ANATOMY AND PHYSIOLOGY: HEMATOLOGY THE CHILD WITH ALTERATION IN OXYGENATION:

TRANSPORT
 The Hematologic System is made up of the Blood, the
Spleen, Bone Marrow, and the Liver DISORDERS AFFECTING RBC
 The principal component of the hematologic system is the
blood. IRON DEFICIENCY ANEMIA
 Blood is made up of three main components: red blood  is a common type of anemia- a condition in which blood
cells, white blood cells, and plasma. lacks adequate healthy red blood cells.
 Red blood cells, erythrocytes, are the most common blood  Red blood cells carry oxygen to the body's tissues.
cells. They appear as discs with an indent in the surface,  As the name implies, iron deficiency anemia is due to
and they lack a nucleus. insufficient iron.
 White Blood Cells, or leukocytes, are one of the  Without enough iron, your body can't produce enough of a
body's defenses. substance in red blood cells that enables them to carry
 There are two types: granulocytes and agranulocytes. oxygen (hemoglobin).
 As a result, iron deficiency anemia may leave you tired
There are seven types of leukocytes. and short of breath.
 Neutrophils  Initially, iron deficiency anemia can be so mild that it goes
 Eosinophils unnoticed. But as the body becomes more deficient in iron
 Basophils and anemia worsens, the signs and symptoms intensify.
 Extreme fatigue
 Neutrophils fight bacteria and fungi.  Pale skin
 Eosinophils fight larger parasites and  modulate the  Weakness
inflammatory response with allergies.  Shortness of breath
 Basophils release histamine to induce an  Chest pain
inflammatory response.  Frequent infections
 Headache
There are three types of lymphocytes:  Dizziness or lightheadedness
 B Cells, T Cells, and Natural Killer Cells.  Cold hands and
 B Cells release antibodies and assist T Cell activation.  feet Inflammation
 T Cells can be regulatory, which cause the body to return  soreness of your tongue
to normal after an inflammatory response, they can  Brittle nails
activate and regulate B and T Cells, or they can attack  Fast heartbeat
virus infected or cancer cells.  Unusual cravings for non-nutritive substances,
such as ice, dirt or starch
 Natural killer cells attack virus infected and tumor cells as
 Poor appetite, especially in infants and children
well.
with iron deficiency anemia
 uncomfortable tingling or crawling feeling in your
 Monocytes move to tissues and then differentiate into
legs (restless legs syndrome)
macrophages.
 Macrophages are phagocytic cells, and they eat cellular
 Iron deficiency anemia occurs when your body doesn't
waste, debris, and pathogens.
have enough iron to produce hemoglobin.
 They also stimulate lymphocytes. 
 Blood loss- such as from a peptic ulcer, a hiatal hernia, a
colon polyp or colorectal cancer
 Plasma is a fluid made up of 90% of water, in which blood
 Gastrointestinal bleeding can result from regular use
is suspended.
of some over-the-counter pain relievers, especially
 Plasma allows blood cells to travel through vessels, in the aspirin.
water it contains.
 A lack of iron in your diet.
 Plasma is also made up of minerals, nutrients, and
 An inability to absorb iron- Iron from food is absorbed into
electrolytes.
your bloodstream in your small intestine. (celiac disease)
 Platelets are cells which are critical to blood clotting.
 Pregnancy. Without iron supplementation

 The spleen is an important organ: it acts as a reservoir for RISK FACTORS

blood, and it filters out erythrocytes that can no longer  Women.
carry out their function.  Infants and children.
 And it can still be removed, and the only side effects  Vegetarians.
would be an slight increase in white blood cells, and  Frequent blood donors.
platelets, and increased susceptibility to some diseases.
 With respect to the Hematologic System, the Liver  Because women lose blood during menstruation,
detoxifies the blood.  women in general are at greater risk of iron deficiency
anemia.
 Infants, especially those who were low birth weight or
born prematurely, who don't get enough iron from
breast milk or formula may be at risk of iron  is an autoimmune disorder in which the body fails to make
deficiency. enough healthy red blood cells (RBCs).
 People who don't eat meat may have a greater risk of  Vitamin B-12, or cobalamin, is found in certain foods and
iron deficiency anemia if they don't eat other iron-rich medications.
foods.  IF is a protein made by the stomach’s mucosal (mucus-
 People who routinely donate blood may have an secreting) cells, called parietal cells.
increased risk of iron deficiency anemia since blood  When vitamin B-12 enters the body, it binds with IF. The
donation can deplete iron stores. two are then absorbed in the last part of the small
intestine.
TESTS AND DIAGNOSTICS
 Red blood cell size and color.  The body requires vitamin B-12 and a type of protein
 Hematocrit. called intrinsic factor (IF) to make red blood cells.

 With iron deficiency anemia, red blood cells are CAUSES

smaller and paler in color than normal.  PA is a type of macrocytic anemia and is sometimes
 This is the percentage of your blood volume made up called megaloblastic anemia.
by red blood cells. Normal levels are generally  long-term use of certain medications and antibiotics
between 34.9 and 44.5 percent for adult women and (methotrexate, azathioprine, etc.)
38.8 to 50 percent for adult men.  chronic obstructive pulmonary disease (COPD)
 folate deficiency caused by poor intake or malabsorption
 Hemoglobin. Lower than normal hemoglobin levels
indicate anemia. The normal hemoglobin range is  Because of the large size of the red blood cells
generally defined as 13.5 to 17.5 grams (g) of hemoglobin produced. Anemia is a medical condition in which the
per deciliter (dL) of blood for men and 12.0 to 15.5 g/dL for blood is low in normal red blood cells.
women.
 Ferritin. SIGNS AND SYMPTOMS
 This protein helps store iron in your body, and a low  The progression of PA is very slow, making it difficult for
level of ferritin usually indicates a low level of stored patients to recognize symptoms because they have grown
iron. accustomed to feeling “unwell.”
Commonly overlooked symptoms include:
TREATMENTS  weakness
 Iron supplementation  headaches
 Take iron tablets on an empty stomach.  chest pain
 Don't take iron with antacids.  weight loss
 Take iron tablets with vitamin C. In rare cases of PA, patients may display neurological
symptoms including:
 Vitamin C improves the absorption of iron. Your doctor  unsteady gait
might recommend taking your iron tablets with a glass
 spasticity peripheral neuropathy
of orange juice or with a vitamin C supplement.
 progressive lesions of the spinal cord
 memory loss
 Treating underlying causes of iron deficiency
 (stiffness and tightness in the muscles)
NURSING CARE
 (damage to the nerves in your arms and legs)
 Choose iron-rich foods
 Choose foods containing vitamin C to enhance iron TESTS AND DIAGNOSTICS
absorption
 complete blood count (CBC) test
 Preventing iron deficiency anemia in infants
 vitamin B-12 deficiency test
 IF deficiency test
 Feed your baby breast milk or iron-fortified formula for
 proof of stomach destruction
the first year.
 Between the ages of 4 and 6 months, start feeding  Hemoglobin
your baby iron-fortified cereals or pureed meats at  hematocrit -
least twice a day to boost iron intake.
 After one year, be sure children don't drink more than  hemoglobin - protein bound to oxygen to carry it
24 ounces of milk a day. throughout the blood
 Hematocrit- used to measure how much space red
blood cells use within the blood

TREATMENT/MANAGEMENT
The treatment for PA is a two-part process:
 first, treat any existing vitamin B-12 deficiency and check
PERNICIOUS ANEMIA for iron-deficiency
 second, lifelong surveillance for long-term consequences  Alcohol interferes with folic acid absorption. It also
Treatment begins with: increases folate excretion through the urine.
 vitamin B-12 injections that are slowly decreased over
time
 blood test for iron deficiency followed by regular blood SIGNS AND SYMPTOMS
tests Symptoms of folic acid deficiency are often subtle. They
 CBC tests to measure serum cobalamin and ferritin levels include:
 blood tests to monitor replacement treatments  fatigue
 grey hair
 mouth sores
Symptoms of long-term damage include:  tongue swelling
 upset stomach  growth problems
 difficulty swallowing Symptoms of anemia caused by folic acid deficiency include:
 weight loss  persistent fatigue
 iron deficiency  lethargy
 pale skin
 tender tongue
FOLIC ACID DEFICIENCY
 irritability
 Folic acid, or folate
 diarrhea
 Folic acid deficiency can cause anemia.
 Folic acid is particularly important in women of TESTS AND DIAGNOSTICS
childbearing age.
 Folic acid deficiency is diagnosed with a blood test.
 A deficiency during pregnancy can lead to birth defects.
 Pregnant women will often have folate levels tested during
Most people get enough folic acid from food.
a prenatal checkup.
 Folic acid, or folate, is a type of B vitamin. It helps to:
TREATMENT AND MANAGEMENT
- repair DNA
 Treatment involves increasing dietary intake of folate.
- make DNA
 You can also take a folic acid supplement.
- produce red blood cells (RBCs)
 Folic acid is frequently combined with other B vitamins in
supplements.
CAUSES
 These may be called vitamin B complexes.
 Diet
 Alcohol intake should be decreased, and completely
 A diet low in fresh fruits, vegetables, and fortified
eliminated for pregnant women.
cereals is the main cause of folic acid deficiency.
 To prevent folic acid deficiency, eat a proper nutritious
 In addition, overcooking your food can sometimes
diet. Foods that contain high amounts of folate include:
destroy the vitamins.
 leafy green vegetables, such as broccoli and spinach
 Folic acid levels in your body can become low in just
 citrus
a few weeks, if you don’t eat enough folate-rich foods.
 fruit such as bananas and melons
 Disease
 tomato juice
 Diseases that affect absorption in the gastrointestinal
 eggs
tract can cause folic acid deficiencies. Such diseases
 beans and legumes
include:
 mushrooms
 Crohn’s disease
 The recommended folate dose is 400 micrograms per day.
 celiac disease
 Women who may become pregnant should take a folate
 Medication Side Effects
supplement. Folate is critical for normal fetal growth.
 Certain medications can cause folic acid deficiency.
These include:
 phenytoin (Dilantin)
SICKLE CELL ANEMIA
 trimethoprim-sulfamethoxazole
 sulfasalazine  inherited form of anemia — a condition in which there
aren't enough healthy red blood cells to carry adequate
 Celiac disease- serious autoimmune condition oxygen throughout your body.
disease that occurs in genetically predisposed people  the red blood cells become rigid and sticky and are
where in the ingestion of gluten leads to damage in shaped like sickles or crescent moons.
the small intestine.  These irregularly shaped cells can get stuck in small blood
 Gluten- substance present in cereals grains (protein) vessels, which can slow or block blood flow and oxygen to
especially wheat, responsible for the elastic texture of parts of the body.
the dough
 A mixture of 2 proteins can cause disease.  Normally, your red blood cells are flexible and round,
moving easily through your blood vessels.
 Excessive Alcohol Intake
 There's no cure for most people with sickle cell anemia.
 However, treatments can relieve pain and help prevent Tests to detect sickle cell genes before birth
further problems associated with sickle cell anemia.  Sickle cell disease can be diagnosed in an unborn baby
by sampling some of the fluid surrounding the baby in the
SIGNS AND SYMPTOMS mother's womb (amniotic fluid) to look for the sickle cell
 Anemia. Sickle cells are fragile. They break apart easily gene.
and die, leaving you without a good supply of red blood
cells. TREATMENTS
 Red blood cells usually live for about 120 days before  Bone marrow transplant offers the only potential cure for
they die and need to be replaced. sickle cell anemia. But finding a donor is difficult and the
 But sickle cells die after an average of less than 20 procedure has serious risks associated with it, including
days. death.
 This results in a lasting shortage of red blood cells  Treatments may include medications to reduce pain and
(anemia). prevent complications, blood transfusions and
 Episodes of pain. Periodic episodes of pain, called crises, supplemental oxygen, as well as a bone marrow
are a major symptom of sickle cell anemia. transplant.
 Hand-foot syndrome. Swollen hands and feet may be the
first signs of sickle cell anemia in babies. MEDICATIONS
 Frequent infections. Sickle cells can damage your spleen,  Antibiotics. Children with sickle cell anemia may begin
an organ that fights infection. taking the antibiotic penicillin when they're about 2 months
 Delayed growth. Red blood cells provide your body with of age and continue taking it until they're at least 5 years
the oxygen and nutrients you need for growth. A shortage old.
of healthy red blood cells can slow growth in infants and  Pain-relieving medications.
children and delay puberty in teenagers.  Hydroxyurea (Droxia, Hydrea). When taken daily,
 Vision problems. Some people with sickle cell anemia hydroxyurea reduces the frequency of painful crises and
experience vision problems. may reduce the need for blood transfusions.
 Hydroxyurea seems to work by stimulating production of
CAUSES fetal hemoglobin — a type of hemoglobin found in
 caused by a mutation in the gene that tells your body to newborns that helps prevent the formation of sickle cells.
make hemoglobin — the red, iron-rich compound that  Assessing stroke risk
gives blood its red color.  Using a special ultrasound machine (transcranial),
 With each pregnancy, two people with sickle cell traits doctors can learn which children have a higher risk of
have: stroke.
 A 25 percent chance of having an unaffected child  Vaccinations to prevent infections
with normal hemoglobin  Blood transfusions
 A 50 percent chance of having a child who also is a  Supplemental oxygen
carrier  Stem cell transplant
 A 25 percent chance of having a child with sickle cell  also called a bone marrow transplant, involves
anemia replacing bone marrow affected by sickle cell anemia
with healthy bone marrow from a donor.
RISK FACTORS
 The risk of inheriting sickle cell anemia comes down to NURSING CARE
genetics.  Take folic acid supplements daily, and choose a healthy
 For a baby to be born with sickle cell anemia, both parents diet.
must carry a sickle cell gene.  Drink plenty of water.
 The gene is more common in families that come from  Avoid temperature extremes.
Africa, India, Mediterranean countries, Saudi Arabia, the  Exercise regularly, but don't overdo it.
Caribbean islands, and South and Central America.  Use over-the-counter medications with caution. Some
 In the United States, it most commonly affects blacks. medications, such as the decongestant pseudoephedrine,
can constrict your blood vessels and make it harder for the
TESTS AND DIAGNOSTICS sickle cells to move through freely.
 New born screening  Fly on airplanes with pressurized cabins. Unpressurized
 A blood test can check for hemoglobin S — the defective aircraft cabins may not provide enough oxygen. Low
form of hemoglobin that underlies sickle cell anemia. oxygen levels can trigger a sickle crisis.
 In adults, a blood sample is drawn from a vein in the arm.  Plan ahead when traveling to high-altitude areas. There is
 In young children and babies, the blood sample is usually less oxygen at higher altitudes, so you may require
collected from a finger or heel. supplemental oxygen to avoid triggering a sickle cell crisis.
 The sample is then sent to a laboratory, where it's
screened for hemoglobin S.
Additional tests APLASTIC ANEMIA
 To confirm any diagnosis, a sample of blood is  condition that occurs when your body stops producing
examined under a microscope to check for large enough new blood cells.
numbers of sickle cells — a marker of the disease.
 Aplastic anemia leaves you feeling fatigued and with a
higher risk of infections and uncontrolled bleeding. TESTS AND DIAGNOSTICS
 aplastic anemia can develop at any age.  Blood tests. Normally, red blood cell, white blood cell and
 Aplastic anemia may occur suddenly, or it can occur platelet levels stay within a certain range.
slowly and get worse over a long period of time.  Bone marrow biopsy. To confirm a diagnosis, you'll need
 Treatment for aplastic anemia may include medications, to undergo a bone marrow biopsy.
blood transfusions or a stem cell transplant.
TREATMENT AND DRUGS
SIGNS AND SYMPTOMS  Blood transfusions- Red blood cell and platelets.
 Fatigue  Stem cell transplant
 Shortness of breath with exertion  Immunosuppressants- cyclosporine (Gengraf, Neoral,
 Rapid or irregular heart rate Sandimmune) and anti-thymocyte globulin
 Pale skin (Thymoglobulin)
 Frequent or prolonged infections  Bone marrow stimulants- sargramostim (Leukine),
 Unexplained or easy bruising filgrastim (Neupogen) and pegfilgrastim (Neulasta), and
 Nosebleeds and bleeding gums epoetin alfa (Epogen, Procrit) — may help stimulate the
 Prolonged bleeding from cuts bone marrow to produce new blood cells.
 Antibiotics, antivirals
 Skin rash
 Dizziness
NURSING CARE
 Headache
 Resting when you need to. Anemia can cause fatigue and
 Aplastic anemia can progress slowly over weeks or
shortness of breath with even mild exertion. Take a break
months, or it may come on suddenly.
and rest when you need to.
 The illness may be brief, or it may become chronic.
 Avoiding contact sports. Because of the risk of bleeding
 Aplastic anemia can be very severe and even fatal.
associated with a low platelet count, avoid activities that
may result in a cut or fall.
CAUSES
 Protecting yourself from germs. You can reduce your risk
 Aplastic anemia develops when damage occurs to your of infections with frequent hand-washing and by avoiding
bone marrow, slowing or shutting down the production of sick people. If you develop a fever or other indicators of an
new blood cells. infection, see your doctor for treatment.
 Bone marrow is a red, spongy material inside your bones
that produces stem cells, which give rise to other cells.
 Stem cells in the bone marrow produce blood cells red THALASSEMIA
cells, white cells and platelets.  is an inherited blood disorder characterized by less
 In aplastic anemia, the bone marrow is described in hemoglobin and fewer red blood cells in your body than
medical terms as aplastic or hypoplastic meaning that it's normal.
empty (aplastic) or contains very few blood cells Major types of thalassemia
(hypoplastic).  Alpha-Thalassemia
 Beta-Thalassemia
Factors that can temporarily or permanently injure bone
marrow and affect blood cell production include:  If you have mild thalassemia, you may not need treatment.
 Radiation and chemotherapy treatments. But, if you have a more severe form of thalassemia, you
 Exposure to toxic chemicals. may need regular blood transfusions.
 Use of certain drugs- such as those used to treat
rheumatoid arthritis and some antibiotics, can cause ALPHA-THALASSEMIA
aplastic anemia.  when the body has a problem producing alpha globin
 Autoimmune disorders.  occurs when the gene that controls the making of alpha
 A viral infection. globins is absent or defective.
 Pregnancy.  When a child has alpha thalassemia, there is a mutation in
 Unknown factors. chromosome 16.

RISK FACTORS  It can be mild to severe and is most commonly found

 Treatment with high-dose radiation or chemotherapy for in people of African, Middle Eastern, Chinese,
cancer Southeast Asian, and, occasionally, Mediterranean
 Exposure to toxic chemicals descent.
 The use of some prescription drugs — such as
chloramphenicol, which is used to treat bacterial  Alpha globin is made on chromosome 16.
infections, and gold compounds used to treat rheumatoid  So, if any gene that tells chromosome 16 to produce
arthritis alpha globin is missing or mutated, less alpha globin is
 Certain blood diseases, autoimmune disorders and made.
serious infections  This affects hemoglobin and decreases the ability of red
 Pregnancy, rarely blood cells to transport oxygen around the body.
 Doctors also might recommend a folic acid supplement for
Types of Alpha Thalassemia kids with hemoglobin H disease to help the body make
 Alpha globin is made by four genes and one or more can new red blood cells.
be mutated or missing, so there are four kinds of alpha  Blood transfusion
thalassemia:
 One missing or abnormal gene makes a child a silent BETA-THALASSEMIA
alpha thalassemia carrier.  when the body has a problem producing beta globin
 Two missing or mutated genes is a condition called  occurs when the gene that controls the production of beta
alpha thalassemia minor or having alpha thalassemia globin is defective.
trait.  Beta thalassemia can be mild to severe and is more
common in people of Mediterranean, African, and
 Silent alpha thalassemia carriers have no signs or Southeast Asian descent.
symptoms of the disease, but are able to pass  A child can only get beta thalassemia by inheriting it from
thalassemia on to their children. his or her parents.
 Children with this condition may have red blood cells  Genes are "building blocks" that play an important role in
that are smaller than normal (microcytosis) and determining physical traits and many other things about
sometimes very slight anemia. us.
 When someone has beta thalassemia, there is a mutation
 People with alpha thalassemia minor usually don't have in chromosome 11.
any symptoms at all, but can pass thalassemia on to their
 Beta globin is made on chromosome 11 (beta globin,
children.
along with alpha globin, is one of the proteins that makes
 The two abnormal genes can be on the same up hemoglobin).
chromosome (called the cis position) or one on each
 So, if one of the genes that tells chromosome 11 to
chromosome (called the trans position).
produce beta globin is altered, less beta globin is made.
 If two genes on the same chromosome are affected, the
 This affects hemoglobin and decreases the ability of red
person can pass along a two-gene defect to his or her
blood cells to transport oxygen around the body.
child.
 This situation is much more common in people of Asian Types of Beta Thalassemia:
descent.
 Beta thalassemia minor, or beta thalassemia trait,
happens when one of the beta globin genes is mutated.
 Three missing or mutated genes is called hemoglobin
 People with this condition typically have very mild
H disease. Signs and symptoms will be moderate to
symptoms and require no treatment, but they can
severe.
pass thalassemia on to their children.
 Four missing or mutated genes is a condition known
 Beta thalassemia major (Cooley's anemia) happens when
as alpha thalassemia major or hydrops fetalis. This
both of the beta globin genes are mutated.
almost always leads to a fetus dying before delivery
 This is the most severe form of beta thalassemia.
or a newborn baby dying shortly after birth.
Babies with beta thalassemia major often seem
healthy immediately after birth but start to develop
SIGNS AND SYMPTOMS
symptoms within the first 2 years of life.
 fatigue, weakness, or shortness of breath
 This condition causes severe symptoms with life-
 a pale appearance or a yellow color to the skin (jaundice) threatening anemia that requires regular blood
 irritability transfusions.
 deformities of the facial bones  Beta thalassemia intermedia may also occur when both of
 slow growth the beta globin genes are mutated, but the mutations are
 a swollen abdomen less severe than those that typically cause beta
 dark urine thalassemia major.
 People with this condition usually have moderately
DIAGNOSTICS severe anemia and sometimes require regular blood
 Blood tests transfusions.
If both parents are carriers of the alpha thalassemia disorder,
doctors can conduct test on a fetus before birth. This is done SIGNS AND SYMPTOMS
through either:  fatigue, weakness, or shortness of breath
 chorionic vilius sampling, which takes place about 11  a pale appearance or a yellow color to the skin (jaundice)
weeks into pregnancy and involves removing a tiny piece  irritability
of the placenta for testing  deformities of the facial bones
 amniocentesis, which is usually done about 16 weeks  slow growth
into the pregnancy and involves removing a sample of  a swollen abdomen
the fluid that surrounds the fetus  dark urine
 Babies who begin to show symptoms of beta thalassemia
TREATMENT
after a few healthy months mAay fail to grow normally
(failure to thrive);
 have trouble feeding; and have episodes of fever,  People with genetic mutations and familial types of
diarrhea, and other intestinal problems. polycythemia and certain hemoglobin abnormalities also
carry risk factors for this condition as mentioned in earlier
DIAGNOSTICS/TREATMENTS sections.
 Same as alpha thalassemia
SIGNS AND SYMPTOMS
NURSING CARE  weakness, fatigue, headache, itching, bruising, joint pain,
 Avoid excess iron. dizziness, or abdominal pain
 Eat a healthy diet.  Polycythemia vera
 Avoid infections.  bleeding problems or clotting events
 Itching after showers or baths
 Joint pains
POLYCYTHEMIA  tender redness of the palms and soles is called
 an increased number of red blood cells in the blood. erythromelalgia.
 Hemoglobin levels greater than 16.5 g/dL (grams per
deciliter) in women and greater than 18.5 g/dL in men Secondary Polycythemia Symptoms
suggest polycythemia.  more closely attributed to the underlying condition, such
 In terms of hematocrit, a value greater than 48 in women as, chronic lung disease
and 52 in men is indicative of polycythemia.  shortness of breath
 Production of red blood cells (erythropoiesis) occurs in the  chronic cough,
bone marrow and is regulated in a series of specific steps.  Sleep disturbance (sleep apnea)
 One of the important enzymes regulating this process is  dizziness
called erythropoietin (Epo).  poor exercise tolerance, or fatigue may be common in
 The majority of Epo is produced and released by the patients with polycythemia
kidneys, and a smaller portion is released by the liver.
 Polycythemia can result from internal problems with the If Polycythemia Is Related To Kidney Cancer, Liver Cancer, Or
production of red blood cells. Other Erythropoietin Secreting Tumors:
 If polycythemia is caused due to another underlying  weight loss
medical problem, it is referred to as secondary  abdominal pain or fullness,
polycythemia.  Jaundice may be predominant.

CAUSES DIAGNOSTICS
 Two main conditions that belong to this category are:  CBC
 polycythemia vera (PV or polycythemia rubra vera  A chest X-ray, electrocardiogram (EKG), and
[PRV]) and echocardiogram may be performed to screen for lung
 primary familial and congenital polycythemia (PFCP). disease or heart disease.
 Polycythemia vera (PV) is related to a genetic mutation in  Hemoglobin analysis
the JAK2 gene, which is thought to increase the sensitivity
of bone marrow cells to Epo, resulting in increased red TREATMENT
blood cell production.  phlebotomy (drawing blood or blood letting) is the most
 Levels of other types of blood cells (white blood cells essential part of the treatment. The recommended
and platelets) are also often increased in this hematocrit of less than 45 in men and less than 42 in
condition. women is the goal of phlebotomy.
 Primary familial and congenital polycythemia (PFCP) is a  hydroxyurea (Hydrea)
condition related to a mutation in the EPOR gene and  Aspirin
causes increased production of red blood cells in
response to Epo. NURSING CARE
 Polycythemia due to a secondary cause such as long
RISK FACTORS standing smoking or exposure to carbon monoxide can be
 Hypoxia from long standing (chronic) lung disease and prevented by omitting these risks.
smoking are common causes of polycythemia.  Reducing risk factors for heart failure, such as, controlling
 Chronic carbon monoxide (CO) exposure can also be risk high blood pressure and diabetes mellitus, can potentially
factor for polycythemia reduce the risk of polycythemia.
 Chronic carbon monoxide exposure is a risk factor for
people working in underground tunnels or parking
garages, cab drivers in highly polluted and congested
cities, or workers in factories with exposure to engine
exhaust.
 People living at high altitudes may also be at risk of
developing polycythemia due to low environmental oxygen
levels.
DISORDERS OF THE WBC NURSING CARE
 Good hygiene, including frequent hand washing and good
NEUTROPENIA dental care, such as regular tooth brushing and flossing
 an abnormally low count of neutrophils, a  Avoiding contact with sick people
 The lower your neutrophil count, the more vulnerable you  Always wearing shoes
are to infectious diseases.  Cleaning cuts and scrapes, then covering them with a
 neutropenia fewer than about 500 cells per microliter of bandage
blood.  Using an electric shaver rather than a razor
 Avoiding animal waste and, when possible, not changing
 bacteria normally present in your mouth and digestive infants' diapers
tract can cause infections.  Avoiding unpasteurized dairy foods; undercooked meat;
and raw fruits, vegetables, grains, nuts, and honey
CAUSES  Staying out of hot tubs, ponds, and rivers
 Cancer or other diseases that damage bone marrow
 Congenital disorders characterized by poor bone marrow
function NEUTROPHILIA
 Viral infections that disrupt bone marrow function  Neutrophils are white blood cells (WBC) that move from
 Autoimmune disorders that destroy neutrophils or bone the blood into the cells to kill invading bacteria and fungi. 
marrow cells  Neutrophilia is the most common form of leukocytosis
 Overwhelming infections that use up neutrophils faster  This results in a neutrophil count of over 8,000.
than they can be produced Drugs that destroy neutrophils
or damage bone marrow  leukocytosis a condition involving an increased
number of leukocytes in the blood. 
Possible Causes Of Neutropenia Include:
 Alcoholism or chronic alcohol use CAUSES
 Aplastic anemia  Acute infection. Infection such as caused by certain
 Chemotherapy agents would trigger neutrophilia.
 Chronic idiopathic neutropenia in adults  Fungal infections are also included in the list.
 Drugs, such as antibiotics and diuretics  Inflammation. There are noninfectious inflammations that
 Hepatitis A would trigger increase of neutrophils.
 Hepatitis B
 Hepatitis C  Bacterial and viral infections are just some of the
 HIV/AIDS common neutrophilia-causing infections.
 These inflammation-triggering conditions are burns;
SIGNS AND SYMPTOMS post operation, autoimmune conditions, and an acute
 symptoms from infection or the underlying problem attack of myocardial infarction are just some of the
causing the neutropenia. states that induce elevation of neutrophil levels.
 Infections can occur as a complication of neutropenia.  Trauma or tissue damage induces an inflammatory
They occur most often in the mucous membranes, such reaction.
as the inside of the mouth and the skin.
These infections can appear as:  Cigarette smoking. This can induce elevation of
 Fever (most common sign of infection) neutrophils in the system due to the inflammation that it
results to.
 Ulcers
 Stress. Basically, there shall be increased neutrophils
 Abscesses (collections of pus)
once stress strikes such as instances where a person is
 Rashes
anxious and has a seizure episode.
 Wounds that take a long time to heal
 Drugs. Taking of certain drugs seem to elevate WBC
count and those are corticosteroids. Malignancy such as
LABORATORY AND DIAGNOSTICS
(cancer) carcinoma, sarcoma, etc. could cause
 Blood Test
neutrophilia.
TREATMENTS SIGNS AND SYMPTOMS
 Antibiotics for bacterial infections  Infection. This is in congruence with the systemic
 Drugs to suppress the immune system inflammatory response from an acquired infection.
 A treatment called granulocyte colony-stimulating factor  Bleeding leading to hypotension, tachycardia and most
(G-CSF). This stimulates the bone marrow to produce probably sepsis.
more white blood cells  Hypothermia or decreased body temperature.
 Treating an underlying infection  Tachypnea and dyspnea would most likely happen. These
 Stem cell transplants are respiratory-related symptoms.

DIAGNOSTICS AND TESTS

 CBC
 ANC (Absolute Neutrophil Count) Acute leukemia requires aggressive, timely
TREATMENT ANDF MANAGEMENT treatment.
 Referral to a hematologist. This is necessary in order to  Chronic leukemia. This type of leukemia involves more
identify certain conditions, such as blood problems. mature blood cells.
 There might be abnormal enlargement of the spleen  These blood cells replicate or accumulate more
that is why neutrophilia is present. slowly and can function normally for a period of time.
 Bone marrow aspiration. This shall identify presence of  Some forms of chronic leukemia initially produce no
hematological problem. early symptoms and can go unnoticed or
 Bone marrow depression may be present that is why undiagnosed for years.
testing the sample from the bone marrow aspiration is The second type of classification is by type of white blood cell
necessary. affected:
 Close supervision of the blood results are indeed  Lymphocytic leukemia. This type of leukemia affects
necessary in order to check progress of the condition. This the lymphoid cells (lymphocytes), which form
is needed to be monitored so that success of the lymphoid or lymphatic tissue.
treatment course can be attained.  Myelogenous leukemia. This type of leukemia affects
 Discuss with the doctor the need of taking drugs that could the myeloid cells. Myeloid cells give rise to red blood
cause neutrophilia. cells, white blood cells and platelet-producing cells.
 Asking for alternative medications from such should be
helpful in preventing the disease process. TYPES OF LEUKEMIA
 Maintain a healthy lifestyle so that one can avoid acquiring  Acute lymphocytic leukemia (ALL). This is the most
acute infections that are highly causative of neutrophilia. common type of leukemia in young children. ALL can also
 Having yearly influenza shots can also avoid occur in adults.
acquiring viral infections.  Acute myelogenous leukemia (AML). AML is a common
 Reduce or slowly stop bad habits that can alter one’s type of leukemia. It occurs in children and adults. AML is
natural defenses of the body is a preventive measure the most common type of acute leukemia in adults.
for neutrophilia.  Chronic lymphocytic leukemia (CLL). With CLL, the most
common chronic adult leukemia, you may feel well for
years without needing treatment.
LEUKEMIA  Chronic myelogenous leukemia (CML). This type of
 cancer of the body's blood-forming tissues, including the leukemia mainly affects adults.
bone marrow and the lymphatic system.  A person with CML may have few or no symptoms for
 Leukemia usually starts in the white blood cells. months or years before entering a phase in which the
 Your white blood cells are potent infection fighters — they leukemia cells grow more quickly.
normally grow and divide in an orderly way, as your body
needs them. RISK FACTORS
 But in people with leukemia, the bone marrow produces  Previous cancer treatment. People who've had certain
abnormal white blood cells, which don't function properly. types of chemotherapy and radiation therapy for other
cancers have an increased risk of developing certain
SIGNS AND SYMPTOMS types of leukemia.
 Fever or chills  Genetic disorders. Genetic abnormalities seem to play a
 Persistent fatigue, weakness role in the development of leukemia. Certain genetic
 Frequent or severe infections disorders, such as Down syndrome, are associated with
 Losing weight without trying increased risk of leukemia.
 Swollen lymph nodes, enlarged liver or spleen  Certain blood disorders. People who have been
diagnosed with certain blood disorders, such as
 Easy bleeding or bruising
myelodysplastic syndromes, may have an increased risk
 Recurrent nosebleeds
of leukemia.
 Tiny red spots in your skin (petechiae)
 Exposure to high levels of radiation. People exposed to
 Excessive sweating, especially at night
very high levels of radiation, such as survivors of a nuclear
 Bone pain or tenderness reactor accident, have an increased risk of developing
leukemia.
CAUSES  Exposure to certain chemicals. Exposure to certain
 occur when some blood cells acquire mutations in their chemicals, such as benzene — which is found in gasoline
DNA . and is used by the chemical industry — also is linked to
increased risk of some kinds of leukemia.
CLASSIFICATION OF LEUKEMIA  Smoking. Smoking cigarettes increases the risk of acute
The first type of classification is by how fast the leukemia myelogenous leukemia.
progresses:
 Family history of leukemia. If members of your family have
 Acute leukemia. In acute leukemia, the abnormal blood been diagnosed with leukemia, your risk of the disease
cells are immature blood cells (blasts). may be increased.
 They can't carry out their normal work, and they
multiply rapidly, so the disease worsens quickly.
TESTS AND DIAGNOSTICS
 Physical exam. Your doctor will look for physical signs of
leukemia, such as pale skin from anemia and swelling of
your lymph nodes, liver and spleen.
 Blood tests. By looking at a sample of your blood, your
doctor can determine if you have abnormal levels of white
blood cells or platelets — which may suggest leukemia.
 Bone marrow test. Your doctor may recommend a
procedure to remove a sample of bone marrow from your
hipbone.
 The bone marrow is removed using a long, thin
needle.
 The sample is sent to a laboratory to look for
leukemia cells.
 Specialized tests of your leukemia cells may reveal
certain characteristics that are used to determine your
treatment options.

TREATMENT AND DRUGS

 Chemotherapy. Chemotherapy is the major form of
treatment for leukemia. This drug treatment uses
chemicals to kill leukemia cells.
 Biological therapy. Biological therapy works by helping
your immune system recognize and attack leukemia cells.
 Targeted therapy. Targeted therapy uses drugs that attack
specific vulnerabilities within your cancer cells.
 For example, the drug imatinib (Gleevec) stops the
action of a protein within the leukemia cells of people
with chronic myelogenous leukemia. This can help
control the disease.
 Radiation therapy. Radiation therapy uses X-rays or other
high-energy beams to damage leukemia cells and stop
their growth.
 Stem cell transplant. A stem cell transplant is a procedure
to replace your diseased bone marrow with healthy bone
marrow.

NURSING CARE
 Learn enough about leukemia to make decisions about
your care.
 Keep friends and family close.
 Find someone to talk with.

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