion to patient
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