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Hematologic Disorders and

Pregnancy
NCM 108
A. Anemia and Pregnancy
1. Iron- Deficiency Anemia
2. Folic Acid- Deficiency Anemia
3. Sickle- cell Anemia
4. Thalassemia
5. Malaria

B. Coagulation disorders
1.Von Willebrand Disease
2.Hemophilia B
3.Idiopathic Thrombocytopenic Purpura
The 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. Many women enter pregnancy with a deficiency of iron stores resulting from
a combination of a diet low in iron, heavy menstrual periods, or unwise weight-reducing programs.
The type of anemia is characteristically a microcytic (i.e., small red blood cell) and hypochromic
(i.e., less hemoglobin than the average red cell) anemia, which occurs when such an inadequate
supply of iron is ingested that iron is not available for incorporation into red blood cells.
• A woman experiences extreme fatigue and poor exercise tolerance because she cannot transport
oxygen effectively. The condition is mildly associated with low birth weight and preterm birth.
Because the body recognizes that it needs increased nutrients, some women with this condition
develop pica, or the craving and eating of substances such as ice or starch. It is also associated with
restless leg syndrome.
• To prevent this common anemia, women should take prenatal vitamins containing 27 mg of iron as
prophylactic therapy during pregnancy. In addition, they need to eat a diet high in iron and vitamins
(e.g., green leafy vegetables, meat, and legumes) so the supplement is truly a supplement. Women
who develop iron-deficiency anemia will be prescribed therapeutic levels of medication (120 to 200
mg elemental iron per day), usually in the form of ferrous sulfate or ferrous gluconate. Iron is
absorbed best in an acid medium. Advise women, therefore, to take iron supplements with orange
juice or a vitamin C supplement, which supplies ascorbic acid.
The Woman with Folic-acid Deficiency
Anemia
• The anemia that develops is a megaloblastic anemia (enlarged red blood cells).
Because of the size of the cells, the mean corpuscular volume will be elevated in
contrast to the lowered level seen with iron-deficiency anemia. Slow to progress,
the deficiency may take several weeks to develop or may not be apparent until the
second trimester of pregnancy. Full blown, it may be a contributory factor in early
miscarriage or premature separation of the placenta.
• All women expecting to become pregnant are advised to begin a supplement of
400 μg folic acid daily in addition to eating folate-rich foods (e.g., green leafy
vegetables, oranges, dried beans). Over-the-counter women’s multivitamin
preparations generally do contain adequate folic acid for pregnancy so be certain
women are specifically taking a prenatal or women’s multivitamin. Women who
develop folic acid–deficiency anemia are prescribed even higher or therapeutic
levels of folic acid.
The 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, sickling hemoglobin (HbS) results; if it
is substituted for the amino acid lysine, nonsickling hemoglobin (HbC) results. An individual who is heterozygous
(i.e., has only one gene in which the abnormal substitution has occurred) has the sickle-cell trait (HbAS). If the
person is homozygous (i.e., has two genes in which the substitution has occurred), sickle-cell disease (HbSS)
results .
• With the disease, the majority of red blood cells are irregular or sickle shaped, so they cannot carry as much
hemoglobin as normally shaped red blood cells can. When oxygen tension becomes reduced, as occurs at high
altitudes, or blood becomes more viscid than usual, such as occurs with dehydration, the cells clump together
because of their irregular shape, resulting in vessel blockage with reduced blood flow to organs. The cells then will
hemolyze (i.e., be destroyed), thus reducing the number available and causing a severe anemia.
• Because a pregnant woman with sickle-cell anemia has vascular stasis, they are more susceptible to bacteriuria
than other women; periodically collect a clean-catch urine sample during pregnancy to detect developing
bacteriuria while a woman is still asymptomatic. Throughout pregnancy, monitor a woman’s nutritional intake to
be certain she is consuming sufficient amounts of folic acid and possibly an additional folic acid supplement.
Ensure the woman is drinking at least eight glasses of fluid daily to be certain she is guarding against dehydration.
• Assess a woman’s lower extremities at prenatal visits for varicosities or pooling of blood in leg veins, which can
lead to red cell destruction. Standing for long periods during the day increases this pressure, whereas 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 periods of standing and adequate rest
periods.
The Woman with Thalassemia

• The thalassemias are a group of autosomal recessively inherited blood disorders


that lead to poor hemoglobin formation and severe anemia. They occur most
frequently in Mediterranean, African, and Asian populations . Symptoms first
appear in childhood. Treatment focuses on combating anemia through such
measures as folic acid supplementation and perhaps blood transfusion to infuse
hemoglobin-rich red blood cells. Women with thalassemia do not usually take an
iron supplement during pregnancy because they could receive an iron overload
because iron is infused with blood transfusions.
The Woman with Malaria
• Malaria is a protozoan infection that is transmitted to people by Anopheles mosquitoes The
infection causes red blood cells to stick to the surface of capillaries causing obstruction of these
vessels and resulting in end-organ anoxia and blood not reaching organs effectively.
• Malaria can be prevented by wearing clothing that covers most of the body as well as using an
insect repellent when in an area infested with mosquitoes, sleeping at night with a mosquito net, or
keeping windows closed to prevent mosquitoes from entering. As further prevention, urge women
to delay travel to endemic areas until after pregnancy if possible.
• Treatment is with a combination of antimalarial drugs, which will both stop the course of the
disease and help reduce the incidence of low birth weight and preterm birth.
Sulfadoxine/pyrimethamine is safe to administer during the last trimester of pregnancy.
Chloroquine is safe to administer all during pregnancy and so is the drug of choice . Quinine,
atovaquone and proguanil (Malarone), or tetracyclines, although effective against the disease,
should not be used at any point in pregnancy or with women who are breastfeeding as they are
teratogenic.
Coagulation Disorders and Pregnancy
1. Von Willebrand Disease
2. Hemophilia B
3. Idiopathic Thrombocytopenic Purpura
Von Willebrand Disease
• von Willebrand disease, is a coagulation disorder inherited as an autosomal
dominant trait and so does occur in women . Women will have normal platelet
counts, but bleeding time is prolonged. Levels of factor VIII–related antigen
(VIII-R) and factor VIII coagulation activity (VIII-C) are both reduced. From the
time she was a child, a woman with the disorder might have noticed menorrhagia
or frequent episodes of epistaxis. If these symptoms were not severe, however, the
condition can go undiagnosed until pregnancy when a woman experiences a
spontaneous miscarriage or postpartum hemorrhage. Replacement of the missing
coagulation factors by infusion of cryoprecipitate or fresh frozen plasma may be
necessary before labor to prevent excessive bleeding with birth.
Hemophilia B

• Hemophilia B (Christmas disease, factor IX deficiency) is a sex-linked disorder,


so the actual disease occurs only in males. However, female carriers may have
such a reduced level of factor IX (only 33% of normal) that hemorrhage with
labor or a spontaneous miscarriage can be a serious complication.
• Carriers of the disorder need to be identified before pregnancy. Restoration of
factor IX levels can be quickly restored by infusion of factor IX concentrate or
fresh frozen plasma.
Idiopathic Thrombocytopenic Purpura(ITP)
• Idiopathic thrombocytopenic purpura (ITP), which is a decreased number of platelets, is not
inherited, can occur at any time in life, and so occasionally occurs during pregnancy. The cause of
the condition is unknown, but because symptoms usually occur shortly after a viral invasion such
as an upper respiratory tract infection, it is assumed to be an autoimmune reaction (an antiplatelet
antibody that destroys platelets is apparently released).
• Laboratory studies reveal a marked thrombocytopenia (platelet count may be as low as
20,000/mm3 from a usual count of 150,000/mm3). Without an adequate level of platelets, the
woman is prone to frequent nosebleeds and minute petechiae or large ecchymoses appear on her
body.
• Oral prednisone or a platelet transfusion or plasmapheresis may be administered to temporarily
increase the platelet count to prevent increased bleeding at birth. The antiplatelet factor can cross
the placenta and cause accompanying platelet destruction in the newborn or allow a newborn to be
born with the illness, so a careful assessment of the baby is necessary at birth.
That in all things God may be glorified.

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