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Page 1: Hemolytic Anemia PPT

Hemolytic anemia: Sickle Cell Hemolytic anemia: Sickle Cell anemia (SSA)anemia (SSA)

Congenital hemolytic anemia resulting from a Congenital hemolytic anemia resulting from a defective Hb molecule (HbS) that causes RBCs defective Hb molecule (HbS) that causes RBCs to become sickle-shaped.to become sickle-shaped.

Eight percent of African American are Eight percent of African American are heterozygous (carriers) for SSA thereby heterozygous (carriers) for SSA thereby inheriting one affected gene or the sickle cell inheriting one affected gene or the sickle cell trait.trait.

One percent of Africa American are One percent of Africa American are homozygous (identical genes) for the disorder, homozygous (identical genes) for the disorder, thereby inheriting a defective gene form both thereby inheriting a defective gene form both parent or SSAparent or SSA

Sickle cell trait (heterozygous) is a generally Sickle cell trait (heterozygous) is a generally mild condition that produce few, if any mild condition that produce few, if any manifestationmanifestation

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Cont…Cont… SSA is caused by an autosomal defect( one gene is SSA is caused by an autosomal defect( one gene is

affected) that results in the synthesis of hemoglobin S. affected) that results in the synthesis of hemoglobin S. Produced by a mutation in the beta chain of the Produced by a mutation in the beta chain of the

hemoglobin molecule through a substitution of the hemoglobin molecule through a substitution of the amino acid valine for glutamine in both beta chainsamino acid valine for glutamine in both beta chains

During decreased oxygen tension in the plasma, the During decreased oxygen tension in the plasma, the hemoglobin S causes the RBCs to elongate, become hemoglobin S causes the RBCs to elongate, become rigid, and assume a crescent, sickled shape causing rigid, and assume a crescent, sickled shape causing the cells to clump together, obstruct capillary blood flow the cells to clump together, obstruct capillary blood flow causing ischemia and possible tissue infraction causing ischemia and possible tissue infraction

With normal oxygenation , the sickled RBCs resume With normal oxygenation , the sickled RBCs resume their normal shape; repeated episodes of sickling and their normal shape; repeated episodes of sickling and unsickling weaken the cell membrane, causing them to unsickling weaken the cell membrane, causing them to hemolyze and be removed.hemolyze and be removed.

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Sickled Cell anemia Sickled Cell anemia

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Cont..Cont..

Crisis is usually painful and can last from 4 to 6 days. Crisis is usually painful and can last from 4 to 6 days. Clients are always anemic Hb 7 to 10g/dl Clients are always anemic Hb 7 to 10g/dl Crisis can occur in different forms.Crisis can occur in different forms.3. Sickle cell crisis3. Sickle cell crisis: results from tissue hypoxia and necrosis due to : results from tissue hypoxia and necrosis due to

inadequate blood flow to a specific region or organinadequate blood flow to a specific region or organ

2. Aplastic crisis2. Aplastic crisis: results from a virus. The HB drops so rapidly that : results from a virus. The HB drops so rapidly that the bone marrow can not compensate. the bone marrow can not compensate.

3. Sequestration crisis3. Sequestration crisis: rare seen in infants ages 8 months to 2 : rare seen in infants ages 8 months to 2 years. Results when other organ pool the sickled cells. By age 10 years. Results when other organ pool the sickled cells. By age 10 children have had a splenic infarction and the spleen is no longer children have had a splenic infarction and the spleen is no longer functional.functional.

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Conditions likely to trigger a Conditions likely to trigger a sickle cell crisissickle cell crisis

hypoxiahypoxia Low environmental and or body tempLow environmental and or body temp Excessive exerciseExcessive exercise High altitudesHigh altitudes Inadequate oxygen during anesthesia Inadequate oxygen during anesthesia InfectionInfection StressStress DehydrationDehydration

..

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Assessment findings: Assessment findings:

Sickle Cell AnemiaSickle Cell Anemia Aching bonesAching bones Chronic fatigueChronic fatigue Frequent infectionFrequent infection Jaundice or pallorJaundice or pallor joint swellingjoint swelling Leg ulcers (esp ankles)Leg ulcers (esp ankles) Severe localized and Severe localized and

generalized paingeneralized pain TachycardiaTachycardia DyspneaDyspnea Priapism (Unexplained, Priapism (Unexplained,

painful erection)painful erection)

Sickle cell crisis (general Sickle cell crisis (general S&S)S&S) HematuriaHematuria IrritabilityIrritability LethargyLethargy Pale lips, tongue, and nail Pale lips, tongue, and nail

bedsbeds Sever painSever pain

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Assessment findings contAssessment findings cont

Painful crisisPainful crisis Dark crisisDark crisis Low grade feverLow grade fever Severe abdominal, thoracic, muscle or bone Severe abdominal, thoracic, muscle or bone

painpain Tissue anoxia and necrosisTissue anoxia and necrosis Worsening jaundice Worsening jaundice

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Assessment findings contAssessment findings cont

Aplastic anemiaAplastic anemia DyspneaDyspnea Lethargy and Lethargy and

sleepinesssleepiness Marked decreased in Marked decreased in

bone marrow activitybone marrow activity PallorPallor Possible comaPossible coma RBC hemolysisRBC hemolysis

Acute sequestrationAcute sequestration Hypovolemic shockHypovolemic shock LethargyLethargy Liver congestionLiver congestion PallorPallor Worsened chronic Worsened chronic

jaundicejaundice

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Diagnostic and lab testDiagnostic and lab test

Hematologic studies: RBC, WBC Hematologic studies: RBC, WBC plateletsplatelets

Hb electrophoresis:Hb electrophoresis: Hb levels:Hb levels: Lateral CXR:Lateral CXR:

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Medical management Medical management

Activity:Activity: Nutritional supplements:Nutritional supplements: MonitoringMonitoring Labs studies:Labs studies: IV therapyIV therapy Treatments:Treatments: Blood transfusionsBlood transfusions Analgesics:Analgesics: Analgescis and antipyretic:Analgescis and antipyretic: Antineoplastic:Antineoplastic: Oxygen therapyOxygen therapy Position for comfortPosition for comfort Antibiotics:Antibiotics: Genetic counselingGenetic counseling

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Nursing managementNursing management

Activity:Activity: Nutritional supplements:Nutritional supplements: MonitoringMonitoring Labs studies:Labs studies: IV therapyIV therapy Treatments:Treatments: Blood transfusionsBlood transfusions Meds Meds Oxygen therapyOxygen therapy Position for comfortPosition for comfort Antibiotics:Antibiotics: educationeducation

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Complications Complications

RetinopathyRetinopathy NephropathyNephropathy Cerebral vessel occlusionsCerebral vessel occlusions Hypovolemic shockHypovolemic shock NecrosisNecrosis infection and gangreneinfection and gangrene Death Death

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Nursing diagnosisNursing diagnosis

Acute PainAcute Pain Ineffective tissue perfusionIneffective tissue perfusion Impaired gas exchangeImpaired gas exchange Risk for infectionRisk for infection Deficient knowledgeDeficient knowledge powerlessnesspowerlessness

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Evaluation Evaluation

Client verbalize pain reliefClient verbalize pain relief Client does not develop an infectionClient does not develop an infection Client adheres t vaccination scheduleClient adheres t vaccination schedule Client identifies precipitation factors Client identifies precipitation factors

leading to crisis stateleading to crisis state

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PolycythemiaPolycythemia

Refers to an Refers to an ↑ circulating RBCs and ↑ circulating RBCs and concentration of HB in the bloodconcentration of HB in the blood

It is a term used when the HCT is > 55% It is a term used when the HCT is > 55% in males and > 50% in females.in males and > 50% in females.

Also known as Also known as Polycythemia Vera, PV, or Polycythemia Vera, PV, or myeloproliferative red cell disorder; it can myeloproliferative red cell disorder; it can be primary or secondary. be primary or secondary.

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Polycythemia: PrimaryPolycythemia: Primary

Common in men of European Jewish Common in men of European Jewish Neoplastic stem cell disorder characterized by Neoplastic stem cell disorder characterized by

↑ production of RBC, granulocytes and ↑ production of RBC, granulocytes and plateletsplatelets

With the overproduction of RBC, increased With the overproduction of RBC, increased viscosity results in congestion of blood in viscosity results in congestion of blood in tissues, the liver and spleentissues, the liver and spleen

Thrombi form, acidosis develops, and tissue Thrombi form, acidosis develops, and tissue infarction occurs as a result of the diminished infarction occurs as a result of the diminished circulatory flow of blood caused by the circulatory flow of blood caused by the increase viscosityincrease viscosity

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Polycythemia: SecondaryPolycythemia: Secondary

Most common formMost common form The disturbance is not in the development of RBC but in The disturbance is not in the development of RBC but in

the abnormal increase of erythropoietin, causing the abnormal increase of erythropoietin, causing excessive erythropoiesis excessive erythropoiesis

The increase in RBC production caused by increased The increase in RBC production caused by increased erythropoietin release is a physiologic response to erythropoietin release is a physiologic response to hypoxia; hypoxia stimulates the release of erythropoietin hypoxia; hypoxia stimulates the release of erythropoietin in the kidneyin the kidney

Chronic hypoxia states may be produced by prolonged Chronic hypoxia states may be produced by prolonged exposure to high altitudes, pulmonary diseases, exposure to high altitudes, pulmonary diseases, hypoventilation and smokinghypoventilation and smoking

The results of an increased RBC production include The results of an increased RBC production include increased viscosity of blood, which alters circulatory flowincreased viscosity of blood, which alters circulatory flow

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Assessment Findings Assessment Findings

Ruddy completionRuddy completion Dusky mucosaDusky mucosa VertigoVertigo HeadachesHeadaches DizzinessDizziness Dyspnea and OrthopneaDyspnea and Orthopnea TachycardiaTachycardia EcchymosisEcchymosis Hepatomegaly and splenomegalyHepatomegaly and splenomegaly Increased gastric secretionIncreased gastric secretion Weakness and fatigueWeakness and fatigue PruritusPruritus ExpistaxisExpistaxis GI bleedingGI bleeding Angina Angina

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Diagnostic findingDiagnostic finding

↑ ↑ hemoglobin and erythrocyte counthemoglobin and erythrocyte count ↑↑WBCWBC ↑ ↑ plateletsplatelets ↑↑uric aciduric acid ↑↑histaminehistamine

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Therapeutic management Therapeutic management

Management of the underlying condition Management of the underlying condition causing the hypoxiacausing the hypoxia

Repeated phlebotomy to decrease blood Repeated phlebotomy to decrease blood volume; the goal is to keep the HCT less volume; the goal is to keep the HCT less that 45 to 48%that 45 to 48%

Hydration to decrease blood viscosityHydration to decrease blood viscosity

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Planning and ImplementationPlanning and Implementation

Assist in phlebotomyAssist in phlebotomy Measures to relieve pruritus including Measures to relieve pruritus including

cool and tepid bathcool and tepid bath Accurate monitor of Intake and outputAccurate monitor of Intake and output Nursing measures to prevent thrombotic Nursing measures to prevent thrombotic

events.events. Administration of medication to prevent Administration of medication to prevent

complications complications

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Medication therapyMedication therapy

Mylosuppressant agent to inhibit bone Mylosuppressant agent to inhibit bone marrow activity( hydroyurea, melphalan, marrow activity( hydroyurea, melphalan, redioactive phosphorus)redioactive phosphorus)

Allopurinal and colchicineAllopurinal and colchicine Antiplatelet agents Antiplatelet agents AnalgesicAnalgesic Histamine antagonistsHistamine antagonists Antihistamine Antihistamine

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Client education Client education

Good hydration about 3L/dayGood hydration about 3L/day Disease processDisease process Signs and symptom of complication Signs and symptom of complication Prevention of bleeding Prevention of bleeding Emphasize the importance of check upEmphasize the importance of check up Teach client to avoid products that Teach client to avoid products that

contain ironcontain iron Discuss ways to prevent thrombosisDiscuss ways to prevent thrombosis

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Evaluation Evaluation

The HCT is within normal rangeThe HCT is within normal range The client does not develop complication The client does not develop complication

associated wit thrombusassociated wit thrombus The clients maintain adequate hydrationThe clients maintain adequate hydration

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Neutropenia Neutropenia

Leukopenia is condition of fewer WBCs than normal, Leukopenia is condition of fewer WBCs than normal, results from neutropenia or lymphopeniaresults from neutropenia or lymphopenia

Neutropenia results from decreased production or Neutropenia results from decreased production or increased destruction of neutrophils < 2,000/mmincreased destruction of neutrophils < 2,000/mm³³

Is not a disease but a syndromeIs not a disease but a syndrome It may occur as a primary Hematologic disorder but It may occur as a primary Hematologic disorder but

may also be caused by drugs, infections, an other may also be caused by drugs, infections, an other medical condition such as sepsis and nutritional medical condition such as sepsis and nutritional deficienciesdeficiencies

If the leukocyte count is decreased, or if immature If the leukocyte count is decreased, or if immature WBC predominate in the circulation, the phagocytic WBC predominate in the circulation, the phagocytic function of these cells is impairedfunction of these cells is impaired→ susceptibility of → susceptibility of infectionsinfections

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AssessmentAssessment

There is no real symptoms associated There is no real symptoms associated with Neutropenia until the patient with Neutropenia until the patient becomes infected. becomes infected.

Routine CBC with diff such as those Routine CBC with diff such as those obtain after chemotherapy can be reveal obtain after chemotherapy can be reveal before the onset of infectionbefore the onset of infection

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Therapeutic managementTherapeutic management

Treatment depends on its causeTreatment depends on its cause If the etiology is drug induced, D/C meds If the etiology is drug induced, D/C meds

if possibleif possible Corticosteroids are used if the etiology is Corticosteroids are used if the etiology is

immunologic immunologic If client develops a fever, identification If client develops a fever, identification

and treatment of he infections is and treatment of he infections is instituted instituted

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Planning andPlanning and ImplementationImplementation

Monitor for signs of infectionMonitor for signs of infection Obtain culturesObtain cultures Administer antibioticsAdminister antibiotics Administer meds that stimulate the production Administer meds that stimulate the production

of neutrophils( neupogen or Leukine)of neutrophils( neupogen or Leukine) Enforce strict hand washing by all individuals in Enforce strict hand washing by all individuals in

contact with the clientcontact with the client Institute reverse isolationInstitute reverse isolation Avoid invasive proceduresAvoid invasive procedures Neutropenia precautions Neutropenia precautions

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Client educationClient education

Teach client and family to report signs Teach client and family to report signs and symptomsand symptoms

Teach strict hand washingTeach strict hand washing Teach clients methods to maintain good Teach clients methods to maintain good

personal hygienepersonal hygiene Explain condition and the rationale for Explain condition and the rationale for

therapeutic interventiontherapeutic intervention

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Evaluation Evaluation

Neutrophil count is normalizedNeutrophil count is normalized The client is free of infectionThe client is free of infection The client and family verbalize methods The client and family verbalize methods

of limiting exposure to pathogens of limiting exposure to pathogens

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Lymphomas: Hodgkin's diseaseLymphomas: Hodgkin's disease

Lymphomas is a group if malignant Lymphomas is a group if malignant neoplasms that affects the lymphatics neoplasms that affects the lymphatics system resulting in the proliferation of system resulting in the proliferation of lymphocytes; lymphomas can be lymphocytes; lymphomas can be classified as Hodgkin's and non classified as Hodgkin's and non Hodgkin's lymphomas Hodgkin's lymphomas

Neoplastic cells of lymphoid origin Neoplastic cells of lymphoid origin

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Hodgkin’s diseaseHodgkin’s disease

More common in men and has two peaks;15 -35yrs of More common in men and has two peaks;15 -35yrs of age and 55-75yrs; incidence is higher in whites than in age and 55-75yrs; incidence is higher in whites than in African AmericanAfrican American

The cause of the disease is unknown although several The cause of the disease is unknown although several factors include infection with Epstein-Barr virus, familial factors include infection with Epstein-Barr virus, familial pattern, and exposure to toxinspattern, and exposure to toxins

Is characterized by the presence of Reed-Sternberg Is characterized by the presence of Reed-Sternberg cells that proliferate in a single lymph and travel cells that proliferate in a single lymph and travel continuously through the lymphatic to other lymph continuously through the lymphatic to other lymph nodes and organsnodes and organs

The tumor originates in a lymph node (in majority of The tumor originates in a lymph node (in majority of cases from the cervical nodes) and infiltrates the cases from the cervical nodes) and infiltrates the spleen, lungs and liverspleen, lungs and liver

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Non-Hodgkins lymphomaNon-Hodgkins lymphoma Most common form of lymphoma affects usually adults Most common form of lymphoma affects usually adults

from 50 to 70 years old. It is more common in men than from 50 to 70 years old. It is more common in men than women and in whiteswomen and in whites

There is no know cause but the incidence of non-There is no know cause but the incidence of non-Hodgkin’s lymphomas is linked to viral infections, Hodgkin’s lymphomas is linked to viral infections, immune disorders, genetic abnormalities, exposure to immune disorders, genetic abnormalities, exposure to chemicals, and infection with chemicals, and infection with Helicobacter pyloriHelicobacter pylori

Non-Hodgkin's lymphomas has a similar patho to Non-Hodgkin's lymphomas has a similar patho to Hodgkins disease, also Reed-Sternberg cells are Hodgkins disease, also Reed-Sternberg cells are absent and the method of lymph node infiltration is absent and the method of lymph node infiltration is differentdifferent

In the majority of cases, the disease involves malignant In the majority of cases, the disease involves malignant B cells; the lymphoma usually originates outside the B cells; the lymphoma usually originates outside the lymph nodes; the lymphoid tissues involved become lymph nodes; the lymphoid tissues involved become infiltrated with malignant cells; the cells that make up infiltrated with malignant cells; the cells that make up the lymphoid tissue become abnormal and crowd out the lymphoid tissue become abnormal and crowd out normal cells. normal cells.

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Clinical manifestation Clinical manifestation Hodgkin’s diseaseHodgkin’s disease

Usually begins with a firm and Usually begins with a firm and painless enlargement of one or more painless enlargement of one or more lymph nodes on one side of the necklymph nodes on one side of the neck

FatigueFatigue WeaknessWeakness AnorexiaAnorexia DysphagiaDysphagia DyspneaDyspnea PruritusPruritus CoughCough JaundiceJaundice AbnormalAbnormal Bone pain Bone pain Development of severe but brief pain Development of severe but brief pain

at the site of Hodgkin's after ingestion at the site of Hodgkin's after ingestion of alcoholof alcohol

Enlarged lymph nodes, liver and Enlarged lymph nodes, liver and spleenspleen

B symptoms: fever without chills, B symptoms: fever without chills, night sweats, weight loss night sweats, weight loss unintentionalunintentional

Non-HodgkinsNon-Hodgkins Painless lymph node enlargementPainless lymph node enlargement B symptomsB symptoms Abdominal pain, nausea, vomitingAbdominal pain, nausea, vomiting HematuriaHematuria Peripheral neuropathy, cranial Peripheral neuropathy, cranial

nerves, HA, visual disturbances, nerves, HA, visual disturbances, changes in mental status and changes in mental status and seizures.seizures.

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Diagnostic findings Diagnostic findings

Hodgkin’s diseaseHodgkin’s disease Normocytic, Normocytic,

normochromic anemianormochromic anemia Presence of Reed-Presence of Reed-

Sternberg cells in bone BxSternberg cells in bone Bx Mediastinal Mediastinal

lymphadenopathy lymphadenopathy revealed by CXR, CT revealed by CXR, CT scan scan

Mediastinal mass and Mediastinal mass and pulmonary infiltrates on pulmonary infiltrates on CXRCXR

Non-Hodgkins lymphoma Non-Hodgkins lymphoma Lymphocytopenia Lymphocytopenia X-ray ma reveal pulmonary X-ray ma reveal pulmonary

infiltratesinfiltrates Lymph node Bx help to Lymph node Bx help to

identify the cell type and identify the cell type and patternpattern

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Therapeutic management Therapeutic management

Hodgkin’s diseaseHodgkin’s disease LymphangiographyLymphangiography Staging laparotomyStaging laparotomy Radiation therapyRadiation therapy ChemotherapyChemotherapy

Non-hodgkin’s Non-hodgkin’s Staging of the disease Staging of the disease

from CT scan and bone from CT scan and bone marrowmarrow

Combination Combination chemotherapychemotherapy

Radiation therapyRadiation therapy Interferon, interleukins Interferon, interleukins

and tumor necrosis and tumor necrosis factorfactor

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Planning and Planning and intervention intervention

Assist in balancing activity with rest periodsAssist in balancing activity with rest periods Maintain good nutritional stateMaintain good nutritional state Provide measures to decrease the discomfort Provide measures to decrease the discomfort

associated with pruritusassociated with pruritus Institute intervention for clients on Institute intervention for clients on

chemotherapy or radiationchemotherapy or radiation Provide intervention to enable client to deal Provide intervention to enable client to deal

with body images changes with body images changes refer to cancer society refer to cancer society

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Client educationClient education

discuss with client and family the nature discuss with client and family the nature of he disease, the course of the therapyof he disease, the course of the therapy

Teach about the medication prescribedTeach about the medication prescribed Teach about signs and symptoms that Teach about signs and symptoms that

require immediate medical intervention require immediate medical intervention

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Evaluation Evaluation

The client does not develop complication The client does not develop complication of bleeding of infectionof bleeding of infection

The client regains normal weightThe client regains normal weight The client verbalized absence of painThe client verbalized absence of pain The client and family verbalize The client and family verbalize

understanding of reasons of ongoing understanding of reasons of ongoing treatment and interventions. treatment and interventions.

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Reading Reading

Thalassemia Thalassemia ThrombocytopeniaThrombocytopenia DICDIC Leukemia Leukemia


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