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
