Hemoglobin

The component of RBCs that allows them to carry out the transport of oxygen is hemoglobin, composed
of globin, a protein, and heme, an iron-containing pigment. It is the heme portion that combines with
oxygen and carbon dioxide for transport.

Hemoglobin levels are highest at birth (13.7 to 20.1 g/100 ml)

they reach a low at approximately 3 months of ago (9.5 to 14.5 g/100 ml)

then gradually rise again until adult values are reached at puberty (11 to 16 g/100 ml).

Bilirubin

Iron is reused by the bone marrow to construct new RBCs.

Indirect bilirubin is fat-soluble and so cannot be excreted by the kidneys. It is therefore converted by the
liver enzyme glucuronyl transferase into direct bilirubin, which is water-soluble and excreted in bile.

In the newborn, generally, liver function is so immature that the conversion from indirect to direct
bilirubin is difficult, allowing a portion of bilirubin to remain in the indirect form.

If excessive hemolysis (destruction) of RBCs occurs from other than usual causes, a child will also show
signs of jaundice.

Leukocyte

Leukocytes (WBCs) are nucleated cells and few in number compared with RBCs (there is approximately
only 1 WBC to every 500 RBCs). Their primary function is defense against antigen invasion

Thrombocyte

Thrombocytes are round, nonnucleated bodies formed by the bone marrow; their function is capillary
hemostasis and primary coagulation.

megakaryocytes - Immature thrombocytes

ASSESSMENT:

History

Chief concern: Fatigue, easy bruising, epistaxis.

Past medical history: Low birthweight; blood loss at birth; lack of vitamin K administration at birth.

Nutrition: "Picky eater" or presence of pica. Increased milk intake.

Past illnesses: History of recent illness; history of recent medicine ingestion.

Family history: Inherited blood disorder; parents known to have sickle-cell trait, thalassemia minor, or
hemophilia in family.

Physical examination

General appearance: Obese infant, Fatigue

Eyes: Retinal hemorrhage

Face: Bossing of maxillary bone

Mouth: Pale mucous membrane, Ecchymotic or bleeding gumline

Heart: Increased rate, possible murmur

Skin: Petechiae, ecchymosis, Blood oozing from wound or injection point, Jaundice, Pallor, Bronze color

Abdomen: Pain on palpation, Increased liver or spleen size

Genitourinary: Delayed secondary sex characteristics

Extremities: Spoon-shaped nails, Joint swelling, pain

Neurologic: Weak muscle tone

TREATMENT:

BONE MARROW ASPIRATION AND BIOPSY

provides samples of bone marrow so the type and quantity of cells being produced can be determined

children: iliac crests or spines

adults: sternum

neonates: anterior tibia

When the marrow cavity is reached, the stylus is removed, a syringe is attached to the needle, and bone
marrow is aspirated (which appears as thick blood in the syringe)

Monitor pulse and BP and observe dressing for 15 minutes every hour.

BLOOD TRANSFUSION

Transfusions of blood or its products are commonly used in the treatment of blood disorders, and may
blood, packed RBCs, washed RBCs (as much “foreign” matter is removed as possible to reduce the
possibility of an antagonistic reaction), plasma, plasma factors, platelets, WBCs, and albumin.

Packed RBCs are the most common form of transfusion used with children because they help minimize
the risk of fluid overload

Before any transfusion, ensure a signed consent form is obtained that respects sociocultural or religious
beliefs.

Obtain vital signs to establish a baseline and monitor these about every 15 minutes during the first hour
and about every half hour for the remainder of the transfusion.

Keep the infusion rate slow for the first 15 minutes; then, if no reaction occurs, increase the rate to
about 10 ml/kg/hr or as otherwise prescribed.
HEMATOPOIETIC STEM CELL TRANSPLANTATION

Stem cell transplantation is the intravenous infusion of hematopoietic stem cells from bone marrow
obtained by marrow aspiration or from peripheral or umbilical cord blood drawn from a compatible
donor to reestablish marrow function in a child with deficient or nonfunctioning bone marrow.

Allogeneic transplantation is the transfer of stem cells from an immune-compatible (histocompatible)

donor, usually a sibling, or from a national cord blood bank or national volunteer donor registry

Syngeneic transplantation (which is rare) involves a donor and recipient who are genetically identical
(i.e., identical twins).

Autologous transplantation involves use of the child's own stem cells removed from cord blood banked
at the time of the child's birth.

Graft-versus-host disease (GVHD) is a potentially lethal immunologic response of donor T cells to the
tissue of the bone marrow recipient.

Careful tissue typing, intravenous administration of a corticosteroid and an immunosuppressant before

transplant, and irradiation of blood products (which helps to inactivate mature T lymphocytes) before
the infusion all can help reduce the incidence of this complication.

SPLENECTOMY

One of the purposes of the spleen is to remove damaged or aged blood cells.

it can be performed by laparoscopy so, although still a procedure with risks, it does not require as long a
recovery period.

A second-function of the spleen is to strain blood particles that might lead to blood clots, as well as
invading microorganisms, from the blood plasma so phagocytes and lymphocytes can destroy them.

Children are more susceptible to pneumococcal infections and other bacterial infections because the
spleen is no longer present

DISORDERS OF THE RED BLOOD CELL

Anemia occurs when the rate of RBC production falls below that of cell destruction, or when there is a
loss of

RBCs, causing their number and the hemoglobin level to fall below that of production.

A. NORMOCHROMIC NORMOCYTIC
Normochromic (normal color), normocytic (normal cell size)
The RBCs appear normal in both color and size; however, there simply are
too few of them for effective oxygen transport.
a. ACUTE BLOOD-LOSS ANEMIA
Sx:
pale
tachycardia
 tachypnea
Newborns: gasping respirations, intercostal retractions, and
cyanosis.
listless and inactive, dizzy, and, possibly, comatose.
Dx: --
Tx:
Control of bleeding by addressing its underlying cause
Transfusing additional RBCs
Keep child warm
Infant under incubator
Until blood transfusion is available: IVF of normal saline or
lactated Ringer’s
b. ANEMIA OF ACUTE INFECTION
Acute infection or inflammation, especially in infants, can cause
increased destruction or decreased production of erythrocytes.
Management involves treatment of the underlying condition. When
the condition is reversed, blood values will return to normal.
c. ANEMIA OF RENAL DISEASE
Either acute or chronic renal disease can cause loss of function in
kidney cells, which causes an accompanying decrease in
erythropoietin production, resulting in a normocytic, normocyctic
anemia. Administration of recombinant erythropoietin can increase
RBC production and correct the anemia, but not the renal disease.
d. ANEMIA OF NEOPLASTIC DISEASE
Malignant growths such as leukemia or lymphoma (common
neoplasms of childhood) result in normochromic, normocytic anemias
because the invasion of bone marrow by proliferating neoplastic cells
impairs RBC production.
e. HYPERSPLENISM
The overactive spleen then leads to increased destruction of RBCs
which can cause anemia and to pancytopenia (deficiency of all cell
elements of blood). Therapeutic management consists of treating the
underlying splenic disorder and includes a possible splenectomy.
f. APLASTIC ANEMIA
Aplastic anemias result from depression of hematopoietic activity in
the bone marrow. Congenital aplastic anemia (Fanconi syndrome) is
inherited as an autosomal recessive trait. Acquired aplastic anemia is a
decrease in bone marrow production, which occurs if a child is
excessively exposed to radiation, drugs, or chemicals known to cause
bone marrow damage.
Sx:
 pale
fatigues easily
anorexia
child bruises easily
petechiae (pinpoint, macular, purplish-red spots caused by an
intradermal
submucous hemorrhage).
may have excessive nosebleeds or gastrointestinal bleeding.
Observe closely for signs of cardiac decompensation ( tachycardia,
tachypnea, shortness of breath, or cyanosis)
Dx:
Tx:
immediately discontinue any drug or chemical suspected of causing
the bone marrow dysfunction
hematopoietic stem cell transplantation
Packed RBCs, platelet transfusions, cyclosporine, antithymocyte
globulin (ATG), and an RBC-stimulating factor (erythropoietin)
oral corticosteroid (prednisone)
g. HYPOPLASTIC ANEMIAS
also result from depression of hematopoietic activity in bone marrow
and can also be either congenital or acquired; with hypoplastic
anemias, only RBCs are affected.
Congenital hypoplastic anemia (Blackfan-Diamond syndrome) is a
rare disorder apparently caused by an inherent defect in RBC
formation that affects both sexes and shows symptoms as early as the
first 6 to 8 months of life
Sx:
With iron-deficiency anemia, blood cells appear hypochromic and
microcytic and are few in number;
however, in hypoplastic anemia, they are not only few in number but
also their structure is normochromic and normocytic.
Dx:
Tx:
congenital form: corticosteroid therapy
transfusions of packed RBCs
Iron chelation program subcutaneous infusion of deferoxamine
(Desferal)
B. HYPOCHROMIS ANEMIAS
When hemoglobin production is inadequate, erythrocytes appear pale
(hypochromia) and are also usually reduced in diameter (microcytic).
 a. IRON-DEFICIENCY ANEMIA
Without adequate iron, hemoglobin cannot be incorporated
into RBCs.
Infant:
A newborn usually has enough iron reserve to last for the first 6
months, however, preterm infants and whose mother is iron deficient
during pregnancy may be given an iron supplement beginning at 2
months of age.
Older Child:
In children older than 2 years of age, chronic blood loss is the most
frequent cause of iron-deficiency. Adolescent girls with heavy
menstrual periods can become iron deficient when this is combined
with frequent attempts to diet or with overconsumption of snack foods
that are low in iron.
Sx:
pale conjunctiva
“fair skinned"
infants:
develop poor muscle tone and reduced activity
soft systolic precordial murmur upon auscultation
spoon-shaped or depressed in contour fingernails
RBC: poikilocytic (irregular in shape)
school-age:
poor school achievement
chronic fatigue
pica
Dx:
Laboratory studies
Tx:
ferrous sulfate for 4 to 6 weeks
b. CHRONIC INFECTION ANEMIA
When infections are chronic, however, anemia of a hypochromic,
microcytic type occurs, which is probably caused by impaired iron
metabolism.
C. MACROCYTIC (MEGALOBLASTIC) ANEMIAS
A macrocytic anemia is one in which the RBCs appear abnormally large.
Such cells are actually immature erythrocytes or megaloblasts (nucleated
immature red cells)
a. ANEMIA OF FOLIC ACID DEFICIENCY
 A deficiency of folic acid combined with vitamin C deficiency
produces an anemia in which the erythrocytes grow abnormally large.
Megaloblastic arrest, or inability of RBCs to mature past this early
stage, may occur in the first year of life
b. PERNICIOUS ANEMIA (VITAMIN B12 DEFICIENCY)
Vitamin B12 is necessary for the maturation of RBCs. Pernicious
anemia results from a deficiency in vitamin B12, either from
inadequate intake or malabsorption
Sx:
pale and anorexic
irritable
chronic diarrhea
tongue appears smooth and beefy red due to papillary atrophy
If not identified and treated at that point: neuropathologic findings
(ataxia, hyporeflexia, paresthesia, and a positive Babinski reflex)
Dx:
Tx:
vitamin B12-deficient diet: temporary injections of vitamin B12
lack of the intrinsic factor: lifelong monthly intramuscular injections
of vitamin B12
D. HEMOLYTIC ANEMIAS
Hemolytic anemias are those in which the number of erythrocytes is low
because there is increased erythrocyte destruction.
a. CONGENITAL SPHEROCYTOSIS
Congenital spherocytosis is a hemolytic anemia that occurs most
frequently in the White Northern European population and is inherited
as an autosomal dominant trait.
Sx:
Chronic jaundice
splenomegaly
Gallstones (older school-age children and adolescents)
Dx:
Tx:
Blood transfusion
b. GLUCOSE-6-PHOSPHATE DEHYDROGENASE DEFICIENCY
The enzyme glucose-6-phosphate dehydrogenase (G6PD) is necessary
for maintenance of RBC life; lack of the enzyme results in premature
destruction of RBCs. The disease is an X-linked recessive trait
inheritance and is found most often in persons of African,
Mediterranean, or Asian decent
 Sx:
hemolysis
low-grade fever
back pain
Dx:
blood smear show Heinz bodies (oddly shaped particles in RBCs)
rapid enzyme scteening test
electrophoretic analysis of RBCs.
Tx:
avoid common drugs such as acetylsalicylic acid
c. SICKLE-CELL ANEMIA
Sickle-cell anemia is an autosomal recessive inherited disorder carried
on the ß chain of hemoglobin; the amino acid valine takes the place of
the normally appearing glutamic acid.
Sx:
erythrocytes: elongated and crescent shaped (sickled)
acute pain
cell destruction
Dx:
prenatally by chorionic villi sampling or from cord blood during
amniocentesis
neonatal screening
Tx:
Pain relief
Adequate hydration
Oxygenation
d. SICKLE-CELL CRISIS
Sickle-cell crisis is the term used to denote a sudden, severe onset of
sickling. There is pooling of many new sickled cells in blood vessels
causing consequent tissue hypoxia beyond the blockage (a vaso-
occlusive crisis).
• A sequestration crisis occurs when there is splenic seques tration of
RBCs or severe anemia occurs due to pooling and increased
destruction of sickled cells in the liver and spleen. Shock symptoms
occur from hypovolemia. The spleen is enlarged and tender.
• A hyperhemolytic crisis occurs when there is increased destruction
of RBCs.
• A megaloblastic crisis occurs if the child has folic acid or vitamin B
deficiency (new RBCs cannot be fully formed due to lack of these
ingredients).
 • An aplastic crisis (temporary cessation of RBC production) occurs
when there is a sudden decrease in RBC production. This form usually
occurs with infection. It creates a severe anemia.
Sx:
fever
anemia
Stasis of blood and infarction (local pain)
Infants have swelling of the hands and feet (a hand-foot syndrome)
protruding abdomen
symptoms of fever, tachypnea, wheezing, or cough that leads to
pneumonia
Eye sclera become icteric (yellowed)
Priapism, a persistent painful erection (vaso-occlusion in the penis)
Dx:
blood-spot screening
urine cultures
chest X-ray
complete blood count
Tx:
Acetaminophen (Tylenol)
narcotic analgesic (IV morphine)
intensive IVF replacement therapy
Hydroxyurea, an antineoplastic agent
Antibiotics
Blood transfusion (usually packed RBCs)
Hematopoietic stem cell transplantation
E. THALASSEMIAS
The thalassemias are autosomal recessive anemias associated with
abnormalities of the b chain of adult hemoglobin (hemoglobin A).
a. THALASSEMIA MINOR (HETEROZYGOUS B-THALASSEMIA)
Children with thalassemia minor, a mild form of this anemia, produce
a combination of both defective ß hemoglobin and normal
hemoglobin. The condition represents the heterozygous form of the
disorder and can be compared with children having the sickle-cell
trait.
Sx:
Pallor
Tx:
require no treatment
b. THALASSEMIA MAJOR (HOMOZYGOUS B-THALASSEMIA)
 Thalassemia major is also called Cooley anemia or Mediterranean
anemia. Because this is a B-chain hemoglobin defect, symptoms do
not become apparent until a child's fetal hemoglobin has largely been
replaced by adult hemoglobin during the second half of the first year
of life.
Sx:
bone pain
protrusion of the upper teeth
parietal and frontal bossing
marked malocclusion
base of the nose may be broad and flattened
eyes may be slanted with an epicanthal fold, as in Down syndrome
osteoporotic (of lessened density) tissue
anorexia and vomiting
Epistaxis
Dx:
Tx:
Stem cell transplantation
Digitalis
Diuretics
low-sodium diet
hypertransfusion therapy
splenectomy
oral iron-chelating agent
c. AUTOIMMUNE ACQUIRED HEMOLYTIC ANEMIA
Occasionally, autoimmune antibodies (abnormal antibodies of the
immunoglobulin [Ig]G class) attach themselves to RBCs, destroying
them or causing hemolysis.
Sx:
low-grade fever
anorexia
lethargy
pallor
cterus
Both urine and stools appear dark
In a few children:
high fever
hemoglobinuria
marked jaundice
enlarged liver and spleen
 Dx:
Laboratory findings reveal RBCs have become extremely
small and round (spherocytosis)
Direct Coombs test result is positive
Tx:
single blood transfusion
corticosteroid therapy (oral prednisone)
splenectomy or stronger immunosuppressive agents
(cyclophosphamide
(Cytoxan) or azathioprine (Imuran))
F. POLYCYTHEMIA
Polycythemia is an increase in the number of RBCs. The condition results
from increased erythropoiesis, which occurs as a compensatory response to
insufficient oxygenation of the blood in order to help supply more oxygen to
body cells.
Plethora (marked reddened appearance of the skin) occurs because of the
increase in total RBC volume. Erythrocytes are usually macrocytic (large),
and the hemoglobin content is high.
DISORDERS OF BLOOD COAGULATION
A. PURPURAS
Purpura refers to a hemorrhagic rash or small hemorrhages in the superficial
layer of skin.
a. IDIOPATHIC THROMBOCYTOPENIC PURPURA
Idiopathic thrombocytopenic purpura (ITP) is the result of a decrease
in the number of circulating platelets in the presence of adequate
megakaryocytes (precursors to platelets).
sx:
miniature petechiae or as large areas of asymmetric ecchymosis most
prominent over the legs, although they may occur anywhere on the
body
Epistaxis or bleeding into joints may be present.
dx:
Laboratory studies reveal marked thrombocytopenia.
Bone marrow examination
tx:
Oral prednisone
intravenous immunoglobulin (IVIG)
Rh-positive children: anti-D immunoglobulin
Platelet transfusion
Acetaminophen (Tylenol)
 b. HENOCH-SCHOLEIN SYNDROME
Henoch-Schönlein purpura (also called anaphylactoid purpura) is
caused by increased vessel permeability, which leads to bleeding in
the small blood vessels.
sx:
purpural rash occurs typically on the buttocks, posterior thighs, and
extensor surface of the arms and legs, tips of the ears may be
involved.
rash begins as a crop of urticarial lesions that change to pink
aculopapules.
become hemorrhagic (bright red)
brown macular spots
child's joints are tender and swollen.
gastrointestinal symptoms (abdominal pain, vomiting, or blood in
stools)
dx:
biopsy
Laboratory studies
tx:
oral corticosteroid therapy (prednisone)
mild analgesics for a short period
B. HEMOLPHILIAS
Hemophilias are inherited disorders of blood coagulation.
a. HEMOPHILIA A (FACTOR VIII DEFICIENCY
The classic form of hemophilia is caused by deficiency of the
coagulation component factor VIII, the antihemophilic factor, and
transmitted as a sex-linked recessive trait. Factor VIII is an intrinsic
factor of coagulation; its absence causes the intrinsic system for
manufacturing thromboplastin be incomplete.
sx:
infant who bleeds excessively after circumcision
the lower extremities (where the child bumps things) become heavily
bruised
soft tissue bleeding
painful hemorrhage into joints, which become swollen and warm
dx:
partial thromboplastin time (PTT)
tx:
administration of factor VIII supplied by fresh whole blood, fresh or
frozen plasma, or a concentrate of factor VIII (powdered form)
 E-aminocaproic acid, a fibrinolytic enzyme
factor IX concentrate (Proplex or Konyne)
b. VON WILLEBRAND DISEASE
von Willebrand disease, an inherited autosomal dominant disorder,
affects both sexes and is most common among Whites
sx:
Bleeding time is prolonged, with most hemorrhages occurring from
mucous membrane sites
Epistaxis is a major problem
In girls, menstrual flow is unusually heavy and may cause
embarrassment
from stained clothing.
dx:
tx:
factor VIII replenishment
administration of desmopressin (DDÁVP)
c. CHRISTMAS DISEASE (HEMOPHILIA B, FACTOR IX DEFICIENCY)
Christmas disease, first noted in 1952 in a patient by the name of
Stephen Christmas, is caused by factor IX deficiency. It is transmitted
as a sex-linked recessive trait, and only approximately 15% of people
with hemophilia have this form. Treatment is with a concentrate of
factor IX, which is available for home administration.
d. HEMOPHILIA C (FACTOR XI DEFICIENCY)
Hemophilia C, or plasma thromboplastin antecedent deficiency, is
caused by factor XI deficiency, is transmitted as an autosomal
recessive trait, and occurs in both sexes. The symptoms are generally
mild compared with those in children with factor VIII or factor IX
deficiencies. Bleeding episodes are
treated with administration of desmopressin (DDAVP) or transfusion
of fresh blood or plasma
e. DISSEMINATED INTRAVASCULAR COAGULATION
is an acquired disorder of blood clotting that results from excessive
trauma or some similar underlying stimulus, such as an acute infection
or trauma. A child begins to develop petechiae or have uncontrolled
bleeding from puncture sites from injections or intravenous therapy.
Ecchymoses and petechiae form on the skin.
Observe all children with a serious illness carefully for signs such as
these of increased bleeding to help identify that this is happening.