Hematology Disorders

Contents:

1. Sickle cell anemia

2. Immuno hemolytic Anaemia
3. Iron deficiency anemia
4. Vitamin B12 & Folic acid deficiency anemia
5. Aplastic anemia
6. Polycythemia
Sickle cell anemia
 Sickle cell anemia is a severe hemolytic
anemia that results from inheritance of the
sickle hemoglobin gene. This gene causes
the hemoglobin molecule to be defective.
The sickle hemoglobin (HbS) acquires a
crystal-like formation when exposed to low
oxygen tension. The oxygen level in venous
blood can be low enough to cause this
change;
 Consequently, the RBC containing (HbS)
loses its round, very pliable, biconcave disk
shape and becomes deformed, rigid, and
sickle-shaped .
 These long, rigid RBCs can adhere to the
endothelium of small vessels; when they pile
up against each other, blood flow to a region
or an organ may be reduced (Hoffman, et al.,
2000). If ischemia or infarction results, the
patient may have pain, swelling, and fever.
SICKLE CELL CRISIS


There are three types of sickle cell crisis in the adult population.

The most common is the very painful sickle crisis, which

results from tissue hypoxia and necrosis due to inadequate
blood flow to a specific region of tissue or organ.
Aplastic crisis results from infection with the human

parvovirus. The hemoglobin level falls rapidly and the marrow

cannot compensate, as evidenced by an absence of
reticulocytes.
Sequestration crisis results when other organs pool the sickled

cells. Although the spleen is the most common organ

responsible for sequestration in children, by 10 years of age
most children with sickle cell anemia have had a splenic
infarction and the spleen is then no longer functional
(autosplenectomy). In adults, the common organs involved in
sequestration are the liver and, more seriously, the lungs.
ACUTE CHEST SYNDROME

Acute chest syndrome is manifested by a

rapidly falling hemoglobin level, tachycardia,
fever, and bilateral infiltrates seen on the
chest x-ray. These signs often mimic
infection; in fact, recent studies have
identified infection as a major cause of acute
chest syndrome (Vichinsky, et al., 2000).
Another common cause is
pulmonary fat embolism.
Clinical Manifestations
 • Acute pain (especially back, chest, and long
bones) from vascular occlusion of the small
vessels as the sickled cells clump
• Fever as body responds to acute sickling
episode and accompanying provoking event
• Painful, swollen joints due to vaso-occlusive
process
• Fatigue due to chronic anemia
• Stroke (cerebrovascular accident) due to vaso-
occlusive process
• Enlarged liver (hepatomegaly)
• Enlarged heart and systolic murmur
INTERPRETING TEST RESULTS

• Low RBC count due to chronic hemolytic anemia;

the RBCs have a shorter
lifespan.
• Elevated WBCs.
• Increased reticulocytes.
• Presence of Howell-Jolly bodies and target cells.
• Sickle cells appear in blood smear.
• Indirect bilirubin level elevated.
• Hemoglobin electrophoresis shows majority
hemoglobin S (80 to 98 percent).
TREATMENT
During acute episodes pain control, hydration, and
oxygenation are the focus of treatment. The
underlying cause that sent the patient into crisis will
also need to be treated concurrently.
• Administer analgesics to alleviate the pain associated
with the vaso-occlusive process:
• narcotic pain control necessary when pain is severe
• Warm compresses on joint.
• Blood transfusion of packed RBC when anemia
indicates.
• Supplemental oxygen if hypoxic.
• Adequate hydration, using IV fluids.
• Treat infections.
COLLABORATIVE PROBLEMS/
POTENTIAL COMPLICATIONS
Based on the assessment data, potential complications may
include:
• Hypoxia, ischemia, infection, and poor wound healing
leading to skin breakdown and ulcers
• Dehydration
• Cerebrovascular accident (CVA, brain attack, stroke)
• Anemia
• Renal dysfunction
• Heart failure, pulmonary hypertension, and acute chest
syndrome
• Impotence
• Poor compliance
• Substance abuse related to poorly managed chronic pain
NURSING INTERVENTION

• Increase fluid intake.

• Monitor IV fluids.
• Monitor pain control.
• Record fluid intake and output to monitor renal function.
• Administer supplemental O2 to increase available
oxygen.
• Explain to the patient:
• Avoid the cold.
• No cold compresses.
• Plan for rest periods during the day.
NURSING DIAGNOSES


Based on the assessment data, major nursing

diagnoses for the
patient with sickle cell crisis may include:
• Acute pain related to tissue hypoxia due to
agglutination of
sickled cells within blood vessels
• Risk for infection
• Risk for powerlessness related to illness-induced
helplessness
• Deficient knowledge regarding sickle crisis
prevention
2:IMMUNE HEMOLYTIC ANEMIA

 Hemolytic anemias can result from exposure of

the RBC to antibodies. Alloantibodies (i.e,
antibodies against the host, or “self”) result from
the immunization of an individual with foreign
antigens
(eg, the immunization of an Rh-negative person
with Rh-positive blood).
 The most common type of alloimmune hemolytic
anemia in adults results from
a hemolytic transfusion reaction.
 Autoantibodies are developed by an
individual for varying reasons. In many
instances, the person’s immune system is
dysfunctional, so that it falsely recognizes its
own RBCs as foreign and
produces antibodies against them.
 Another mechanism is a deficiency in

suppressor lymphocytes, which normally

prevent antibody formation against a
person’s own antigens.
classification
 Autoimmune hemolytic anemias can be
classified based on the body temperature
involved when the antibodies react with the
RBC antigen.
 Warm-body antibodies bind to RBCs most

actively in warm conditions (37°C).

 cold-body antibodies react in cold (0°C).
Clinical Manifestations

 The hemolysis may be very mild, so that the

patient’s marrow compensates adequately and
the patient is asymptomatic.
 Most patients complain of fatigue
and dizziness.
 Splenomegaly is the most common physical
finding, occurring in more than 80% of patients;
 hepatomegaly,
 lymphadenopathy, and jaundice are also common.
Assessment and Diagnostic Findings

 The laboratory tests show a low hemoglobin level

and hematocrit.
RBCs appear abnormal; spherocytes are
common.
 serum bilirubin level is elevated, and if the
hemolysis is severe, the haptoglobin level is low
or absent.
 The Coombs test (also referred to as the direct
antiglobulin test [DAT]), which detects antibodies
on the surface of RBCs, shows a positive result.
 Medical Management

 high doses of corticosteroids (1 mg/kg per day) until hemolysis

decreases.
 In severe cases, blood transfusions may be required.
 If neither corticosteroid therapy nor Splenectomy is successful,

immunosuppressive agents may be administered.

 Immunoglobulin administration
 If corticosteroids or immunosuppressive agents are used, the

taper must be very gradual to prevent a rebound “hyperimmune”

response and exacerbation of the hemolysis.
 For patients with cold-antibody hemolytic anemia, treatment

may not be required, other than to advise the patient to keep

warm; relocation to a warm climate may be necessary.
NURSING ALERT

 It can be difficult to cross-match blood when

antibodies are present. If imperfectly cross-
matched RBCs must be transfused, the nurse
begins the infusion very slowly (10 to 15 mL
over 20 to 30 minutes) and monitors the
patient very closely for signs and symptoms
of a hemolytic transfusion reaction
3:IRON DEFICIENCY ANEMIA
 Iron deficiency anemia typically results when
the intake of dietary iron is inadequate for
hemoglobin synthesis. The body can store
about one fourth to one third of its iron, and it
is not until those stores are depleted that iron
deficiency anemia actually begins to develop.
 Iron deficiency anemia is the most common
type of anemia in all age groups, and it is the
most common anemia in the
world.
Causes:
 bleeding (from ulcers, gastritis, inflammatory
bowel disease, or gastrointestinal tumors).
 menorrhagia (excessive menstrual bleeding) and
pregnancy with inadequate iron
supplementation.
 Patients with chronic alcoholism
often have chronic blood loss from the
gastrointestinal tract.
 Other causes include iron malabsorption, as is
seen after gastrectomy or with celiac disease.
Clinical Manifestations
• Weakness due to anemia and tissue hypoxia
• Pallor due to decreased amount of oxygen
getting to surface tissues
• Fatigue due to anemia and hypoxemia
• Koilonychia—thin, concave nails raised at
edges, also called spoon nails
• Tachycardia and tachypnea on exertion due
to increased demand for oxygen
Assessment and Diagnostic Findings
• Decrease in serum hemoglobin as fewer RBCs are made.
• Serum ferritin is low.
• Mean corpuscular volume (MCV) initially normal, then low
—microcytic anemia.
• Mean corpuscular hemoglobin (MCH) initially normal, then
low—hypochromic anemia.
• Serum iron level is low.
• Serum iron-binding capacity is increased.
• Transferrin saturation decreases.
• Peripheral blood smear shows poikilocytosis (red blood
cells of different shapes).
• Platelet count may increase.
Medical Management

 Except in the case of pregnancy, the cause of iron

deficiency should be investigated.
 oral iron preparations—ferrous sulfate, ferrous
gluconate, and ferrous fumarate.
 intravenous or intramuscular administration of
iron dextran.
 Increase dietary intake of iron
Nursing Management

• Monitor intake and output.

• Monitor vital signs for tachycardia or
tachypnea.
• Monitor for reactions to parenteral iron
therapy.
• Explain to the patient:
• Check for bleeding.
• Increase iron in diet.
• Teach dietary sources of iron.
4:MEGALOBLASTIC ANEMIAS
(Vitamin B12 & folic acid deficiency anemias)

In the anemias caused by deficiencies of

vitamin B12 or folic acid, identical bone
marrow and peripheral blood changes occur,
because both vitamins are essential for
normal DNA synthesis. In either anemia, the
RBCs that are produced are abnormally large
and are called megaloblastic RBCs.
Pathophysiology
FOLIC ACID DEFICIENCY

Folic acid, a vitamin that is necessary for

normal RBC production, is stored as
compounds referred to as folates.
The folate stores in the body are much
smaller than those of vitamin B12, and they
are quickly depleted when the dietary intake
of folate is deficient (within 4 months).
Folate deficiency occurs
 people who rarely eat uncooked
vegetables.
 Alcohol increases folic acid
requirements,
patients with chronic hemolytic anemias
 In women who are pregnant
 Some patients with malabsorptive
diseases of the small bowel
VITAMIN B12 DEFICIENCY

intrinsic factor is normally secreted by cells

within the gastric mucosa; normally it binds with
the dietary vitamin B12 and travels with it to the
ileum, where the vitamin is absorbed. Without
intrinsic factor, orally consumed vitamin B12
cannot be absorbed, and RBC production is
eventually diminished.
A deficiency of vitamin B12
can occur in several ways. Inadequate
dietary intake is rare but can develop in
strict vegetarians who consume no meat or
dairy products.
 Crohn’s disease
 ileal resection or gastrectomy.
 absence of intrinsic factor
HALLMARK SIGNS AND SYMPTOMS

• Pallor due to anemia

• Weakness and fatigue due to anemia
• Tingling in hands and feet—“stocking-glove paresthesia”—due to
bilateral demyelination of dorsal and lateral columns of spinal cord nerves
• Diminished vibratory and position sense
• Poor balance due to effect on cerebral function
• Dementia appears later in the disease
• Atrophic glossitis—beefy red tongue
• Nausea may lead to anorexia and weight loss
• Premature graying of hair
Symptoms of folic acid and vitamin B12 deficiencies are similar, and the
two anemias may coexist. However, the neurologic manifestations of
vitamin B12 deficiency do not occur with folic acid deficiency, and they
persist if B12 is not replaced.
INTERPRETING TEST RESULTS

• Decreased hemoglobin due to decreased production of

RBCs.
• Increased MCV—macrocytic anemia.
• Positive Schilling test due to decrease in intrinsic factor.
• Decreased amount of hydrochloric acid in the stomach
(hypochlorhydria) due to changes within the parietal cells
of the gastric mucosa.
• Positive Romberg test due to ataxia and neurologic
changes.
• Diminished sensation when testing for vibration,
position sense, or propioception of extremities.
Medical management
Lifelong replacement with vitamin B12 will correct
the anemia and improve the neurologic changes
that have occurred. Initially the patient is given
weekly injections of B 12 to combat the deficiency.
The injections eventually become monthly for
lifelong maintenance.
Oral supplementation is not effective in these
patients because they cannot adequately absorb
vitamin B12 due to insufficient intrinsic factor.
• Administer vitamin B 12 by IM injection.
• Transfusion of packed RBC if anemia is severe.
Cont….
 Folate deficiency is treated by increasing the
amount of folic acid in the diet and
administering 1 mg of folic acid daily. Folic
acid is administered intramuscularly only for
people with malabsorption problems .
NURSING ALERT
 Even when the anemia is severe, RBC transfusions
should not be used because the patient’s body
has compensated over time by expanding the
total blood volume.
Administration of blood transfusions to such
patients, particularly those who are elderly or
who have cardiac dysfunction, can precipitate
pulmonary edema. If transfusions are required,
the RBCs should be transfused slowly, with
careful attention to signs and
symptoms of fluid overload.
5:Aplastic anemia
The bone marrow stops producing a sufficient
amount of RBC, WBC, and platelets, thereby
increasing the risk of infection and hemorrhage.
The red cells remaining in circulation are normal
in size and color.
Causes:
 The cause may also be unknown or idiopathic.
This may be due to
 chemical exposure,
 high dose radiation exposure,
 exposure to toxins.
 Cancer treatments such as radiation therapy and
chemotherapeutic agents may suppress bone
marrow function, which will result in anemia (low
RBC), thrombocytopenia (low platelets), and
leukopenia (low WBC).
PROGNOSIS


The bone marrow dysfunction may be slow-

onset or sudden. The lifespan of the
RBC is longer than the platelets and WBC, so
the anemia may show up later than
the effects of losing the other cells. Some
exposures to toxic agents or medications
are severe and potentially fatal in susceptible
individuals.
HALLMARK SIGNS AND SYMPTOMS

• Fatigue due to hypoxemia

• Weakness due to tissue hypoxia
• Pallor due to lack of oxygen reaching superficial tissues
due to anemia
• Infections due to low white blood cell production, causing
decreased ability
to fight infection
• Bruising (ecchymosis), and tiny subcutaneous (SC)
hemorrhages (petechiae) due to decrease in platelets,
altering clotting ability
• Bleeding from mucous membranes (GI tract, mouth, nose,
vagina)
INTERPRETING TEST RESULTS


• Low hemoglobin.
• Low hematocrit.
• Low RBC count.
• Thrombocytopenia—low platelet count.
• Leukopenia—low WBC.
• Reticulocyte count low.
• Positive fecal occult blood test.
• Decreased cell counts in bone marrow
biopsy as body stops producing.
TREATMENT

• Administer hematopoietic growth factor to correct anemia

in patients with low erythropoietin levels:
• erythropoietin by SC injection
• Administer human granulocyte colony-stimulating factor
(G-CSF) to correct low WBC levels:
• Packed RBC transfusions when anemia is symptomatic.
• Platelet transfusion for severe bleeding.
• Bone marrow transplant replaces functioning stem cells.
• Administer immunosuppressive drugs,, and
corticosteroids.
• Splenectomy when spleen is enlarged and destroying
RBCs.
NURSING INTERVENTION


• Monitor vital signs for changes.

• Record intake and output of fluids.
• Protect patient from falls.
• Avoid IM injections due to altered clotting ability.
• Explain to the patient:
• No aspirin due to effect on platelet aggregation (clotting ability).
• Plan to take rest periods during activities due to fatigue.
• Only use an electric razor to decrease risk of bleeding due to
decreased
platelet count.
• Call your physician, nurse practitioner, or physician assistant for
signs of
bleeding or bruising.
6: Polycythemia

 Polycythemia refers to an increased volume of

RBCs. It is a term used when the hematocrit is
elevated (to more than 55% in males, more
than 50% in females). Dehydration (decreased
volume of plasma) can cause an elevated
hematocrit, but not typically to the level to be
considered polycythemia.
Classifications:

 Polycythemia is classified as either primary or

secondary.
1:POLYCYTHEMIA VERA
Polycythemia vera, or primary polycythemia, is
a proliferative disorder in which the myeloid
stem cells seem to have escaped normal
control mechanisms.
 The RBC elevation is predominant; the
hematocrit can exceed 60%. This phase can
last for an extended period
(10 years or longer).
 The spleen resumes its embryonic function

of hematopoiesis and enlarges.

 the bone marrow may become fibrotic, with

a resultant inability to produce as many

cells (“burnt out” or spent phase).
2:SECONDARY POLYCYTHEMIA

Secondary polycythemia is caused by

excessive production of erythropoietin. This
may occur in response to a reduced amount
of oxygen, which acts as a hypoxic stimulus,
as in cigarette smoking,
chronic obstructive pulmonary disease, or
cyanotic heart disease, or in non pathologic
conditions such as high altitude
Clinical manifestations:
 • Facial skin and mucous membranes dark and flushed
(plethora)
• Hypertension due to increased peripheral vascular
resistance and thickening of the blood
• Itching worse after warm shower due to histamine
• Headache and difficulty concentrating
• Vision blurred, tinnitus (ringing in ears), and hearing
changes
• Thrombosis due to vascular stasis
• Spleen enlargement (splenomegaly)
• Tissue hypoxia and possible infarction of heart,
spleen, kidneys, and brain due to thrombosis
INTERPRETING TEST RESULTS

• Increased RBC count.

• Increased hemoglobin.
• Increased hematocrit level.
• Increased WBC count.
• Increased platelet count.
• Increased uric acid level.
• Increased potassium.
• Increased vitamin B 12 level.
• Bone marrow panhyperplasia; iron stores
absent.
TREATMENT

Treatment is aimed at maintaining

bloodflow to the smaller vessels and
diminishing the amount of excess blood
cells being made by the bone marrow.
• Periodic scheduled phlebotomy—the
removal of 500 ml of blood—to reduce
the hematocrit level to below 45; may be
done weekly.
Cont….
• Adequate hydration.
• Anticoagulants such as aspirin.
• Administer myelosuppressive medication:
• Administer medication to lower uric acid
• Radiation therapy.
• Antihistamine for pruritis.
Management of secondary polycythemia may
not be necessary; when it is, it involves
treating the primary problem.