HEMATOLOGIC DISORDERS AND PREGNANCY

Hematologic disorders during pregnancy involve either blood formation or coagulation disorders.

ANEMIA AND PREGNANCY

Because the blood volume expands during pregnancy slightly ahead of the red cell count, most women
have a psuedoanemia of early pregnancy. This condition is normal and should not be confused with true
types of anemia that can occur as complications of pregnancy.

A WOMAN WITH IRON DEFICIENCY ANEMIA

Iron deficiency anemia is the most common anemia of pregnancy, complicating as many as 15% to 25%
of all pregnancies (Malee, 2003). Many women enter pregnancy with a deficiency of iron stores resulting
from a diet low in iron, heavy menstrual periods, or unwise weight-reducing programs. Iron stores are
apt to be low in women who were pregnant less than two years before the current pregnancy or those
from low socioeconomic levels who have not had iron-rich diets. When the hemoglobin level is below
12mg/dl (hematocrit under 33%), iron-deficiency is suspected. It is confirmed by a corresponding low
serum iron level and an increase iron-blinding capacity.

Iron is made available to the body by absorption from duodenum into the blood stream after it
is ingested. In the blood stream, it is bound to transferring for transport to the liver, spleen, and bone
marrow. At these sites it is incorporated into hemoglobin or stored as ferritin.

Iron-deficiency anemia is characteristically a microcytic(small red blood cell), hypochromic (less

hemoglobin than average red cell) anemia because when an inadequate supply of iron is ingested, iron
is unavailable for incorporation into red blood cells. Both hematocrit and hemoglobin will be reduced
(under 33% and 12mg/dl, respectively). The serum transferring level will be under 100mg/dl, the
transferring saturation saturation level will be under 5%, the serum iron level will be under 30mg/dl, and
mean corpuscular hemoglobin concentration will be under 30; iron-binding capacity, in contrast, will be
increased (over 400mg/dl). Iron-deficiency anemia is mildly associated with low birth weight and
preterm birth (Mungen, 2003). Because the body recognizes that needs increased nutrients, some
women develop pica, or the craving and eating of substances such as ice or starch. A woman
experiences extreme fatigue and poor exercise tolerance because she cannot transport oxygen
effectively.

To prevent this common anemia, women should take parenteral vitamins containing an iron
supplement of 60mg elemental iron as prophylactic therapy during pregnancy. In addition, they need to
eat a diet high in iron and vitamins (green leafy vegetables, meat , legumes, fruit). Women who develop
iron-deficiency anemia will be prescribed therapeutic level of medication (120 to 180mg elemental
iron/day), usually in the form of ferrous sulfate or ferrous glocunate. Iron is best absorbed from an acid
medium. Therefore, advice women to take iron supplements with orange juice or a vitamin c
supplement. If they are not already enrolled in a WIC program but are eligible, making a referral could
help ensure a better diet. When women begin to take a prescribed iron supplement, new red blood
cells should begin to increase, or their reticulocyte counts should rise from a normal range (0.5% to
1.5%) to 3% to 4% by two weeks time. Some women report constipation or gastric irritation when taking
oral iron supplements. Increasing roughage in the diet and always taking the pills with food help reduce
this symptoms.

If iron-deficiency anemia is severe and a woman has difficulty with oral iron therapy,
intramuscular or intravenous iron dextran can be prescribed.

A WOMAN WITH FOLIC ACID-DEFICIENCY ANEMIA

Folic acid, or folacin, one of the B vitamins, is necessary for the normal formation of red blood
cell in the mother as well as being associated with preventing neural tube defects in the fetus. Folic acid-
deficiency anemia is seen in 1% to 5% of pregnancies(Malee, 2003). It occurs most often in multiple
pregnancies because of the increased fetal demand; in women with secondary hemolytic illness in which
there is rapid destruction and production of new red blood cells; in women who are taking hydantoin,
an anticonvulsant agent that interferes with folate absorption; and women who hve been taking oral
contraceptives. The anemia that develops is megaloblastic anemia (enlarged red blood cells). The mean
corpuscular volume will be elevated, in contrast to the lowered level seen with iron-deficiency anemia.
The deficiency may take a number of weeks to develop, so it often becomes most apparent during the
second trimester of pregnancy. It may be a contributory factor in early miscarriage or premature
separation of the placenta.

Because the fetal effects of deficiency occur in the first few weeks of fetal development,
women expecting to 400 mg folic acid daily(de-Jong-Van Den Berg et al.,2005)in addition to eating
folacin-rich foods (green leafy vegetables,oranges,dried beans). During pregnancy, the folic acid
requirement increases to 600 mg/day. Over -the- counter multivitamin preparations generally do not
contain adequate folic acid for pregnancy, whereas vitamins specifically designed for pregnancy do.
Women who develop folic acid-deficiency anemia are prescribed even higher or therapeutic levels of
folic acid. At prenatal visits, ask whether a woman is taking her prescribed vitamin. To save money ,
women may not have a prescription filled and may be using over-the-counter, less expensive types, not
aware of the difference.

A WOMAN WITH SICKLE CELL ANEMIA

Sickle cell anemia is a recessively inherited hemolytic anemia caused by an abnormal amino acid
in the beta chain of hemoglobin. If the abnormal amino acid replaces the amino acid valine, sickle
hemoglobin (HbS) results; if it is substituted for the amino acid lysine, non-sickling hemoglobin (HbC)
results. An individual who is heterozygous (has only one gene in which the abnormal substitution has
occurred) has the sickle cell trait (HbAS). IF the person is homozygous (has two genes in which the
substitution has occurred) , sickle cell disease (HbSS)results.

With the disease, majority of red blood cells are irregular sickle-shaped so cannot as more
hemoglobin as normally shaped red blood cells. When oxygen tension becomes more viscid than usual
(dehydration), the cells tend to clump because of the irregular shape. This clumping can result in vessel
blockage with reduced blood flow to organs. The cells then will hemolyze, reducing the number
available and causing a severe anemia. Approximately 1 in every 10 african Americans has the sickle trait
(i.e., carries a recessive gene for S hemoglobin but is asymptomatic); theoretically, 1 in every 400 african
Americans has the disease. Although the sickle cell trait does not appear to influence the course of
pregnancy, prematurity, miscarriage, or perinatal mortality rates of these may be higher for women
with the homozygous disease (Ser Jeant et al.,2004). Women with the trait do seem to have an
increased of asymptomatic bacteriuria, resulting in an increased incidence of pyelonephritis.

At anytime in life, sickle cell anemia is the threat to life if vital blood vessels such as those to the
liver, kidneys, heart, lungs or brain become blocked. In pregnancy, blockage to the placental circulation
can directly compromise the fetus, causing low birth weight and possibly fetal death.

Assessment

All African American women who have not been previously tested should be screened for sickle cell
anemia at a first prenatal visit. Hemoglobin levels for all women with sickle cell disease should be obtain
through out pregnancy. A woman with sickle cell disease may normally have a hemoglobin level of 6 to 8
mg/100 ml. unless she receives active interventions to raise this level, she will maintain it during
pregnancy, reducing oxygen of the fetus. Hemolysis in a sickle cell crisis may occur so rapidly that a
woman’s hemoglobin level can fall to 5 or 6 mg/100 ml in a few hours. There is an accompanying rise in
her indirect bilirubin level because he cannot conjugate the bilirubin released from so many red blood
cells so quickly destroyed.

Because a pregnant woman with sickle cell anemia is more susceptible to bacteriuria while a
woman is still asymptomatic.

Throughout pregnancy, monitor a woman’s diet to be certain she is consuming sufficient

amounts of folic acid and possibly an additional folic acid supplement, which may be necessary to build
new red blood cells. Her fluid intake should also be carefully monitored. She should consume at least
eight glasses of fluids daily. Early in pregnancy, when she may be nauseated, her fluid intake can easily
decrease and dehydration and a subsequent sickle cell crisis may occur.

Assess a woman’s lower extremities at prenatal visits for varicosities or pooling of blood in leg
veins, which are apt to occur from uterine pressure as pregnancy advances. Such pooling and pressure
can lead to red cells destruction. Standing for long periods during the day increases this pressure, where
as sitting on a chair with the legs elevated or lying on the side in a modified Sims’ position encourages
venous return from the lower extremities. Help a woman plan her day so she has limited long period of
standing and adequate rest periods.

Fatal health is usually monitored during pregnancy by an ultrasound examination at 16 to 24

weeks to assist for intrauterine growth restriction and by weekly nonstress or ultrasound examinations
beginning at about 30 weeks. Blood flow through the uterus and placenta may be measured by blood
flow velocity. If blood flow velocity is reduced, the change of intrauterine growth restrictions is
increased.
Therapeutic Management

Intervention to prevent sickle cell crisis an include periodic exchange transfusion throughout pregnancy
to replace sickled cell with cells with normal cells. An exchange transfusion serves a secondary purpose
of removing a quantity of the increased bilirubin resulting from the breakdown of red blood cells as well
as restoring the hemoglobin level(malee, 2003). If crisis occurs, controlling pain, administering oxygen as
needed, and increasing the fluid volume of the circulatory system to lower viscosity are important
interventions. The fluid administered is often hypotonic (0.5 saline) to keep plasma tension low because
of the difficulty a woman has concentrating urine to remove large amount of fluid. As a rule, women
with sickle cell disease are not given an iron supplement during pregnancy. These cell cannot
incorporate in iron in the usual manner that normal cell can, so excessive iron buildup may result.
Women do need a folic acid supplement to kept the new cell produced from being megaloblastic (Katz,
2003).

A women develops an infections that raises her temperature and causes her to perspire more
than normally (creates dehydration) or contracts a respiratory infection that compromises air exchange
so that her PO2 is lowered , hospitalization for observation may be necessary to rule out the
development of a sickle cell crisis and subsequent Hemolysis of crowded cell.

When the fetus is mature, the time and method of birth are individualized. Keep a woman well
hydrated in labor. If an operative birth is necessary, she generally receives nerve block anesthesia rather
than a general anesthetic to avoid the threat of hypoxia.

Women generally are interested in determining at birth whether their child has inherited the
disease. Because the disorder is recessively inherited, if one of the parents has the disease and the other
is free of the disease and trait, the chances that the child will inherit the disease are zero. If a woman
has the disease and her partner has the trait, the chances that the child will be born with the disease are
50%. If both parents have the disease, all their children will also have the disease.

Symptoms of sickle cell disease do not become clinically apparent until the child’s fetal
hemoglobin has converted to a largely adult pattern (in 3 to 6 months). Fetal hemoglobin is composed of
two alpha and two beta chain, it will not be manifested clinically until this chain appears. Electrophoresis
of red blood cells obtained during fetal life by percutaneous umbilical blood sampling or amniocentesis,
however, can reveal the presence of the disease on the few beta chains already present in utero.
Newborn have approximately 15% adult hemoglobin at birth, so electrophoresis testing at birth can also
reveal if the disease is present. Screening is routine in some settings.