Exam 12: Peds Hematologic Disorders
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| Blood Components | Plasma. Erythrocytes. Leukocytes. Thrombocytes.
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| Eythrocytes | transport oxygen and carbon dioxide.
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| Leukocytes | defense against infections
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| Granular (Leukocytes) | basophils, neutrophils, eosinophils
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| Nongranular (Leukocytes) | lymphocytes, monocytes
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| Thrombocytes (platelets) | along with plasma responsible for blood coagulation.
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| marrow of the long bones (tibia and femur) | During childhood RBCs are formed in
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| hematopoesis | By adolescence ___ takes place in every available space of the marrow of the ribs, sternum, vertebrae, pelvis, skull, clavicle and scapula.
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| Erythropoietin | (produced in the liver of the fetus and the kidneys after birth) regulates the production of blood.
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| Lymphatic system | drains regions of the body to lymph nodes, where organisms are destroyed and antibody production is stimulated. Lymphocytes-Lymphatic Vessels- Lymph Nodes- Spleen- Tonsils, adenoids, and thymus gland.
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| Lymphocytes | Produced in the lymphoid tissue.
Mainly found in lymph tissues.
Fight infection.
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| Spleen | Largest organ in the lymphatic system.
Main function is to bring blood into contact with lymphocytes.
Enlarges during infection, hemolytic anemias and liver malfunction.
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| Anemia | condition in which there is a reduction in the number of RBCs or the amount of hemoglobin is below normal values for age, or both. is the most common hematologic disorder of infancy and childhood.
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| Anemia Fact | Severe anemia decreases peripheral resistance which increases the amount of blood returned to the heart. This produces a murmur. Because the workload of the heart is increased, exercise, infection or emotional stress may cause cardiac failure.
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| Anemia Diagnostic Evaluation | Alterations in the CBC such as decreased RBCs and decreased HgB and Hct may be the first indication of anemia.
In older children an indication may be fatigue, lack of energy and pallor if the condition is severe.
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| Anemia Therapeutic Management | Supportive medical treatment may include:
(a)Supplemental oxygen therapy.
(b)Bed rest.
(c)IV fluids.
Providers seek to reverse the anemia by treating the underlying cause.
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| Anemia Signs of Note | Tachycardia and palpitations.
Tachypnea.
Dyspnea and shortness of breath.
Hyperpnea or breathlessness.
Dizziness or lightheadedness.
Diaphoresis.
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| Anemia Complication Prevention | Provide oxygen as ordered and needed.
Prevent exposure to infectious agents and people.
Observe for signs of infection.
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| Iron-Deficiency Anemia | Inadequate supply of dietary iron is the most prevalent nutritional disorder in the US and the most common mineral disturbance.
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| Iron-Deficiency Anemia: Pathophysiology | Iron deficiency anemia may be cause by a number of factors that decrease the supply of iron, impair its absorption, increase the body’s need for iron, or affect the synthesis of Hgb.
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| Iron-Deficiency Anemia: Therapeutic Management | with dietary counseling and the administration of iron supplements (Ferrous Sulfate). Iron-fortified infant formula and cereal is used for supplementing formula-fed infants. Transfusions are indicated for severe anemia and in cases of infection.
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| Sickle-Cell Disease | an inherited defect in which normal adult hemoglobin (hemoglobin A) is partly or completely replaced by abnormal sickle hemoglobin.
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| sickle cell trait | When the child inherits both HbA and HbS, they are said to have
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| Sickle-Cell Disease; Pathophysiology | The clinical features are primarily the result of obstruction caused by the sickled RBCs and increased RBC destruction. Infarcts generally develop in the spleen, brain, heart, lungs, GI tract, kidneys and bones causing acute pain in the affected area.
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| Sickle cell crisis | a period of exacerbation of symptoms and is painful and can be fatal.
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| Vaso-occlusive crisis | painful; characterized by distal ischemia and pain.
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| Splenic sequestration | pooling of large amounts of blood in liver and spleen with the spleen becoming massively enlarged.
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| Aplastic crisis | bone marrow stops producing RBCs (can be precipitated by viral infection).
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| Hyperhemolytic | an accelerated rate of RBC destruction characterized by anemia, jaundice, and reticulocytosis.
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| Acute chest syndrome | serious complication very similar clinically to pneumonia. The signs and symptoms include:
Chest pain.
Fever.
Cough.
Tachypnea.
Wheezing.
Hypoxia.
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| sickledex | used for screening once a person is in the hospital. If the test is positive, further testing needs to be done to distinguish whether the child has the trait or the disease.
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| Hemoglobin electrophoresis | "fingerprinting" of the protein is an accurate, rapid and specific test for detecting the different forms of the disease.
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| Sickle Cell Anemia: Medical Management | Rest to minimize energy expenditure and oxygen use. Hydration (oral and IV). Electrolyte replacement. Analgesics for the severe pain. Blood replacement to treat anemia and hydration to reduce the viscosity of the sickled blood. Antibiotics.
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| Beta-Thalassemia | refers to a variety of inherited blood disorders characterized by deficiencies in the rate of production of specific globin chains in Hgb.
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| Four forms of Beta-Thalassemia | Thalassemia Minor. Thalassemia trait. Thalassemia Intermedia. Thalassemia Major.
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| Thalassemia Minor | asymptomatic form.
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| Thalassemia Trait | Produces a mild microcytic anemia.
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| Thalassemia Intermedia | Manifested as Splenomegaly and moderate to severe anemia.
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| Thalassemia Major | AKA "Cooley Anemia". Results in severe anemia that can lead to cardiac failure and death in early childhood without treatment.
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| Beta-Thalassemia: Pathophysiology | there is a partial or complete deficiency in the synthesis of the hemoglobin molecule.
Consequently there is a compensatory increase in different chains which increases the production of defective hemoglobin.
This damages the RBC causing severe anemia
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| Beta-Thalassemia: Diagnostic Evaluation | Hematologic studies reveal the characteristic changes in RBCs.
Low Hgb and Hct levels are seen when the anemia is severe.
Hgb electrophoresis confirms the diagnosis and x-rays reveal bone involvement.
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| Aplastic Anemia (AA) | a bone marrow failure condition in which the formed elements of blood are simultaneously depressed. primary (congenital) or secondary (acquired).
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| Several factors contribute to the development of acquired aplastic anemia | Infection with human parvovirus, hepatitis or overwhelming infection.
Irradiation.
Drugs such as chemotherapy agents and several antibiotics.
Exposure to household or industrial chemicals.
Idiopathic, where there is no identifiable precipitating cause
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| Aplastic Anemia (AA): Diagnostic Evaluation | Anemia.
Leukopenia.
Decreased platelet count.
Definitive diagnosis is made from bone marrow aspiration.
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| Aplastic Anemia (AA): Medical Management | Antilymphocyte globulin (ALG) or antithymocyte globulin (ATG) is the principal drug treatment used for AA.
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| Hemophilia | a disorder where the blood does not clot normally and even the slightest injury can cause severe bleeding.
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| Common forms of Hemophilia | Factor VIII deficiency (hemophilia A, or classic hemophilia); accounts for 80 – 85 % of all hemophilia cases.
Factor IX deficiency (hemophilia B, or Christmas disease).
Von Willebrand disease; affects both males and females.
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| Common signs and symptoms of hemophilia | Prolonged bleeding anywhere from or in the body.
Hemorrhage.
Subcutaneous and intramuscular hemorrhages.
Hemarthrosis especially the knees, ankles and elbows.
Hematomas.
Spontaneous hematuria.
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| Hemophilia: Therapeutic Management | Corticosteroids are used for hematuria, acute hemarthrosis and chronic synovitis.
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| Hemophilia Considerations | The nurse should maintain a high level of suspicion when a child with hemophilia demonstrates signs such as a headache, slurred speech, loss of consciousness and black tarry stools.
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| Thrombocytopenic Purpura (ITP) | an acquired hemorrhagic disorder that occurs in childhood. Unknown cause; it is the most frequently occurring thrombocytopenia of childhood. Two forms: acute, self-limiting or chronic (> 6 mos duration).
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| ITP is characterized by | Thrombocytopenia excessive destruction of platelets.
Purpura, a discoloration caused by petechiae beneath the skin.
Normal bone marrow with normal or increased number of immature platelets and eosinophils.
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| ITP Diagnostic Evaluation | Easy bruising, petechiae, ecchymoses.
Bleeding from mucous membranes, epistaxis, bleeding gums and internal hemorrhage.
Hematomas over lower extremities.
Testing is commonly done to rule out other disorders.
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| Leukemia. | : a malignant disease of the blood-forming organs of the body that results in an uncontrolled growth of immature white blood cells (blasts or stem cells).
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| Leukemia’s are classified according to the type of WBC affected | Acute Lymphocytic (ALL): most common.
Acute Non-Lymphocytic (myelogenous), (ANLL, AML).
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| Leukemia Manifestations: Phase 1 (Initial Phase) | Low-grade fever.
Pallor.
Tendency to bruise.
Leg and joint pain, pathologic fractures.
Listlessness.
Abdominal pain.
Enlargement of lymph nodes.
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| Leukemia Manifestations: Phase 2 (Disease Progresses Phase) | Hepatomegaly and splenomegaly.
Anemia despite transfusions.
Lemon-yellow skin discoloration.
Petechiae and purpura.
Ulcerations of mucous membranes of the mouth and anal regions.
Anorexia, N/V, weight loss.
Dyspnea.
Enlargement of kidneys and testi
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| Leukemia Diagnosis | History and symptoms.
Blood tests: CBC, differential.
Bone marrow aspiration.
Spinal tap determines CNS involvement.
Kidney and liver function tests.
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| Hodgkin's Disease | a malignancy of the lymph system primarily affecting the lymph nodes. Rare – five out of every 1 million children develop Hodgkin’s. Presence of giant multinucleated cells called Reed-Sternberg cells.
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| Hodgkin's Disease: Manifestations | Painless lump along neck.
Unexplained low-grade fever, night sweats.
Anorexia, unexplained weight loss, general Malaise.
Rash and pruritus.
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| Hodgkin's Disease: Diagnosis | X-ray films.
Body scan.
Lymphangiogram.
Node biopsy.
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| Hodgkin's Disease: Stage I | restricted to single group or localized in a group of lymph nodes; patient is asymptomatic.
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| Hodgkin's Disease: Stage II | two or more lymph nodes in the same area or same side of diaphragm.
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| Hodgkin's Disease: Stage III | involves lymph nodes on both sides of diaphragm; involves adjacent organ or spleen.
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| Hodgkin's Disease: Stage IV | diffuse disease; least favorable prognosis.
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| Hodgkin's Disease: Treatment | -Radiation therapy.
-Chemotherapy:
(a)MOPP regimen (Mustargen (mechlorethamine), Oncovin (vancristine), Prednisone, and Procarbazine.
(b)ABVD regimen (adriamycin, bleomycin, vinblastine, dacarbazine).
-Splenectomy.
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