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The Child with Hematologicor Immunologic Dysfunction
ERNIE B. GUEVARRA, M.D.
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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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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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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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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 non-myeloblastic 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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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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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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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