CARE OF CHILDREN WITH HEMATOLOGICAL AND IMMUNOLOGICAL DISORDERS

INTRODUCTION
Blood is the life-maintaining fluid that circulates through the body's heart, arteries, veins, and capillaries. Carries away waste matter and carbon dioxide, and brings nourishment, electrolytes, hormones, vitamins, antibodies, heat, and oxygen to the tissues. Functions of blood are many and complex many disorders that require clinical care Conditions include benign (non-cancerous) disorders / cancers that occur in blood.

Functions of the Hematologic system and its formed elements

a. b. c. It is responsible for oxygenation of cells, removal of end products of metabolism, immune protection, clotting and heat regulation. Functions of erythrocytes Transports Hgb which provides O2 to cells Hgb portion acts as acid base buffer Carbonic anhydrase allows CO2 to react with blood to be transported to the lungs. Functions of leukocytes Neutrophils and monocytes are phagocytes involved in inflammatory reactions Eosinophils- involved in allergic or hypersensitivity reactions Basophils- responsible for histamine release that increases blood vessel permeability at the site of injury

 Functions of platelets Clot formation Releases SEROTONIN, a vasoconstrictor, at the site of injury to decrease blood flow

ASSESSMENT OF AND THERAPEUTIC TECHNIQUES FOR HEMATOLOGIC DISORDERS

a. 1. a. b. c. a. b. c. Health history General considerations Activity Lack of energy Tires easily SOB Diet Lack of Fe in the diet Poor growth, appetite Tendency to bruise or bleed easily d. Recurrent infections e. Illness in siblings Family considerations a. History of bleeding tendencies b. Recent infections c. Malignancy d. Anemia e. Maternal HIV Physical examinationfindings (+) indication of hematologic dysfunction a. lymph- inspect and palpate nodes for tenderness and enlargement

b. Skin: pallor, petechiae, cyanosis, ecchymoses, purpura, clubbing of nails c. Oral cavity: pallor, bleeding d. Neurologic: lethargy, irritability e. GI: hepatosplenomegaly f. Musculoskeletal: bone pain, joint swelling and pain

DIAGNOSTIC TESTS
TESTS FOR BLOOD COAGULATION PROTHROMBIN TIME (PT) Definition Normal value Measures 11-13 sec (PT) action of prothrombin after complete thrombplastin is added to the blood in a test tube Reveals deficiencies in prothrombin, factor V, VII and X

Partial Thromboplastin time (PTT)

Measures activity of thromboplastin after incomplete thromboplastin is added to the blood in a test tube Reveals deficiencies in thromboplastin, factors VII-XII

30-45 sec.

Bleeding time

Measures the time 3-10 minutes required for bleeding at a stab wound on the earlobe to cease Reveals deficiencies in platelet formation and vasoconstrictive ability

Clot retraction

Measures platelet Retraction at side function of test tube in 1 hr; Interval of placement of complete in 24 hr. blood in a tube to the point of clot shrinks and expels serum

tourniquet

Measures capillary 0-2 petechiae per fragility and platelet cm. area function Response of tissue application of tourniquet to forearm for 5-10 mins.
Evaluates thromboplastin function Childs blood is allowed to clot and PT is then done on the serum; if clot formation used a lot of prothrombin,

Prothrombin consumption time

COMPONENTS OF A COMPLETE BLOOD COUNT (CBC)

1. RBC, Hgb, and Hct- measures the O2 carrying capacity of the blood 2. WBC- used ot diagnose infection; will INCREASE 3. Differential WBCs: used to diagnose bacterial, viral and fungal infections 4. Mean corpuscular volume (MCV) and mean corpuscular Hgbused to diagnose IDA 5. Platelet count-determines severity of bleeding or potential

BONE MARROW ASPIRATION AND BIOPSY

Provides samples of bone marrow so that the type and quantity of cells can be determined. In children, the sites include the iliac crests or spines because these have larger marrow compartments during childhood. In neonates, the anterior tibia can be used. For a bone marrow aspiration, a child lies prone on a treatment table . Use of a hard table rather than a bed is advantageous because pressure is needed to insert the needle. Topical anesthesia helps to reduce pain. If conscious sedation is done, monitor VS until stable Monitor pulse and BP every 15 mins. For the 1st hr. to check for bleeding

BLOOD TRANSFUSION
It is used in treatment of many disorders including the anemias and primary immunodeficiency disorders. Blood must be infused with a normal saline solution. The usual amount of blood transfused to children is 15 ml/kg body wt. NURSING RESPONSIBILITITES: 1. Obtain consent. 2. Obtain baseline VS 3. Ensure that the blood is properly typed and cross matched. 4. Monitor VS evry 15 mins. For the 1st hour and approximately every 30 mins for the remaining hrs. 5. Give infusion slowly for 1st 15 mins. And increase rate to 10 ml/kg/hr if no reaction occurs.

RELATIONSHIPS BETWEEN BLOOD TYPES AND ANTIBODIES Blood Type A B AB O Antigens on Red Blood Cell AA B A and B None Can Donate Blood To A, AB B, AB AB A, B, AB, O Antibodies in Serum Anti-B Anti-A None Anti-A and anti-B Can Receive Blood From A, O B, O AB, O O

COMMON BLOOD TRANSFUSION REACTIONS

1. Headache, chills, back pain, dyspnea, hypotension, hemoglobinuria (blood in urine) Cause: anaphylactic reaction to incompatible blood; agglutination of RBCs; kidney tubules may become blocked resulting in kidney failure Time of occurrence: immediately after start of BT Nursing intervention: 1. Discontinue BT 2. Maintain normal saline infusion 3. Administer O2 as needed Anticipate doctors order for diuretic to increase renal tubule flow and reduce tubule plugging and /or heparin to reduce intravascular coagulation 2. Pruritus, urticaria (hives), wheezing Cause: allergy to CHON components of transfusion Time of occurrence: within 1st hr. after start of infusion Nursing intervention: 1. Discontinue transfusion temporarily 2. Give O2 as needed 3. Anticipate order for antihistamine to reduce symptoms

3. Increased Temperature Cause: possible contaminant in transfused blood Time of occurrence: approximately 1 hr. after transfusion Nursing intervention: 1. Discontinue BT 2. Obtain blood culture to rule out bacterial invasion as ordered 4. Increased pulse, dyspnea Cause: circulatory overload Time of occurrence: during course of transfusion

Nursing Intervention: 1. Discontinue BT 2. Give O2 as needed 3. Provide supportive care for pulomary edema and CHF 4. Anticipate order for diuretic to increase excretion of fluid

5. Muscle cramping, twitching of extremities, convulsion Cause: acid-citrate-dextrose anticoagulant in transfusion is combining with serum calcium causing hypocalcemia Time of occurrence: during course of BT Nursing interventions: 1. Discontinue BT 2. Anticipate order for calcium gluconate IV to restore calcium level

6. Fever, jaundice, lethergy, tenderness over liver Cause: hepatitis form contaminated transfusion Time of occurrence: weeks or months after BT Nursing interventions: 1. Obtain transfusion history 2. Refer for care of hepatitis 7. bronze-colored skin Cause: hemosidesrosis or deposition of Fe from transfusion into skin Time of occurrence:after repeasted transfusions Nursing intervention: 1. Support self-esteem with altered body image 2. Administer iron chelating agent (deferoxamine) as ordered to reduce level of accumulating Fe

STEM CELL TRANSPLANTATION

Is the intravenous infusion of hematopeitic stem cells form bone marrow obtained by marrow aspiration or umbilical cord blood form a donor to reestablish marrow function in a child with defective or nonfunctioning bone marrow May be: a. ALLOGENEIC TRANSPLANTATION- Involves transfer of stem cells from an immune-compatible donor b. SYNERGENEIC TRANSPLANTATION- used when donor and recipeint are genetically identical c. AUTOLOGOUS TRANSPLANTATION- Childs own stem cell are used

NURSING RESPONSIBILITY: 1. Administer CYLCLOPHOSPHAMIDE (CYTOXAN) IV to suppress marrow and Tlymphocyte production. 2. Infusion takes 60-90 mins. Monitor cardiac rate and rhythm. 3. Fever and chills are common reactions. Admnister Acetaminophen (tylenol), diazepam (valium), or diphenhydramine Hcl (benadryl) to redcue this reaction. 4. After infusion, take childs temperature at 1 hour and then every 4 hrs. to detect infection. 5. Reinforce strict handwashing. 6. Measure WBC count daily

DISORDERS OF THE RED BLOOD CELLS

1. Normochromic, Normocytic Anemias Are marked by impaird production of erythrocytes by the bone marrow or by abnormal or uncompensated loss of circulating RBCs as with acute hemmorhae. RBCs are normal in both color and size, but there are simply too few of them. Acute Blood-loss anemia Might occur form trauma such as an automobile accident with internal bleeding, acute nephritis or in the newborn from disorders such as placenta previa, maternal-fetal or twin-twin transfusion or trauma to the cord or placenta, or from intestinal parasites

2. -

 Clinical manifestations 1. Shock 2. Pale 3. Tachcardia 4. Children breathe rapidly 5. Newborns may have gasping respirations, sternal retractions, and yanosis 6. They do not respond to O2 therapy. 7. Children become inactice Nursing Mgt: 1. Treat bleeding by addressing the underlying cause. 2. Place child in supine 3. Keep child warm with a blanket or place in an incubator or a radiant heat warmer 4. Administer a blood expander such as normal saline or ringers lactate to expand blood volume and increase BP.

3. -

a. b. c. 4. -

Anemia of Acute Infection Acute infection or inflammation may lead to increased destruction of erythrocytes and therefore lead to decreased eryythrocyte levels. Common conditions include: Osteomyelitis Ulcerative colitis Advanced renal disease MGT: Treatment of the underlying condition Anemia of Renal Disease Renal disease causes loss of function in kidney cells and this causes a decrease in erythropoeitin produciton which decreases the stimulation for RBC production in the one marrow and a resultan normocytic, normochromic anemia occurs. MGT: Administration of recombinant human erythropeitin to increase RBC production and correct anemia but not the renal disease

5. Anemia of Neoplastic Disease - Malignant growths like leukemia or lymhosarcoma results in normocytic, normochromic anema because of impaired RBC production because the bone marrow is invaded by proloiferating neoplastic cells. - MGT: measures designed to achieve remission and transfusion to increase erythrocyte count 6. Aplastic Anemia - Results from depression of hematopeitic activity in the bone marrow. - It can affect formation and development of WBCs, platelets and RBCs. - Can be congenital or acquired a. Congenital aplastic anemia (Fanconis syndrome) - Is inherited as an autosomal recessive trait - A child is born with a number of congenital anomailes such as

Skeletal and renal anomalies, hypogenitalism, and short stature Between 4 and 12 yrs. Of age, a child begins to manifest PANCYTOPENIA (reduction of all blood cell components). b. Acquired Aplastic Anemia A decrease in bone marrow production that can be caused by: a. Exposure to excessive radiation b. Drugs including antibiotics and antineoplastic agents c. Infection: including hepatitis and human parvovirus d. Chemicals: Benzenes and petroleum products PATHOPHYSIOLOGY: 1. Decreased production of RBCs 2. Replacement of cellular elements of bone marrow with fat 3. Results in PANCYTOPENIA manifested as: severe anemia (decreased Hgb and Hct), leukopenia (decreased WBC), thrombocytopenia (decreased platelets)

ASSESSMENT: 1. pallor 2. Easy fatigability reflects lower RBC count and tissue hypoxia 3. Anorexia 4. Child bruises easily or has petechiae (pinpoint, macular, purplish red spots caused by intradermal or submucous hemorrhage)- due to decreased platelet formation 5. Excessive nosebleeds or GI bleeding 6. Recurrent infections due to decreased WBC count 7. Responds poorly to antibiotic therapy 8. Monitor for signs of cardiac decompensation such as tachycardia, tachypnea, SOB or cyanosis 9. Ask for exposure to drugs or chemicals or recent infection

DIAGNOSTIC PROCEDURES
1. Laboratory Studies a. CBC- reveals macrocytic anemia b. Platelet- decreased count 2. Diagnostic studies a. Bone marrow aspiration and biopsy - Reveals replacement of red bone marrow by fatty, yellow marrow

THERAPEUTIC MANAGEMENT
1. 2. a. b. c. d. 3. a. b. The ultimate therapy is STEM CELL TRANSPLANTATION. Medications: globulins: antilymphocyte (ALG) and antithymocyte (ATG) Epoetinalfa (Epogen) therapy: to stimulate erythropoeisis Immunosuppressive agents: cytoxan Oral corticosteroid: prednisone Nursing Mgt. Use good hand washing before and after contact with the child. Assess for bleeding from any orifice; check urine and stool for blood. c. monitor platelet, WBC, Hgb, Hct and neutrophil count

d. e. f.

Limit number of blood drawing procedures Use a BP cuff instead of a torniquet to reduce the number of petechiae. Apply pressure to any puncture site for a full 5 mins. Before applying a bandage. g. Minimize use of adhesive tape to the skin for removal may tear the skin and cause petechiae. h. Pad side and crib rails to prevent bruising. i. Protect IV sites to avoid numerous insertions. j. Administer medication orally or by IV infusion to minimize the number of injection sites. k. Assess diet for foods that the child can chew w/o irritation. l. Urge to use a soft toothbrush. m. check toys for sharp corners w/c may cause scratches

n. Assess need for routine BP determination. Tight cuffs could lead to petechiae. o. Distract child from rough play; suggest stimulating but quiet activities to minimize risk of injury. p. Keep a record of blood drawn; do not draw extra amounts just in case so children do not become more anemic.

HYPOPLASTIC ANEMIAS
Also result from depression of hematopoetic activity in the bone marrow Can be either congenital or acquired In hypoplastic anemias, only RBCs are affected. The RBCs are normochromic andnormocytic but few in number a. Congenital Hypoplastic Anemia (Blackfan-Diamond Syndrome) - A rare disorder that shows symptoms as early as the 1st 6-8 months of life - It affects both sexes and is apparently caused by an inherent defect in RBC formation.
No changes in the leukocytes or platelets occur. b. Acquired Hypoplastic Anemia Is caused by the infection with PARVOVIRUS, the infectious agent of the 5th disease. Reduction of RBC is transient, so no therapy is necessary. MGT: a. In congenital form, children show increased erythropoeisis with corticosteroid therapy. b. Long term transfusions of packed RBCs are necessary. c. Administer an IRON CHELATION PROGRAM such as subcutaneous infusion (hypodermoclysis) of Deferoxamine may be started concurrently with transfusion to

Counteract HEMOSIDEROSIS (deposition of FE in body tissue) w/c is due to a number of transfusions. Deferoxamine a. binds with Fe and aids in excretion from the body in the urine b. It is given 5 or 6 days a week over an 8 hour period c. Assess that voiding is present and specific gravity is normal before administration.

d. An area beside the scapula or the thigh is cleaned with alcohol; a short 25-gauge needle is inserted at a low angle into only the subcutaneous tissue e. Periodic slit lamp eye exams should be done to check fro cataract formation which is a possible adverse effect of Deferoxamine.

HYPOCHROMIC ANEMIAS
When Hemoglobin synthesis is inadequate, the erythrocytes appear pale (HYPOCHROMIA). Hypochromia is generally accompanied by a reduction in the diameter of cells. RBCs are also microcytic.

IRON DEFICIENCY ANEMIA

It is the most common anemia of infancy and childhood Occurs when intake of dietary Fe is inadequate Inadequate Fe prevents proper Hgb formation With IDA, RBCs are both small in size (hypocytic) and pale (hypochromic) due to the stunted Hgb. A daily intake of 6-15 mg of Fe is necessary. IDA occurs omst often between 9 months and 3 yrs. And rises again in adolescence when Fe requirements increase for girls who menstruate. It is also seen in overweight teenagers if they ingest most calories from high CHO not Fe rich foods.

CAUSES OF IDA IN INFANTS

1. 2. 3. 4. Deficient Fe in the diet Low-birth weight Women with IDA during pregnancy Infants born with structural defect such as Gastroesophageal reflux, or pyloric stenosis. 5. Chronic diarrhea MGT: a. Give Fe fortified formula for the 1st yr. b. Fe fortified cereals should be introduced when solid foods are introduced in the 1st year.

CAUSES OF IDA IN OLDER CHILDREN

1. For children older than 2 yrs. Chronic blood loss is the most frequent cause of IDA. This results from GI tract lesions such as polyps, ulcerative colitis, Crohns disease, protein induced enteropathies, parasitic infestation or frequent epistaxis. 2. Adolsecent girls can become Fe deficient because of frequent attempts to diet and overconsumption of snack foods low in Fe.

ANEMIA

THERAPEUTIC MANAGEMENT
1. 2. 3. 4. Treatment focuses on the treatment of the underlying cause. Rule out possibility of GI bleeding. Provide a diet rich in Fe and give extra Vitamin C to enhance absorption. Administer Ferrous Sulfate for 4 to 6 wks. To improve RBC formation and replace Fe store. Nursing Implications when taking FeS04 1. Administer drug on an empty stomach with water to enhance absorption. Of ot causes GI irritation, administer it after meals. 2. Avoid giving it with milk, eggs, coffeee or tea. 3. If liquid preparation is ordered, advise parents to mix it with water to mask the taste and prevent teeth staining. 4. Instruct to drink medication through a straw to prevent staining. 5. Give Fe with a citrus juice to help absorptin. 6. Inform parents and child that stool may turn black. 7. Provide a high fiber diet to minimize risk of constipation 8. Reinfore need for thorough brushing to prevent staining 9. Do follow up blood studies to evaluate drug effectiveness.

 MACROCYTIC (MEGALOBLASTIC) ANEMIAS Is one in which the RBCs are abnormally large. These cells are actually immature erythrocytes or megaloblasts. Are caused by nutritional deficiencies

Anemia of Folic Acid Deficiency - a deficiency of folic acid combined with Vit. C deficiency produces an anemia in which the erythrocytes are abnormally large. - There is accompanying neutropenia and thrmbocytopenia. - MCV and MCH are increased whereas mean corpusuclar Hgb concentration is normal

- Bone marrow contain megaloblasts indicating inhibition Of production of erythrocytes at an early stage. - TX: a. Daily oral administration of folic acid.

a. b. c. d. e.
f.

Pernicious Anemia (vitamin B12 deficiency) Vitamin B12 is necessary for the maturation of RBCs. Results from deficiency or inability to use vitamin B12 which is ofund primarily in foods of animal orgin including both cows milk and breast milk For vit. B12 to be absorbed from the intestine, an intrinsic factor must be present Manifestations of intrinsic factor deficiency are: Pallor Anorexia Irritability Chronic diarrhea0 Tongue appear smooth and beefy red due to papillary atrophy Neuropathologic findings like ataxia, hyporeflexia, paresthesia and (+) Babinski reflex are less noticeable.

 Lab findings reveal low serum levels of Vitamin B12. Mgt: a. If the anemia is due to a B12 deficient diet, temporary injections will reverese symptoms. b. If it is caused by lack of intrinsic factor, lifelong monthly IM injection of vitamin B12 may be necessary.

Hemolytic Anemias -those in which the number of erythrocytes decreases due to increased destruction of erythrocytes.

 Congenital Spherocytosis - Is a hemolytic anemia that is inherited as an autosomal dominant trait. - It occurs most frequently in the white Northern European population. - Cells are small and defective. - The hemolysis of RBCs appears to occur in the spleen apparently from excessive absorption of sodium into the cell.

Chronic jaundice and splenomegaly develop. Mean corpuscular Hgb concentration is increased Gallstones may be present in older children and adolescent Tx: - Splenectomy at approximately 5-6 yrs.

 Glucose 6 Phosphate Dehydrgenase Deficiency (G6PD) - The enzyme G6PD is necessary for maintenance of RBC life. - Lack of the enzyme results in premature destruction of RBC. - It is transmitted as a sex-linked recessive trait. - Occurs in 2 identifiable forms: a. Children with congenital nonspherocytic hemolytic anemia have hemolysis, jaundice and splenomegaly and may have aplastic crises.

b. Others have drug induced forms in which blood patterns are normal until child is exposed to fava beans or drugs such as antipyretics, sulfonamides, antimalarials, and naphthaquinolones (the most common is ASA. Approximately after 2 days of ingestion, child shows evidence of hemolysis. DX: - A blood smear will show HEINZ bodies (oddly shaped particles in RBCs). - Rapid enzyme screening test - Newborn screening test

SICKLE CELL ANEMIA

It is the presence of abnormally shaped (elongated) RBCs. It is an autosomal recessive inherited disorder of the beta chain of Hgb; the amino acid valine takes the place of the normally appearing glutamic acid. Pathophysiology: a. In Hgb S, the defect is a substitution of valine ofr glutamine b. Erythrocytes containing Hgb S (HbS) beocme sickled in situations of decreased O2 tension and decreased hydration. c. Sickled RBCs are crescent shaped, have reduced O2 carrying ability and decreased life span

 Sickled RBCs are rigid, cause trapping and increased blood viscosity, capillary stasis and thrombosis eventually tissue ischemia nad necrossis result SICKLE CELL CRISIS - denotes a sudden, severe, onset of sickling. - Can occur when child has an illness causing DHN or a respiratory infection that results to lowered O2 exchange or lowered arterial O2 level; after extrenely strenous exercise

Diagnostic Procedures: a. Hgb electrophoresis (fingerprinting) - Detects homozygous and heterozygous forms of the disease and percentages of various Hgb forms b. Sickle-turbidity test (sickledex): screening test for Hgb S c. Blood smear: may reveal shape of RBC to be sickled rather than normal biconcave disk d. Antenatal screening possible through amniocentesis

 Pediatric complications a. Delayed growth, development and onset of puberty b. Impaired fertility c. Priapism- prolonged or constant penile erection that is painful and infrequently associated wuth sexual arousal; can result from urinary calculi; caused by micorcirculating obstruction and engorgement of the penis Mgt: a. bedrest b. Sedation c. administer Demerol d. Enuresis- especially at night

Therapeutic Mgt: a. Medications 1. Analgesics to control severe pain during crisis 2. antibiotics to treat existing infection b. Treatments 1. Rest 2. O2 administration 3. Fluid and electrolyte replacement 4. Blood replacement c. Nursing Mgt: 1. Assess for signs of hypoxia: irritabilty, restlessnss, agitation, hyperventilation, increased apical pulse and RR, confusion, cyanosis 2. Monitor Iand O 3. Assess for signs of infection

4.

Provide rest periods to decrease O2 expenditure 5. Administer blood products as ordered 6. Assess location, severity, duration and quality of pain 7. Assess intensity f pain using age appropriate pain scale 8. Administer analgesics as ordered 9. Apply heat to affected area 10. Enocurage relaxation techniques: DBE, guided imagery 11. Gently handle painful joints and extremities, provide support with pillows

THALASSEMIAS
Are autosomal recessive anemias associated with abnormalities of the beta chain of adult hemoglobin (HgbA). 1. Thalassemia Minor (Heterozygous BetaThalassemia) - A mild form of anemia which produces both defective beta Hgb and normal Hgb. - RBC count will be normal but the Hgb concentration will be decreased 2-3g/100 ml below normal levels. - Blood cells are moderately hypochromic and microcytic. 2. Thalassemia-Major (Homozygous Beta-Thalassemia) - Or Cooleys anemia or Mediterranean Anemia - RBCs are hypochromic and microcytic - Fragmented poikilocytes and basophilic stippling (eveness of Hgb concentration) are present - Hgb level is <5g/100 ml - Serum Fe level is high - Fe saturation is 100%

 ASSESSMENT 1. Bone pain 2. Characterisitc change in the shape of the skull (parietal and frontal bossing) and protrusion of the upper teeth with marked malocclusion. 3. Base of the nose may be broad and flattened 4. The eyes may be slanted with an epicanthal folds 5. An x-ray of bone shows marked osteoporotic tissue possibly resulting in fractures 6. Hepatosplenomegaly 7. Anorexia and vomiting 8. epistaxis

9.

DM due to pancreatic hemosiderosis (deposition of Fe) 10. Cardiac dilatation with murmur 11. Arryhthmias and heart failurefrequent cuase of death Therapeutic Mgt: 1. Administer diuretics, digitalis 2. Proivde a low Na diet 3. Transfusion of packed RBCs every 2-4 wks will maintain Hgb lbtween 10 and 12 g/100 ml. 4. Administer Fe chelating agent such as Deferoxamine to remove excessive store of Fe (given Sqover 6-8 hrs. as they sleep at night) 5. Splenectomy

 Polycythemia - An increase in the number of RBCs - Results from increased erythropoeisis - Usually caused by chronic pulmonary disease and congenital heart disease - Plethora (marked reddened appearance of the skin) occurs because of the increase in total RBC volume

Mean corpuscular Hgb is elevated, mean corpuscular Hgb concentration will be normal. Treatment: treatment of the underlying cause

DISORDERS OF THE WHITE BLOOD CELLS

DISORDER Neutropenia DESCRIPTION Reduced no. of WBC CAUSES/TREATMENT Transient phenomenon with nonpyrogenic infections such as viral disease Possible side effect from some drugs such as Phenytoin (Dilantin), chloramphenicol or chlorpromazine Treatments: possibly WBC transfusion, prophylactic antibiotics

eosinophilia

Increased number of eosinophils

Associted with allergic disorders and with parasitic invasion Normally occurs in the preschool period Abnormally elevated in childhood illnessess like pertussis, infectious mononucleosis and lymphocytic

Lymphocytosis Increased number of lymphocytes

HEMOPHILIA
Inherited bleeding or coagulation, disorder. Persons with hemophilia lack the ability to stop bleeding because of the low levels, or complete absence, of specific proteins, called "factors," in their blood that are necessary for clotting. Proper clotting of blood helps prevent excessive bleeding. Types of hemophilias hemophilia A - lack of factor VIII hemophilia B - lack of factor IX

CAUSES
Hemophilia types A and B are inherited diseases passed on from a gene located on the X chromosome. Females carrier of hemophilia has the hemophilia gene on one of her X chromosomes, and there is a 50 percent chance that she may pass the defective gene to her male offspring. Males who inherit the defective gene will develop hemophilia. Males with hemophilia do not pass the gene to their sons; however, they do pass the gene to their daughters.

 Females who inherit the defective gene will become carriers who may, in turn, have a 50 percent chance of passing it on to their children. Although females who inherit the gene generally have no active problems related to hemophilia, some may have other problems associated with bleeding, such as excessive menstrual bleeding, frequent or severe nosebleeds, or bleeding after dental procedures or surgery. In about 1/3rd of hemophilia cases, there is no family history of the disease. These cases are due to a new or spontaneous development of the defective gene in the female.

SYMPTOMS
Excessive, uncontrollable bleeding Bleeding may occur even if there is no injury. Often occurs in the joints and in the head. Bruising - Occur from small accidents, which can result in a large hematoma. Bleeds easily - Tendency to bleed. Bleeding into a joint - Hemarthrosis can cause pain, immobility, and eventually deformity if not medically managed properly.

DIAGNOSIS & EFFECTS

Complete medical history and physical examination Clotting factor levels Complete blood count (CBC) Assessment of bleeding times DNA testing. Most common cause of disability from hemophilia is chronic joint disease or arthropathy, which is caused by uncontrolled bleeding into the joints. Hemorrhage severe internal or external discharge of blood, is a continuing problem.

TREATMENT
Blood transfusions

Prophylactic (preventive) treatment with infused clotting factors

NURSING MANAGEMENT
1. Assess for signs of active bleeding, hemarthrosis 2. Administer plasma CHON, factor replacement or cryopercipitate as ordered 3. Apply pressure and cold compresses to site of injury 4. Elevate and immobilize affected limb 5. Teach parents of symptoms of bleeding: pain, swelling, limited joint motion 6. Teach parents to administer plasma CHON when signs of bleeding appar 7. Provide a soft toothbrush 8. Inspect toys for sharp edges or parts 9. Pad crib sides 10. Avoid contact sports 11. Encourage iron-rich foods 12. Assess mobility status:joint mobility, pain, stiffness, swelling, muscle tone, ability to perform ADLs.

13. Assess complications of immobility;skin breakdown, contractures, loss of muscle strength, constipation 14. Provide active and passive ROM q 2-4 hrs. as needed.

Immune Thrombocytopenic Purpura (Thrombocytopenia)

Blood disorder characterized by an abnormal decrease in the number of blood platelets, which results in internal bleeding. Acute thrombocytopenic purpura Most common in young children, the symptoms may follow a virus infection and disappears within a year - usually disorder does not recur. Chronic thrombocytopenic purpura Onset of the disorder can happen at any age, and symptoms can last six months or longer. Adults have this form more often than children, and females have it 3 times more often than males.

CAUSES
Medications - including over-the-counter Infection Pregnancy Immune disorders However, about half of all cases are classified as idiopathic.

SYMPTOMS
Internal bleeding, which may cause: ecchymosis bruising , petechiae - tiny red dots on skin or mucous membranes Occasionally, bleeding from the nose, gums, digestive tract, urinary tract Rarely, bleeding within the brain Symptoms may resemble other blood disorders or medical problems.

DIAGNOSIS
Complete medical history and physical examination Additional blood and urine tests Other evaluation procedures Careful review of patient's medications Bone marrow examination

TREATMENT
Treatment of the causative disease Discontinuation of causative drugs Treatment with corticosteroids Treatment with medications Lifestyle changes, such as: use of protective gear , avoidance of certain activities

HEMOCHROMATOSIS
Also called iron overload disease, is the most common genetic disorder. It is a metabolic disorder that causes increased absorption of iron, which is deposited in the body tissues and organs. The iron accumulates in the body where it may become toxic and cause damage.

HODGKIN'S DISEASE
Type of lymphoma, a cancer in the lymphatic system. Rare disease usually occurs most often in people between the ages of 15 and 34, and in people over age 55. Hodgkin's disease causes the cells in the lymphatic system to abnormally reproduce, eventually making the body less able to fight infection. Hodgkin's disease cells can also spread to other organs.

STAGES OF HODGKINS DISEASE

STAGE I II EXTENT OF DISEASE Disease affects single lymph node or single extralymphatic organ or site Disease affects 2 or more lymph node regions on the same side of the diadphragm or there is localized involvement of an extralyumphatic organ or site Disease affects lymph node regions on both sides of the diaphragm or there is localied involvement of an extralymphatic organ or site There is diffuse or disseminated involvement of extralymphatic organs with or without associated lymph node involvement

III

IV

Lymphadenoma Hodgkin's Disease (Pseudo-leukemia of German authors)

SIGNS AND SYMPTOMS

Painless swelling of lymph nodes in neck, underarm, and groin Fever Night sweats Fatigue Weight loss Itching of the skin It may resemble other blood disorders or medical problems, such as influenza or other infections.

RISK FACTORS
Past infection with infectious mononucleosis History of infectious mononucleosis (caused by an infection with the Epstein-Barr virus) Acquired immunodeficiency syndrome (AIDS)

DIAGNOSIS
Additional blood tests X-rays of the chest, bones, liver, and spleen Biopsy of the lymph nodes

TREATMENT
Radiation therapy Chemotherapy

LEUKEMIA
Cancer of the blood cells, usually the white blood cells. Leukemic cells look different than normal cells and do not function properly.

TYPES OF LEUKEMIA
Lymphocytic or myelogenous leukemia Cancer can occur in either the lymphoid or myeloid white blood cells. When the cancer develops in the lymphocytes (lymphoid cells), it is called lymphocytic leukemia. Cancer develops in the granulocytes or monocytes (myeloid cells) myelogenous leukemia.

Acute or chronic leukemia Acute leukemia - The new or immature cells, called blasts, remain very immature and cannot perform their functions. The blasts increase in number rapidly, and the disease progresses quickly. Chronic leukemia - There are some blast cells present, but they are more mature and are able to perform some of their functions. The cells grow more slowly, and the number increases less quickly, so the disease progresses gradually.

LEUKEMIA IS CLASSIFIED INTO ONE OF THE FOUR MAIN TYPES OF LEUKEMIAS

Acute myelogenous leukemia (AML) Chronic myelogenous leukemia (CML) Acute lymphocytic leukemia (ALL) Chronic lymphocytic leukemia (CLL)

SIGNS AND SYMPTOMS

More frequent infections and fevers Anemia and its symptoms: pale skin, fatigue, weakness, bleeding, bruising, fever, chills, loss of appetite, loss of weight, swollen or tender lymph nodes, liver, or spleen, petechiae (tiny red spots under the skin), swollen or bleeding gums, sweating, bone or joint pain. Acute leukemia: headaches, vomiting, confusion, loss of muscle control, seizures, swollen testicles, sores in the eyes or on the skin.

 Chronic leukemia may affect the skin, central nervous system, digestive tract, kidneys, and testicles. The symptoms of acute and chronic leukemias may resemble other blood disorders or medical problems

DIAGNOSIS
Physician examination for swelling in the: liver, spleen, lymph nodes under the arms, in the groin, and in the neck Blood tests and laboratory tests Blood tests to examine the blast (immature) blood cells Bone marrow aspiration and biopsy Lymph node biopsy Spinal tap Imaging procedures, such as x-ray, ultrasound, and computed tomography (CT)

TREATMENT
Chemotherapy Radiation therapy Bone marrow stem cell transplantation Biological therapy Platelet transfusion Red blood cell transfusion Medications to prevent or treat damage to other systems of the body caused by leukemia treatment

ACUTE LYMPHOCYTIC LEUKEMIA [ALL]

ALL is a cancer of the blood in which too many lymphocytes, a type of white blood cell, are produced by the bone marrow and by organs of the lymph system. The lymphocytes fight infection by making antibodies that attack harmful elements. But, in ALL, the cells are immature and overabundant. They crowd out other blood cells, and may collect in the blood, bone marrow, and lymph tissue.

 Acute leukemia can occur over a short period of days to weeks. Chromosome abnormalities (extra chromosomes and structural changes in the chromosome material) are present in the majority of all patients. ALL is the most common type of leukemia in young children. This type of leukemia may also affect adults, especially those age 65 and older.

SIGNS AND SYMPTOMS

Anemia Bleeding Bruising Fever Persistent weakness Fatigue Aches in bones and joints Swollen lymph nodes

DIAGNOSIS
Blood tests and other evaluation procedures Bone marrow aspiration and biopsy Spinal tap/lumbar puncture - A small amount of cerebral spinal fluid (CSF) removed. CSF is the fluid that bathes the brain and spinal cord.

TREATMENT
Chemotherapy Radiation therapy Bone marrow transplantation

ACUTE MYELOGENOUS LEUKEMIA [AML]

AML cancer of the blood in which too many granulocytes are produced in the bone marrow. Bone marrow cells mature into several different types of blood cells. AML affects the young blood cells (called blasts) that develop into a type of white blood cell (called granulocytes). Main function of granulocytes is to destroy bacteria. Blasts, do not mature & become too numerous, remain in the bone marrow and blood.

 Acute leukemia can occur over a short period of days to weeks. Chromosome abnormalities (extra chromosomes and structural changes in the chromosome material) are present in the majority of ALL patients. AML occurs in both children and adults.

SIGNS AND SYMPTOMS

Anemia Bleeding Bruising Fever Persistent weakness Fatigue Aches in bones and joints Swollen lymph nodes

DIAGNOSIS
Complete medical history and physical examination Blood tests Bone marrow aspiration and biopsy Spinal tap/lumbar puncture

TREATMENT
Chemotherapy Radiation therapy Bone marrow transplantation

CHRONIC LYMPHOCYTIC LEUKEMIA [CLL]

CLL cancer of the blood in which too many lymphocytes, a type of white blood cells, are produced by the bone marrow and by organs of the lymph system. Lymphocytes fight infection by making antibodies that attack harmful elements, but in CLL, the cells are immature and over abundant. They crowd out other blood cells, and may collect in the blood, bone marrow and lymph tissue. CLL usually occurs in people 60 years of age or older. It is a slowly progressing disease.

SIGNS AND SYMPTOMS

Persistent weakness Swollen lymph nodes Enlarged spleen Enlarged liver Anemia

DIAGNOSIS
Complete medical history Physical examination Blood tests Bone marrow biopsy
TREATMENT Chemotherapy Radiation therapy Treatment for complications infection or anemia Leukapheresis, a procedure to remove excessive lymphocytes Bone marrow transplantation Splenectomy, surgery to remove the spleen

CHRONIC MYELOGENOUS LEUKEMIA [CML]

CML cancer of the blood in which too many granulocytes, a type of white blood cell, are produced in the bone marrow. Bone marrow cells mature into several different types of blood cells. CML affects the young blood cells (called blasts) that develop into a type of white blood cell (called granulocytes). Main function of granulocytes is to destroy bacteria. The blasts, which do not mature and become too numerous, remain in the bone marrow and blood.

 CML can occur over a period of months or years. Specific chromosome rearrangement is found in patients with CML. Part of chromosome #9 breaks off and attaches itself to chromosome #22, so that there is an exchange of genetic material between these two chromosomes. This rearrangement changes the position and functions of certain genes, which results in uncontrolled cell growth. Other chromosome abnormalities can also be present. CML occurs mainly in adults and is rare in children.

SIGNS AND SYMPTOMS

Anemia Bleeding Bruising Fever Persistent weakness Fatigue Aches in bones and joints Swollen lymph nodes

DIAGNOSIS
Blood tests Bone marrow aspiration and biopsy Spinal tap/lumbar puncture

TREATMENT
Chemotherapy Biological therapy - using the body's immune system to fight cancer Radiation therapy Stem cell transplantation Splenectomy

NURSING MANAGEMENT FOR LEUKEMIA

1. Use strict hand washing before and after contact. 2. Assess for side effects of chemotherapy. 3. Administer an antiemetic 30 mins. Before chemotrherapy is ordered. 4. Assess oral mucosa for pain, ulcers, lesions, stomatits, and effects on eating 5. Assess for bleeding from any orifice; check urine and stool for blood 6. Monitor platelet, WBC,Hgb,Hct count 7. assess for signs of infection 8. Provide mouth rinses and soft bristled toothbrush 9. Administer topical xylocaine before meals as ordered. 10. Provide a soft, bland diet 11. Isolate from persons with URTI. 12. Report immediately any sings of fever, pallor, oozing blood, exposure to a communicable disorder

NON-HODGKIN'S LYMPHOMA
Type of lymphoma, which is a cancer in the lymphatic system. Non-Hodgkin's disease causes the cells in the lymphatic system to abnormally reproduce eventually causing tumors to grow and can also spread to other organs. Etiology is idiopathic

SYMPTOMS
Painless swelling of lymph nodes in neck, underarm, and groin Fever Night sweats Fatigue Weight loss Itching of the skin Recurring infections

RISK FACTORS
Genetic disease of the immune system Unprotected exposure to strong sunlight High-fat, low-fiber diet Smoking Excessive alcohol consumption Environmental factors radiation, chemicals, and infections

Organ transplantation Infections with HIV or HTLV-1 Infections with malaria History of infectious mononucleosis Helicobacter pylori bacterium stomach ulcers

DIAGNOSIS
Blood tests X-rays of the chest, bones, liver, and spleen Biopsy of the lymph nodes, bone marrow, and other sites Lymphangiograms - lymphatic system x-rays CT scan Ultrasonography scan

TREATMENT
Radiation therapy Chemotherapy

THROMBOCYTHEMIA
It is a myeloproliferative blood disorder. It is characterized by the production of too many platelets in the bone marrow. Too many platelets make normal clotting of blood difficult Etiology is idiopathic

SYMPTOMS
Increased blood clots in arteries and veins Bleeding Bruising easily Bleeding from the nose, gums, gastrointestinal tract Bloody stools Hemorrhaging after injury or surgery Weakness Enlarged lymph nodes

DIAGNOSIS
Complete medical history and physical examination Blood counts and elevated platelet levels Bone-marrow biopsy

TREATMENT
Chemotherapy Plateletpheresis - a procedure to remove extra platelets from the blood

BONE MARROW TRANSPLANTATION [BMT]

BMT is a special therapy for patients with cancer or other diseases which affect the bone marrow. A bone marrow transplant involves taking cells that are normally found in the bone marrow (stem cells), filtering those cells, and giving them back either to the patient or to another person. The goal of BMT is to transfuse healthy bone marrow cells into a person after their own unhealthy bone marrow has been eliminated.

 Bone marrow transplantation is not yet a standard treatment therapy, but has been used successfully to treat diseases such as leukemias, lymphomas, aplastic anemia, immune deficiency disorders, and some solid tumor cancers since 1968.

 Bone marrow is the soft, spongy tissue found inside bones. It is the medium for development and storage of about 95 percent of the body's blood cells. Blood cells that produce other blood cells are called stem cells. The most primitive of the stem cells is called the pluripotent stem cell, which is different than other blood cells Renewal - able to reproduce another cell identical to itself. Differentiation - able to generate one or more subsets of more mature cells. It is the stem cells that are needed in bone marrow transplantation.

NORMAL ANATOMY

INDICATION

PROCEDURE

AFTER CARE

GOAL OF BMT
Cure many diseases and types of cancer.

When a person's bone marrow has been damaged or destroyed due to a disease or intense treatments of radiation or chemotherapy for cancer, a marrow transplant may be needed.

A bone marrow transplant can be used to: Replace diseased, non-functioning bone marrow with healthy functioning bone marrow Replace the bone marrow and restore its normal function after high doses of chemotherapy or radiation are given to treat a malignancy process called "rescue". Replace bone marrow with genetically healthy functioning bone marrow to prevent further damage from a genetic disease process (such as Hurler's syndrome, and adrenoleukodystrophy).

BMT BENEFITS
Leukemias Severe aplastic anemia Lymphomas Multiple myeloma Immune deficiency disorders Solid-tumor cancers like breast or ovarian

DIFFERENT TYPES OF BMT

Autologous bone marrow transplant Allogeneic bone marrow transplant A parent - a haploid-identical match is when the donor is a parent and the genetic match is at least half identical to the recipient. An identical twin - a syngeneic transplant is an allogeneic transplant from an identical twin. Identical twins are considered a complete genetic match for a marrow transplant. Unrelated bone marrow transplants (UBMT or MUD for matched unrelated donor) Umbilical cord blood transplant

Stem Cell Transplantations [SCT]

In bone marrow - there is 1 stem cell in every 100,000 blood cells. Breast bone, skull, hips, ribs, and spine contains the stem cells. Harvesting stem cells from bone marrow requires a surgical procedure. Harvesting stem cells from the blood stream is accomplished by a process called apheresis.

Stem Cell Transplantations [SCT]

In bone marrow - there is 1 stem cell in every 100,000 blood cells. Breast bone, skull, hips, ribs, and spine contains the stem cells. Harvesting stem cells from bone marrow requires a surgical procedure. Harvesting stem cells from the blood stream is accomplished by a process called apheresis.