Care of Children With Hematological and Immunological Disorders

8/2/2019 Care of Children With Hematological and Immunological Disorders

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CARE OF CHILDREN WITH

HEMATOLOGICAL ANDIMMUNOLOGICAL DISORDERS


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INTRODUCTION

Blood is the life-maintaining fluid that circulates

through the body's heart, arteries, veins, andcapillaries.

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.


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Functions of the Hematologic system

and its formed elementsa. It is responsible for oxygenation of cells, removal of end products of

metabolism, immune protection, clotting and heat regulation.

b. 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 tothe lungs.

c. Functions of leukocytes

Neutrophils and monocytes are phagocytes involved in inflammatoryreactions

Eosinophils- involved in allergic or hypersensitivity reactions

Basophils- responsible for histamine release that increases blood vesselpermeability at the site of injury


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Functions of platelets

Clot formation

Releases SEROTONIN, a vasoconstrictor, at the site of injury to decrease

blood flow


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ASSESSMENT OF AND THERAPEUTIC

TECHNIQUES FOR HEMATOLOGIC DISORDERS

a. Health history

1. General considerations

Activity

a. Lack of energy

b. Tires easily

c. SOB

Diet

a. Lack of Fe in the diet

b. Poor growth, appetite

c. Tendency to bruise or bleed

easily

d. Recurrent infections

e. Illness in siblings

Family considerations

a. History of bleedingtendencies

b. Recent infections

c. Malignancyd. Anemia

e. Maternal HIV

Physical examination-findings (+) indication of

hematologic dysfunction

a. lymph- inspect and palpatenodes for tenderness andenlargement


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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


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DIAGNOSTIC TESTS

TESTS FOR BLOOD

COAGULATION

Definition Normal value

PROTHROMBIN

TIME (PT)

Measures

action of

prothrombinafter complete

thrombplastin

is added to the

blood in a test

tube Reveals

deficiencies in

prothrombin,

factor V, VII and

X

11-13 sec (PT)


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Partial

Thromboplastin

time (PTT)

Measures activity

of thromboplastin

after incomplete

thromboplastin is

added to the blood

in a test tubeReveals

deficiencies in

thromboplastin,

factors VII-XII

30-45 sec.

Bleeding time Measures the time

required for

bleeding at a stab

wound on the

earlobe to ceaseReveals

deficiencies in

platelet formation

and vasoconstrictive

ability

3-10 minutes


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Clot retraction Measures platelet

function

Interval of placement of

blood in a tube to the point

of clot shrinks and expels

serum

Retraction at side

of test tube in 1 hr;

complete in 24 hr.

tourniquet Measures capillary

fragility and platelet

function

Response of tissue

application of tourniquet toforearm for 5-10 mins.

0-2 petechiae per

cm. area

Prothrombin

consumption

time

Evaluates thromboplastin

function

Childs blood is allowed to

clot and PT is then done onthe serum; if clot formation

used a lot of prothrombin,


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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 andfungal infections

4. Mean corpuscular volume (MCV) and mean corpuscular Hgb-

used to diagnose IDA

5. Platelet count-determines severity of bleeding or potential


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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


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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.


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RELATIONSHIPS BETWEEN BLOOD TYPES AND ANTIBODIES

Blood

Type

Antigens on Red

Blood Cell

Can Donate

Blood To

Antibodies in

Serum

Can Receive Blood

From

A AA A, AB Anti-B A, O

B B B, AB Anti-A B, O

AB A and B AB None AB, O

O None A, B, AB, OAnti-A and

anti-BO


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COMMON BLOOD TRANSFUSION

REACTIONS1. Headache, chills, back pain,

dyspnea, hypotension,

hemoglobinuria (blood in urine)

Cause: anaphylactic reaction to

incompatible blood;

agglutination of RBCs; kidneytubules 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 fordiuretic to increase renal tubuleflow and reduce tubule pluggingand /or heparin to reduceintravascular coagulation

2. Pruritus, urticaria (hives), wheezing

Cause: allergy to CHON components oftransfusion

Time of occurrence: within 1st hr. afterstart of infusion

Nursing intervention:

1. Discontinue transfusiontemporarily

2. Give O2 as needed

3. Anticipate order for antihistamineto reduce symptoms


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3. Increased Temperature

Cause: possible contaminant in

transfused blood

Time of occurrence: approximately 1hr. after transfusion


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 needed3. Provide supportive care for

pulomary edema and CHF

4. Anticipate order for

diuretic to increaseexcretion of fluid


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5. Muscle cramping, twitchingof extremities, convulsion

Cause: acid-citrate-dextroseanticoagulant in transfusionis combining with serumcalcium causinghypocalcemia

Time of occurrence: duringcourse of BT

Nursing interventions:

1. Discontinue BT

2. Anticipate order forcalcium gluconate IV torestore calcium level

6. Fever, jaundice, lethergy, tendernessover liver

Cause: hepatitis form contaminatedtransfusion

Time of occurrence: weeks or monthsafter BT

Nursing interventions:

1. Obtain transfusion history

2. Refer for care of hepatitis

7. bronze-colored skin

Cause: hemosidesrosis or deposition ofFe from transfusion into skin

Time of occurrence:after repeastedtransfusions


1. Support self-esteem with alteredbody image

2. Administer iron chelating agent(deferoxamine) as ordered toreduce level of accumulating Fe


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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


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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


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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 ofthem.

2. 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 thecord or placenta, or from intestinal parasites


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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 aradiant heat warmer

4. Administer a blood expander such as normal saline or ringerslactate to expand blood volume and increase BP.


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3. 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:

a. Osteomyelitis

b. Ulcerative colitis

c. Advanced renal disease

- MGT: Treatment of the underlying condition

4. 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


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5. Anemia of Neoplastic Disease

- Malignant growths like leukemia or lymhosarcoma results in normocytic,normochromic anema because of impaired RBC production because thebone marrow is invaded by proloiferating neoplastic cells.

- MGT: measures designed to achieve remission and transfusion to increase

erythrocyte count6. 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


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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 radiationb. Drugs including antibiotics and antineoplastic agents

c. Infection: including hepatitis and human parvovirus

d. Chemicals: Benzenes and petroleum products

PATHOPHYSIOLOGY:

1. Decreased production of RBCs2. Replacement of cellular elements of bone marrow with fat

3. Results in PANCYTOPENIA manifested as: severe anemia (decreased Hgb andHct), leukopenia (decreased WBC), thrombocytopenia (decreased platelets)


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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 redspots caused by intradermal or submucous hemorrhage)- due todecreased 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


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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


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THERAPEUTIC MANAGEMENT

1. The ultimate therapy is STEM CELL TRANSPLANTATION.

2. Medications:

a. globulins: antilymphocyte (ALG) and antithymocyte (ATG)

b. Epoetinalfa (Epogen) therapy: to stimulate erythropoeisis

c. Immunosuppressive agents: cytoxan

d. Oral corticosteroid: prednisone

3. Nursing Mgt.

a. Use good hand washing before and after contact with the child.

b. Assess for bleeding from any orifice; check urine and stool for

blood.

c. monitor platelet, WBC, Hgb, Hct and neutrophil count


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d. Limit number of blood drawing procedures

e. Use a BP cuff instead of a torniquet to reduce the number of petechiae.

f. Apply pressure to any puncture site for a full 5 mins. Before applying abandage.

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 ofinjection 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


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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 incase so children do not become more anemic.


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HYPOPLASTIC ANEMIAS Also result from depression of

hematopoetic activity in the bonemarrow

Can be either congenital oracquired

In hypoplastic anemias, only RBCsare affected.

The RBCs are normochromicandnormocytic but few innumber

a. Congenital Hypoplastic Anemia(Blackfan-Diamond Syndrome)

- A rare disorder that showssymptoms as early as the 1st 6-8months of life

- It affects both sexes and isapparently caused by aninherent defect in RBCformation.

- No changes in the leukocytes or

platelets occur.b. Acquired Hypoplastic Anemia

- Is caused by the infection withPARVOVIRUS, the infectious agentof the 5th disease.

- Reduction of RBC is transient, sono therapy is necessary.

MGT:

a. In congenital form, children showincreased erythropoeisis withcorticosteroid therapy.

b. Long term transfusions of packedRBCs are necessary.

c. Administer an IRON CHELATIONPROGRAM such as subcutaneousinfusion (hypodermoclysis) ofDeferoxamine may be startedconcurrently with transfusion to


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Counteract HEMOSIDEROSIS(deposition of FE in bodytissue) w/c is due to a numberof transfusions.

Deferoxamine

a. binds with Fe and aids inexcretion from the body inthe urine

b. It is given 5 or 6 days a weekover an 8 hour period

c. Assess that voiding is presentand specific gravity is normalbefore administration.

d. An area beside the scapula orthe thigh is cleaned withalcohol; a short 25-gaugeneedle is inserted at a lowangle into only the

subcutaneous tissuee. Periodic slit lamp eye exams

should be done to check frocataract formation which is apossible adverse effect of

Deferoxamine.


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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.


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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.


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CAUSES OF IDA IN INFANTS

1. Deficient Fe in the diet

2. Low-birth weight

3. Women with IDA during pregnancy

4. 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.


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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 frequentattempts to diet and overconsumption of snack foods low in

Fe.


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ANEMIA


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THERAPEUTIC MANAGEMENT

1. Treatment focuses on the treatment of the underlying cause.

2. Rule out possibility of GI bleeding.

3. Provide a diet rich in Fe and give extra Vitamin C to enhance absorption.

4. Administer Ferrous Sulfate for 4 to 6 wks. To improve RBC formation andreplace Fe store.

Nursing Implications when taking FeS04

1. Administer drug on an empty stomach with water to enhance absorption. Ofot 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 maskthe 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.


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MACROCYTIC(MEGALOBLASTIC)ANEMIAS

Is one in which the RBCs are

abnormally large. These cells are actually

immature erythrocytes ormegaloblasts.

Are caused by nutritional

deficiencies

Anemia of Folic AcidDeficiency

- a deficiency of folic acidcombined with Vit. Cdeficiency produces an anemia

in which the erythrocytes areabnormally large.

- There is accompanyingneutropenia andthrmbocytopenia.

- MCV and MCH are increasedwhereas mean corpusuclarHgb concentration is normal


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- Bone marrow contain

megaloblasts indicatinginhibition Of production of

erythrocytes at an early stage.

- TX:

a. Daily oral administration offolic acid.

Pernicious Anemia (vitamin B12deficiency)

- Vitamin B12 is necessary for thematuration of RBCs.

- Results from deficiency or inability to usevitamin B12 which is ofund primarily infoods of animal orgin including bothcows milk and breast milk

- For vit. B12 to be absorbed from theintestine, an intrinsic factor must bepresent

- Manifestations of intrinsic factordeficiency are:

a. Pallor

b. Anorexia

c. Irritability

d. Chronic diarrhea0

e. Tongue appear smooth and beefy red

due to papillary atrophyf. Neuropathologic findings like ataxia,

hyporeflexia, paresthesia and (+)Babinski reflex are less noticeable.


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Lab findings reveal low serum

levels of Vitamin B12.

Mgt:

a. If the anemia is due to a B12

deficient diet, temporaryinjections 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.


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Congenital Spherocytosis

- Is a hemolytic anemia that isinherited as an autosomaldominant trait.

- It occurs most frequently inthe white Northern Europeanpopulation.

- Cells are small and defective.

- The hemolysis of RBCs appears

to occur in the spleenapparently from excessiveabsorption of sodium into thecell.

Chronic jaundice andsplenomegaly develop.

Mean corpuscular Hgbconcentration is increased

Gallstones may be present inolder children and adolescent

Tx:

- Splenectomy at approximately5-6 yrs.


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Glucose 6 PhosphateDehydrgenase Deficiency (G6PD)

- The enzyme G6PD is necessaryfor maintenance of RBC life.

- Lack of the enzyme results inpremature destruction of RBC.

- It is transmitted as a sex-linkedrecessive trait.

- Occurs in 2 identifiable forms:

a. Children with congenitalnonspherocytic hemolytic anemiahave hemolysis, jaundice andsplenomegaly and may haveaplastic crises.

b. Others have drug induced forms inwhich blood patterns are normaluntil child is exposed to favabeans or drugs such asantipyretics, sulfonamides,antimalarials, and

naphthaquinolones (the mostcommon is ASA. Approximatelyafter 2 days of ingestion, childshows evidence of hemolysis.

DX:

- A blood smear will show HEINZ

bodies (oddly shaped particles inRBCs).

- Rapid enzyme screening test

- Newborn screening test


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SICKLE CELL ANEMIA

It is the presence ofabnormally shaped(elongated) RBCs.

It is an autosomal recessiveinherited disorder of the beta

chain of Hgb; the amino acidvaline takes the place of thenormally appearing glutamicacid.

Pathophysiology:

a. In Hgb S, the defect is asubstitution of valine ofrglutamine

b. Erythrocytes containing Hgb S(HbS) beocme sickled insituations of decreased O2tension and decreasedhydration.

c. Sickled RBCs are crescentshaped, have reduced O2carrying ability and decreasedlife span


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Sickled RBCs are rigid, causetrapping and increased bloodviscosity, capillary stasis andthrombosis eventually tissueischemia nad necrossis result

SICKLE CELL CRISIS- denotes a sudden, severe, onset

of sickling.

- Can occur when child has anillness causing DHN or arespiratory infection that results

to lowered O2 exchange orlowered arterial O2 level; afterextrenely strenous exercise

Diagnostic Procedures:

a. Hgb electrophoresis(fingerprinting)

- Detects homozygous andheterozygous forms of the

disease and percentages ofvarious Hgb forms

b. Sickle-turbidity test (sickledex):screening test for Hgb S

c. Blood smear: may reveal shapeof RBC to be sickled rather thannormal biconcave disk

d. Antenatal screening possiblethrough amniocentesis


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Pediatric complications

a. Delayed growth,development and onset ofpuberty

b. Impaired fertility

c. Priapism- prolonged orconstant penile erection thatis painful and infrequentlyassociated wuth sexualarousal; can result fromurinary calculi; caused bymicorcirculating obstructionand engorgement of thepenis

Mgt: a. bedrest

b. Sedation

c. administer Demerol

d. Enuresis- especially at night

Therapeutic Mgt:

a. Medications

1. Analgesics to control severe painduring crisis

2. antibiotics to treat existinginfection

b. Treatments

1. Rest

2. O2 administration

3. Fluid and electrolyte replacement

4. Blood replacementc. Nursing Mgt:

1. Assess for signs of hypoxia:irritabilty, restlessnss, agitation,hyperventilation, increased apicalpulse and RR, confusion, cyanosis

2. Monitor Iand O

3. Assess for signs of infection


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4. Provide rest periods to decreaseO2 expenditure

5. Administer blood products asordered

6. Assess location, severity,duration and quality of pain

7. Assess intensity f pain using ageappropriate pain scale

8. Administer analgesics as ordered

9. Apply heat to affected area

10. Enocurage relaxation

techniques: DBE, guided imagery11. Gently handle painful joints andextremities, provide supportwith pillows


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THALASSEMIAS

Are autosomal recessive anemiasassociated with abnormalities ofthe beta chain of adulthemoglobin (HgbA).

1. Thalassemia Minor(Heterozygous Beta-

Thalassemia)- A mild form of anemia which

produces both defective betaHgb and normal Hgb.

- RBC count will be normal butthe Hgb concentration will be

decreased 2-3g/100 ml belownormal levels.

- Blood cells are moderatelyhypochromic and microcytic.

2. Thalassemia-Major (HomozygousBeta-Thalassemia)

- Or Cooleys anemia orMediterranean Anemia

- RBCs are hypochromic andmicrocytic

- Fragmented poikilocytes andbasophilic stippling (eveness ofHgb concentration) are present

- Hgb level is


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ASSESSMENT1. Bone pain

2. Characterisitc change in the shapeof the skull (parietal and frontalbossing) and protrusion of theupper teeth with marked

malocclusion.3. Base of the nose may be broad

and flattened

4. The eyes may be slanted with anepicanthal folds

5. An x-ray of bone shows marked

osteoporotic tissue possiblyresulting in fractures

6. Hepatosplenomegaly

7. Anorexia and vomiting

8. epistaxis

9. DM due to pancreatichemosiderosis (deposition of Fe)

10. Cardiac dilatation with murmur

11. Arryhthmias and heart failure-frequent cuase of death

Therapeutic Mgt:

1. Administer diuretics, digitalis2. Proivde a low Na diet

3. Transfusion of packed RBCs every2-4 wks will maintain Hgb lbtween10 and 12 g/100 ml.

4. Administer Fe chelating agent

such as Deferoxamine to removeexcessive store of Fe (given Sqover6-8 hrs. as they sleep at night)

5. Splenectomy


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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 theunderlying cause


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DISORDERS OF THE WHITE BLOOD

CELLSDISORDER DESCRIPTION CAUSES/TREATMENT

Neutropenia Reduced no. of WBC Transient

phenomenon with

nonpyrogenic

infections such as viral

diseasePossible side effect

from some drugs such

as Phenytoin

(Dilantin),

chloramphenicol or

chlorpromazine

Treatments: possibly

WBC transfusion,

prophylactic

antibiotics


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eosinophilia Increasednumber of

eosinophils

Associted with

allergic

disorders and

with parasiticinvasion

Lymphocytosis Increased

number of

lymphocytes

Normally

occurs in the

preschool

period

Abnormally

elevated in

childhood

illnessess likepertussis,

infectious

mononucleosis

andl m hoc tic

HEMOPHILIA


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HEMOPHILIA

Inherited bleeding or coagulation, disorder.

Persons with hemophilia lack the ability to stopbleeding 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


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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 totheir daughters.


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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 haveother 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 ofthe disease. These cases are due to a new or spontaneous

development of the defective gene in the female.


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SYMPTOMS


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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


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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


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TREATMENT

Blood transfusions

Prophylactic (preventive) treatment

with infused clotting factors


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NURSING MANAGEMENT

1. Assess for signs of activebleeding, hemarthrosis

2. Administer plasma CHON,factor replacement orcryopercipitate as ordered

3. Apply pressure and coldcompresses to site of injury

4. Elevate and immobilizeaffected limb

5. Teach parents of symptomsof bleeding: pain, swelling,limited joint motion

6. Teach parents to administerplasma CHON when signs ofbleeding appar

7. Provide a soft toothbrush

8. Inspect toys for sharp edgesor parts

9. Pad crib sides

10. Avoid contact sports

11. Encourage iron-rich foods

12. Assess mobility status:jointmobility, pain, stiffness,swelling, muscle tone, abilityto perform ADLs.


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13. Assess complications

of immobility;skin

breakdown, contractures,

loss of muscle strength,

constipation14. Provide active and

passive ROM q 2-4 hrs. as

needed.

Immune Thrombocytopenic Purpura


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y p p

(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 canhappen 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


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CAUSES

Medications - including over-the-counter

Infection

Pregnancy

Immune disorders

However, about half of all cases are classified asidiopathic.

SYMPTOMS


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SYMPTOMS

Internal bleeding, which may cause: ecchymosis -

bruising , petechiae - tiny red dots on skin or mucousmembranes

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


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DIAGNOSIS


Additional blood and urine tests Other evaluation procedures

Careful review of patient's medications

Bone marrow examination


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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


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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


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HODGKIN S DISEASE

Type of lymphoma, a cancer in the lymphatic system.

Rare disease usually occurs most often in peoplebetween 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.


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STAGES OF HODGKINS DISEASE

STAGE EXTENT OF DISEASE

I Disease affects single lymph node or

single extralymphatic organ or site

II Disease affects 2 or more lymph node

regions on the same side of the

diadphragm or there is localizedinvolvement of an extralyumphatic organ

or site

III Disease affects lymph node regions on

both sides of the diaphragm or there is

localied involvement of an extralymphatic

organ or site

IV There is diffuse or disseminated

involvement of extralymphatic organs

with or without associated lymph node

involvement

Lymphadenoma

Hodgkin's Disease


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(Pseudo-leukemia of German authors)

SIGNS AND SYMPTOMS


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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


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RISK FACTORS

Past infection with infectious mononucleosis

History of infectious mononucleosis (caused by aninfection 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


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TREATMENT

Radiation therapy

Chemotherapy

LEUKEMIA


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LEUKEMIA

Cancer of the blood cells, usually the white bloodcells.

Leukemic cells look different than normal cells anddo not function properly.

TYPES OF LEUKEMIA


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TYPES OF LEUKEMIA

Lymphocytic or myelogenous leukemia

Cancer can occur in either the lymphoid or myeloidwhite 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.


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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 theirfunctions. 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


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TYPES OF LEUKEMIAS

Acute myelogenous leukemia (AML)

Chronic myelogenous leukemia (CML)

Acute lymphocytic leukemia (ALL)

Chronic lymphocytic leukemia (CLL)

SIGNS AND SYMPTOMS


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More frequent infections and fevers

Anemia and its symptoms: pale skin, fatigue, weakness,bleeding, bruising, fever, chills, loss of appetite, loss ofweight, swollen or tender lymph nodes, liver, or spleen,petechiae (tiny red spots under the skin), swollen or bleedinggums, sweating, bone or joint pain.

Acute leukemia: headaches, vomiting, confusion, loss ofmuscle control, seizures, swollen testicles, sores in the eyesor on the skin.


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Chronic leukemia may affect the skin, central nervoussystem, digestive tract, kidneys, and testicles.

The symptoms of acute and chronic leukemias mayresemble other blood disorders or medical problems

DIAGNOSIS


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Physician examination for swelling in the: liver, spleen, lymphnodes 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


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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 ofthe body caused by leukemia treatment

ACUTE LYMPHOCYTIC LEUKEMIA [ALL]


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[ ]

ALL is a cancer of the blood in which too many

lymphocytes, a type of white blood cell, areproduced by the bone marrow and by organs ofthe lymph system.

The lymphocytes fight infection by making

antibodies that attack harmful elements. But, inALL, the cells are immature and overabundant.They crowd out other blood cells, and may collectin the blood, bone marrow, and lymph tissue.

A t l k i h t i d


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Acute leukemia can occur over a short periodof days to weeks. Chromosome abnormalities(extra chromosomes and structural changes inthe chromosome material) are present in themajority of all patients.

ALL is the most common type of leukemia inyoung children. This type of leukemia may alsoaffect adults, especially those age 65 andolder.

SIGNS AND SYMPTOMS


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Anemia

Bleeding

Bruising

Fever

Persistent weakness

Fatigue

Aches in bones and joints Swollen lymph nodes

DIAGNOSIS


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Blood tests and other evaluation procedures


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


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ACUTE MYELOGENOUS LEUKEMIA [AML]


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AML cancer of the blood in which too many granulocytes areproduced in the bone marrow.

Bone marrow cells mature into several different types ofblood cells.

AML affects the young blood cells (called blasts) that developinto a type of white blood cell (called granulocytes).

Main function of granulocytes is to destroy bacteria.

Blasts, do not mature & become too numerous, remain inthe bone marrow and blood.


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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


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Anemia

Bleeding

Bruising

Fever

Persistent weakness

Fatigue

Aches in bones and joints

Swollen lymph nodes

DIAGNOSIS


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Blood tests


Spinal tap/lumbar puncture

TREATMENT

Chemotherapy

Radiation therapy


CHRONIC LYMPHOCYTIC LEUKEMIA [CLL]


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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 areimmature and over abundant. They crowd out other

blood cells, and may collect in the blood, bone marrowand lymph tissue.

CLL usually occurs in people 60 years of age or older. It isa slowly progressing disease.

SIGNS AND SYMPTOMS


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Persistent weakness

Swollen lymph nodes Enlarged spleen

Enlarged liver

Anemia

DIAGNOSIS


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Complete medical history

Physical examination

Blood tests

Bone marrow biopsy

TREATMENT

Chemotherapy

Radiation therapy

Treatment for complications infection oranemia

Leukapheresis, a procedure to remove

excessive lymphocytes


Splenectomy, surgery to remove the spleen

CHRONIC MYELOGENOUS LEUKEMIA [CML]

CML f th bl d i hi h t


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CML cancer of the blood in which too manygranulocytes, a type of white blood cell, are produced inthe bone marrow.

Bone marrow cells mature into several different types ofblood cells.

CML affects the young blood cells (called blasts) thatdevelop 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 i d f th


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CML can occur over a period of months or years.

Specific chromosome rearrangement is found inpatients with CML. Part of chromosome #9 breaks offand attaches itself to chromosome #22, so that thereis an exchange of genetic material between these twochromosomes. This rearrangement changes theposition and functions of certain genes, which resultsin uncontrolled cell growth.

Other chromosome abnormalities can also bepresent.

CML occurs mainly in adults and is rare in children.

SIGNS AND SYMPTOMS


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Anemia

Bleeding

Bruising

Fever

Persistent weakness

Fatigue

Aches in bones and joints

Swollen lymph nodes

DIAGNOSIS


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Blood tests


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


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LEUKEMIA

1. Use strict hand washingbefore and after contact.

2. Assess for side effects ofchemotherapy.

3. Administer an antiemetic 30mins. Before chemotrherapyis ordered.

4. Assess oral mucosa for pain,ulcers, lesions, stomatits, andeffects on eating

5. Assess for bleeding from any

orifice; check urine and stoolfor blood

6. Monitor platelet,WBC,Hgb,Hct count

7. assess for signs of infection

8. Provide mouth rinses and softbristled toothbrush

9. Administer topical xylocainebefore meals as ordered.

10. Provide a soft, bland diet

11. Isolate from persons withURTI.

12. Report immediately any singsof fever, pallor, oozing blood,exposure to a communicabledisorder

NON-HODGKIN'S LYMPHOMA


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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 alsospread to other organs.

Etiology is idiopathic


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SYMPTOMS


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Painless swelling of lymph nodes in neck, underarm, andgroin

Fever Night sweats

Fatigue

Weight loss

Itching of the skin

Recurring infections

RISK FACTORS


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Genetic disease of theimmune system

Unprotected exposure tostrong sunlight

High-fat, low-fiber diet

Smoking

Excessive alcoholconsumption

Environmental factorsradiation, chemicals, andinfections

Organ transplantation

Infections with HIV orHTLV-1

Infections with malaria

History of infectiousmononucleosis

Helicobacter pylori

bacterium stomachulcers

DIAGNOSIS


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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


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It is a myeloproliferative blood disorder.

It is characterized by the production of toomany platelets in the bone marrow.

Too many platelets make normal clotting of

blood difficult Etiology is idiopathic

SYMPTOMS


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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


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Complete medical history and physicalexamination

Blood counts and elevated platelet levels

Bone-marrow biopsy

TREATMENT

Chemotherapy

Plateletpheresis - a procedure to remove extraplatelets from the blood

BONE MARROW TRANSPLANTATION [BMT]


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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), filteringthose cells, and giving them back either to the patient orto another person.

The goal of BMT is to transfuse healthy bone marrowcells into a person after their own unhealthy bonemarrow has been eliminated.


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Bone marrow transplantation is not yet a

standard treatment therapy, but has been usedsuccessfully to treat diseases such as leukemias,lymphomas, aplastic anemia, immunedeficiency 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


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It is the medium for development and storage of about 95percent 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 toitself.

Differentiation - able to generate one or more subsets ofmore mature cells.

It is the stem cells that are needed in bone marrowtransplantation.

NORMAL ANATOMY


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INDICATION


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PROCEDURE


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AFTER CARE


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GOAL OF BMT


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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 forcancer, a marrow transplant may be needed.


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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


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Leukemias

Severe aplastic anemia

Lymphomas

Multiple myeloma

Immune deficiency disorders

Solid-tumor cancers like breast or ovarian


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DIFFERENT TYPES OF BMT


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Autologous bone marrow transplant

Allogeneic bone marrow transplant

A parent - a haploid-identical match is when the donor is a parentand the genetic match is at least half identical to the recipient.

An identical twin - a syngeneic transplant is an allogeneictransplant from an identical twin. Identical twins are considered acomplete genetic match for a marrow transplant.

Unrelated bone marrow transplants (UBMT or MUD for matchedunrelated donor)

Umbilical cord blood transplant

Stem Cell Transplantations [SCT]


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In bone marrow - there is 1 stem cell in every100,000 blood cells.

Breast bone, skull, hips, ribs, and spine containsthe stem cells.

Harvesting stem cells from bone marrow requires asurgical procedure.

Harvesting stem cells from the blood stream isaccomplished by a process called apheresis.

Stem Cell Transplantations [SCT]


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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 processcalled apheresis.

Care of Children With Hematological and Immunological Disorders

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