Anemias

DISORDERS OF THE RED BLOOD

CELLS
ANEMIA

 A reduction in the quantity of the oxygen-

carrying pigment .Hemoglobin in the blood
 Classification
1. Macrocytic anemias
2. Hypochromic
3. Microcytic anemias
4. Normocytic anemias
5. Hemolytic anemia
Normocytic Anemia

 Marked by impaired production of

erythrocytes by the bone marrow or
by abnormal or uncompensated loss
of circulating RBCs
 The remaining RBCs are normal in
color and size, but too few in numbers
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, blood
transfusion, blood expander (PNSS os LRS)
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
Normocytic Anemia
3. Anemia of renal disease
 Causes loss of function of kidney cells an
decrease in erythropoietin production

 Mgt: Administration of Recombinant Human

Erythropoietin, treatment of underlying cause
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
Normocytic Anemia
5. Aplastic anemia
 Result from depression of hematopoiesis in the
bone marrow, formation and development of bld
cells are affected. Occurs bet. 6-8 mos
 May be (1) Congenital(Fanconie’s syndrome)
 Autosomal recessive trait, child is born with number
of congenital anomalies( skeletal, renal anomalies;
hypogenitalism, short stature), between 4-12 y/o
child begin to mannifest symptoms of
PANCYTOPENIA
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,
Normocytic Anemia

 APLASTIC ANEMIA
 Therapeutic mgt: BMT,
administration of Antithymocyte
globulin(ATG), cyclosporin;
testosterone- for RBC growth, BT
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 with
HYPODERMOCLYSIS- SQ infusion of deforaxamine
(Desferal), binds with iron and aids excretion in urine
Hypoplastic anemia

 (2) Acquiredreduction of RBC is transient so

no therapy is needed
 Increases erythropoiesis with
corticosteroid therapy
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
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
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 enlarged heart/spleen,
spoon-shaped fingernails; fearful, less active, less
persistent( CNS maturation is affected)
Iron-deficiency anemia

 Therapeutic mgt: treatment of underlying

cause,iron-fortified formula for 1 yr, iron-rich
diet with extra vit. C
Macrocytic Anemia

 A.K.A. Megaloblastic anemia

 Caused by nutritionl deficiency, characterized by
abnormally large immature erythrocyte or
magaloblast
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
Pernicious Anemia

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

beefy-red tongue
• Mgt: Lifelong monhly intramuscular
injection of vit. B12
Hemolytic Anemia

 Anemia in which there is increased

destruction of erythrocyte may be caused by
abnormalities of erythrocyte structure or
extracellular destruction forces

1. Glucose-6-Phosphate Dehydrogenase Deficiency

 Lack of the enzyme results in premature
destruction of RBC if they are exposed to
antioxidant such as acetylsalicylic acid
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
GLUCOSE-6-PHOSPHATE
DEHYDROGENASE DEFICIENCY
2. Drug induced
• bld patterns are normal until exposed to ava
beans, anttipyretics, sulfonamides ,
antimalarials, acetylsalicylic acid(most
common)
• 2 days after ingestion of antioxidant, child
begins to show signs of hemolysis
• Blood smear- Heinz bodies( oddly shaped
RBC)
• Fever and back pain
• Self-limitting
GLUCOSE-6-PHOSPHATE
DEHYDROGENASE DEFICIENCY

• diagnosed by rapid enzyme screening

test or electrophoretic analysis of RBCs.

• Mgt: parent teaching regarding the

child’s metabolism
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 beta
chain-symptoms are not evident until child’s
hgb changes from fetal to adult form (6 mos)
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
 Sickle cell disease- homozygous
 Sickle cell trait- heterozygous
Sickle-cell Anemia

 Assessment: Hemoglobin electrophoresis-

use to diagnosed sickle-cell anemia
 S/Sx: fever; anemia, Pale, easy fatigability,
anorexia; hand-foot syndrome( swelling due
to infarction); protruding abdomen; (adult)
spleen atrophy due to repeated infarction and
atrophy- infection prone; chest syndrome
(similar to pneumonia); icteric sclerae;
decreased vision; priapism
Sickle-cell Anemia

 Sickle-cell crisis- a sudden, severe onset of

sickling which occur when child has an illness
causing dehydration and respiratory
infection leading to lowered O2 exchange
 Sx: fever, icteric sclerae, acute abdominal
pain(spleenomegally, hepatomegally)
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
THALASsEMIAS

 Autosomal recessive trait associated with

abnormalities of the beta chain of the adult
Hemoglobin (HgbA). Occur mostly on
Mediterranean population, but may affect
African and Asian traits
Thalassemia Minor

 Heterozygous Beta-thalassemia
 Produces both normal and abnormal hgb, rbc
count will be normal but hemoglobin
concentration is decreased
 Cell are moderately hypochromic and microcytic
due to poor hgb formation
 Pt. Shows no symptom other than pallor and
requires no treatment, life expectancy is normal.
Thalassemia Major/Cooley’s
a=Anemia
 Homozygous Beta-thalassemia

 Beta-chain hgb defect therefore Sx appear aonly

after HgbF is replaced by HgbA after 6 mos of life
 poikilocyte and basophilic stippling, high serum
iron level are present
 S/Sx: Anemia: pallor, irritability, anorexia
Thalassemia Major/Cooley’s a=Anemia

Body organ/ system Effects of abnormal production

 Bone marrow  Overstimulation leads to
increased facial-mandibular
growth
 Skin  Bronze-colored from jaundice
and hemosiderosis
 Spleen  Spleenomegaly
 Liver and GB  Cirrhosis/ cholelithiasis
 Pancreas  Destuction of Islets of
Langerhan/DM
 Heart  Failure due to overload
Thalassemia Major/Cooley’s
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