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    DISORDERS OF THE RED BLOODCELLS

    Anemias

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    ANEMIA

    A reduction in the quantity of the oxygen-

    carrying pigment .Hemoglobin in the blood

    Classification1. Macrocytic anemias

    2. Hypochromic

    3. Microcytic anemias4. Normocytic anemias

    5. Hemolytic anemia

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    Normocytic Anemia

    Marked by impaired production of

    erythrocytes by the bone marrow or

    by abnormal or uncompensated lossof circulating RBCs

    The remaining RBCs are normal in

    color and size, but too few in numbers

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    Normocytic Anemia

    1. Acute bld loss anemia

    Bld loss sufficient to cause anemia: internal

    bleeding, nephritis, disorders of the placenta,trauma to the cord

    S/Sx: Reticulocyte ct; Children- Shock,tachypnea; NB- gasping respiration, sternal

    retractions, cyanosis Therapeutic Mgt: control of bleeding, place on

    supine position, keep warm and dry, bloodtransfusion, blood expander (PNSS os LRS)

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    Normocytic Anemia

    2. Anemia of acute

    infection/inflamation

    May lead to increased destruction

    of erythrocytes; osteomyelitis,

    ulcerative colitis, renal disease

    Therapeutic mgt: Treatment of

    underlying condition

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    Normocytic Anemia

    3. Anemia of renal disease

    Causes loss of function of kidney cells an

    decrease in erythropoietin production

    Mgt: Administration of Recombinant HumanErythropoietin, treatment of underlying cause

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    Normocytic Anemia

    4. Anemia of Neoplastic diseas

    Malignant growths such as leukemia,

    lymphosarcoma May have accompanying blood loss if platelet

    formation is decreased

    Mgt: remission of neoplastic process, blood

    transfusion

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    Normocytic Anemia

    5. Aplastic anemia

    Result from depression of hematopoiesis in the

    bone marrow, formation and development of bldcells are affected. Occurs bet. 6-8 mos

    May be (1) Congenital(Fanconies syndrome)

    Autosomal recessive trait, child is born with number

    of congenital anomalies( skeletal, renal anomalies;hypogenitalism, short stature), between 4-12 y/ochild begin to mannifest symptoms ofPANCYTOPENIA

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    Normocytic Anemia

    (2) Acquired- excessive exposure to radiation, drugs,chemicals that damage the bone marrow.

    Drugs: chloramphenicol, sulfonamides, arsenic,hydantoin, benzene, quinine, chomotherapeutic

    agents

    S/Sx: Pale, easy fatigability, anorexia, petechiae, prone

    to infection, cardiac decompensation,

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    Normocytic Anemia

    APLASTIC ANEMIA

    Therapeutic mgt: BMT,

    administration of Antithymocyte

    globulin(ATG), cyclosporin;

    testosterone- for RBC growth, BT

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    Normocytic Anemia

    6. Hypoplastic anemia

    Results from depression of hematopoietic activity,

    on;y RBCs are affected Types: (1) congenital (Blackfan-Diamond

    syndrome)

    Tx: Long term transfusion of PRBC- causes

    HEMOSIDEROSIS- treated withHYPODERMOCLYSIS- SQ infusion of deforaxamine(Desferal), binds with iron and aids excretion in urine

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    Hypoplastic anemia

    (2) Acquiredreduction of RBC is transient so

    no therapy is neededIncreases erythropoiesis with

    corticosteroid therapy

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    Normocytic Anemia

    7. Hypersplenism

    Cells are filtered slowly therefore destroying cells

    in the process Underlying spleenic condition causes the

    syndrome

    Mgt: Spleenectomy, done after 2 years with

    immunization against pneumococci an H.Influenzae + penicillin

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    Hypochromic Anemia

    Results from inadequate hemoglobin

    synthesis, erythrocytes appear

    pale(hypochromia), reduced diameter(microcytic), occurs bet. Ages 9 mos-3 yrs

    1. Iron-deficiency anemia

    Most common anemia in infancy and chldhood

    Occur when intake of iron is inadequate,preventing proper hemoglobin formation

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    Iron-deficiency anemia

    Causes: infants- born to iron deficient mother,GIT structural defects(chalasia, pyloric stenosis);children/>2yrs- GIT lesions, polyps, ulcarative

    colitis Prevention: daily intake of 6-15 mg iron, iron

    supplementation at about 2 mos of age

    S/Sx: pale mucous membrane, poor muscle tone,

    decreased activity, possible enlargedheart/spleen, spoon-shaped fingernails; fearful,less active, less persistent( CNS maturation isaffected)

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    Iron-deficiency anemia

    Therapeutic mgt: treatment of underlying

    cause,iron-fortified formula for 1 yr, iron-rich

    diet with extra vit. C

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    Macrocytic Anemia

    A.K.A. Megaloblastic anemia

    Caused by nutritionl deficiency, characterized by

    abnormally large immature erythrocyte ormagaloblast

    1. Anemia of Folic Acid deficiency

    Due to deficient folic acid and vit.C in diet

    2. Pernicious Anemia (vit. B12 deficiency) Deficient or inability to use vit. B12, appars in teh

    first 2 years of life

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    Pernicious Anemia

    S/Sx- pale, anorexic; irritable; smooth,

    beefy-red tongue

    Mgt: Lifelong monhly intramuscularinjection of vit. B12

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    Hemolytic Anemia

    Anemia in which there is increased

    destruction of erythrocyte may be caused by

    abnormalities of erythrocyte structure orextracellular destruction forces

    1. Glucose-6-Phosphate Dehydrogenase Deficiency

    Lack of the enzyme results in prematuredestruction of RBC if they are exposed toantioxidant such as acetylsalicylic acid

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    GLUCOSE-6-PHOSPHATE

    DEHYDROGENASE DEFICIENCY

    Transmitted as sex-linked recessive trait

    Occur frequently in african-american,Asian, Sephardic jewish and

    mediterranian descent

    Occurs in two identifiable forms

    1. Nonspherocytic hemolytic anemia Hemolysis, jaundice,

    spleenomegaly, aplastic crisis

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    GLUCOSE-6-PHOSPHATE

    DEHYDROGENASE DEFICIENCY2. Drug induced bld patterns are normal until exposed to avabeans, anttipyretics, sulfonamides ,

    antimalarials, acetylsalicylic acid(mostcommon)2 days after ingestion of antioxidant, childbegins to show signs of hemolysisBlood smear- Heinz bodies( oddly shaped

    RBC)Fever and back painSelf-limitting

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    GLUCOSE-6-PHOSPHATE

    DEHYDROGENASE DEFICIENCY

    diagnosed by rapid enzyme screening

    test or electrophoretic analysis of RBCs.

    Mgt: parent teaching regarding thechilds metabolism

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    Sickle-cell Anemia

    The presence of elongated and crescent-

    shaped (sickled) RBCs- when submitted to

    low oxygen tension Autosomal recessive inherited disorder on

    the beta chain of hemoglobin

    Fetal hemoglobin contains gamma not betachain-symptoms are not evident until childs

    hgb changes from fetal to adult form (6 mos)

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    Sickle-cell Anemia

    Can be diagnosed by chorionic villi sampling

    or from cord bld during amniocentesis

    can be identified at birth by NBS Occurs almost exclusively among african-

    american

    S

    ickle cell disease- homozygous Sickle cell trait- heterozygous

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    Sickle-cell Anemia

    Assessment: Hemoglobin electrophoresis-

    use to diagnosed sickle-cell anemia

    S/Sx: fever; anemia, Pale, easy fatigability,anorexia; hand-foot syndrome( swelling dueto infarction); protruding abdomen; (adult)

    spleen atrophy due to repeated infarction and

    atrophy- infection prone; chest syndrome(similar to pneumonia); icteric sclerae;decreased vision; priapism

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    Sickle-cell Anemia

    Sickle-cell crisis- a sudden, severe onset of

    sickling which occur when child has an illness

    causing dehydration and respiratoryinfection leading to lowered O2 exchange

    Sx: fever, icteric sclerae, acute abdominal

    pain(spleenomegally, hepatomegally)

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    Sickle-cell Anemia

    Therapeutic mgt: chld with SCC has 3 primary

    needs: pain relief; adequate hydration;

    oxygenation Hydroxyurea- antineoplastic agent, may increse

    production of hemoglobin F

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    THALASsEMIAS

    Autosomal recessive trait associated with

    abnormalities of the beta chain of the adult

    Hemoglobin (HgbA). Occur mostly onMediterranean population, but may affect

    African and Asian traits

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    Thalassemia Minor

    Heterozygous Beta-thalassemia

    Produces both normal and abnormal hgb, rbc

    count will be normal but hemoglobinconcentration is decreased

    Cell are moderately hypochromic and microcyticdue to poor hgb formation

    Pt. Shows no symptom other than pallor andrequires no treatment, life expectancy i