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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 is normal.

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Thalassemia Major/Cooleys

a=Anemia Homozygous Beta-thalassemia

Beta-chain hgb defect therefore Sx appear aonlyafter HgbF is replaced by HgbA after 6 mos of life

poikilocyte and basophilic stippling, high serumiron level are present

S/Sx: Anemia: pallor, irritability, anorexia

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Thalassemia Major/Cooleys

a=AnemiaBody organ/ system Effects of abnormal production

Bone marrow

Skin

Spleen

Liver and GB

Pancreas

Heart

Overstimulation leads to

increased facial-mandibular growth

Bronze-colored from jaundiceand hemosiderosis

Spleenomegaly

Cirrhosis/ cholelithiasis Destuction of Islets of

Langerhan/DM

Failure due to overload

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Thalassemia Major/Cooleys

a=Anemia Therapeutic Mgt: digitalis, diuretics, low-

sodium diet, BT of PRBC every 2-4 wks (

cosmetic facial alterationosteoporosis,cardiac dilatation will be decreased),

Deferoxamine, spleenectomy to reduce rate

of hemolysis; Bone marrow stem

transplantation