Management

of Patients
with
Nonmalignant
Hematologic
Disorders
P R E P A R E D B Y: L O U I E J A Y A D L A O , M A N , R N
Learning Outcomes
• Differentiate between the hypoproliferative and the hemolytic anemias and
compare and contrast the physiologic mechanisms, clinical manifestation,
medical management, and nursing interventions for each.
• Use the nursing process as a framework for care of patients with anemia.
• Apply the nursing process as a framework for care of patients with sickle cell
crisis.
• Describe the process involved in neutropenia and lymphoma and the general
principles of medical and nursing management of patient with these disorders.
• Specify the medical and nursing management of patients with bleeding and
thrombotic disorders.
Anemia
It is a condition in which the
hemoglobin concentration
is lower than normal; it
reflects the presence of
fewer than the normal
number of erythrocytes
(RBCs) within the
circulation.
 1. Hypoproliferative
(Resuluting in Defective

Classificati
RBC Production)
• Iron Deficiency

on of
• Vitamin B12 Deficiency
(Megaloblastic)

Anemia
• Folate Deficiency
(Megalobastic)
• Decreased erythropoietin
production (from renal
dysfunction)
Classificati
• Bleeding (Resulting
from RBC Loss)
• Epistaxis

on of • Trauma
• Bleeding from

Anemia genitourinary tract

(menorrhagia) µ
Classificati • Hemolytic Anemia
(Resulting from RBC

on of
destruction)
• Altered erythropoiesis
(sickle cell disease

Anemia and thalassemia,

G6PDµ)
Clinic Fatigue – due to decreased oxygen delivery to tissues

al Pallor– pale skin, mucous membranes, and nail beds

Shortness of breath (Dyspnea) – Especially during physical

Manif activity
Dizziness of lightheadedness - As a result of reduced
estati oxygen supply to the brain
Tachycardia – The heart compensates for reduced oxygen by
on pumping faster
Cold hands and feet - poor circulation due to decreased red
blood cells

Brittle nails – associated with chronic anemia

Cognitive impairment – Difficulty concentrating or memory

issues.
Complicatio
ns
• Heart failure
• Paresthesia
• Delirium
 Medical Management of anemia is

Manageme directed toward correcting

or controlling the cause of

nt
the anemia.
Nursing Management

Managing Fatigue

Maintaining Adequate Nutrition

Activity Intolerance
Monitoring and Managing Potential
Complications
Iron Deficiency Anemia

Iron deficiency
anemia typically Iron deficiency
results when the anemia is the most
intake of dietary common type of
iron is inadequate anemia in all age
for hemoglobin groups.
synthesis.
Causes:
Bleeding from ulcers, gastritis, inflammatory bowel disease or GI
tumors.
Menorrhagia (excessive menstrual bleeding)

Pregnancy

Patients with chronic alcoholism, taking ASPARIN, steroids, or

NSAIDS
Iron malabsorption as is seen after gastrectomy, bariatric
surgery, or with celiac or other inflammatory bowel disease;
Clinical
Manifestatio
n
• Smooth, red tongue
(beefy tongue)
• Brittle and ridged
nails
• Angular cheilosis
Assessment The definitive method of
establishing the diagnosis of iron
and deficiency anemia is bone
marrow aspiration
Diagnostic
Findings Hemoglobin (Hgb): Low
hemoglobin levels confirm anemia.
In iron deficiency anemia,
hemoglobin levels are typically
reduced.

Hematocrit (Hct): Measures the

percentage of red blood cells in the
blood. It is usually decreased in
anemia.
Assessme Mean Corpuscular Volume
(MCV): This measures the
size of red blood cells. In iron
nt and deficiency anemia, the red
blood cells are smaller than

Diagnosti
normal (microcytic), resulting
in a low MCV.

c Findings Red Cell Distribution

Width (RDW): Measures the
variation in the size of red
blood cells. In iron deficiency
anemia, the RDW is often
elevated, indicating a mix of
small and normal-sized red
blood cells.
Assessme
Mean Corpuscular
Hemoglobin (MCH): This
measures the amount of

nt and hemoglobin in each red blood

cell. In iron deficiency anemia,
the MCH is reduced, indicating

Diagnosti hypochromic (pale) red blood

cells.

c Findings Low Ferritin: Ferritin is a protein

that stores iron, and low serum
ferritin levels are a key indicator
of iron deficiency. Ferritin levels
typically drop before hemoglobin
levels fall, making it one of the
earliest markers of iron
deficiency anemia.
Assessme
nt and
Total Iron-Binding Capacity (TIBC):

Diagnosti
c Findings Increased TIBC: This measures the
blood's capacity to bind iron with
transferrin, a protein that transports iron.
In iron deficiency anemia, TIBC is
typically elevated because the body
produces more transferrin to try to
capture more iron.
Medical Stool specimens should be tested for occult

Managem blood

ent People 50 years of age or older should have

a colonoscopy, endoscopy, or x-ray
examination of the GI tract to detect
ulcerations, gastritis, polyps, or cancer

Oral iron supplementation is considered to

be the primary mode of treating iron
deficiency anemia. Several oral iron
preparations—ferrous sulfate, ferrous
gluconate, and ferrous fumarate—are
available for treating iron deficiency anemia.
 • The hemoglobin level
may increase in only a
few weeks, and the

Medical anemia can be corrected

in a few months. Iron

Manageme store replenishment

takes much longer, so

nt
the patient must
continue taking the iron
for as long as 6 to 12
months.
Nursing Management
Preventive education is important, because iron deficiency anemia is
common in menstruating and pregnant women.

Food sources high in iron include organ meats (e.g., beef or calf’s
liver, chicken liver), other meats, beans (e.g., black, pinto, and
garbanzo), leafy green vegetables, raisins, and molasses.

Taking iron-rich foods with a source of vitamin C (e.g., orange juice)

enhances the absorption of iron.
Aplastic Anemia
 Aplastic Anemia
• Aplastic anemia is a rare disease
caused by a decrease in or damage to
marrow stem cells, damage to the
microenvironment within the marrow,
and replacement of the marrow with fat.
• Stem cell damage is caused by the
body’s T cells mediating an
inappropriate attack against the bone
marrow, resulting in bone marrow
aplasia (i.e., markedly reduced
hematopoiesis)
Aplastic
Anemia
• In addition to severe
anemia, significant
neutropenia and
thrombocytopenia
also occur.
Pathophysiology

Aplastic anemia can Viral infections and

be acquired or, pregnancy can
rarely, congenital, trigger it, or it may
but most cases are be caused by certain
idiopathic (i.e., medications,
without apparent chemicals, or
cause). radiation damage.
Pathophysiology

Agents that may

Certain toxic materials,
produce marrow
such as inorganic
aplasia include
arsenic, glycol ethers,
benzene and benzene
plutonium, and radon,
derivatives (e.g.,
have also been
airplane glue, paint
implicated as potential
remover, dry-cleaning
causes.
solutions).
Clinical Infections and fever
(due to low white
Manifestatio blood cell count or
neutropenia)
n Easy bruising
(Purpura) and bleeding
(due to low platelet
count or
thrombocytopenia)
Fatigue, weakness,
and pallor (due to low
red blood cell count or
anemia)
Assessme
nt and A CBC reveals pancytopenia

Diagnosti
c Findings A bone marrow aspirate
shows an extremely
hypoplastic or even aplastic
(very few to no cells) marrow
replaced with fat.
Medical
Those who are younger than 60 years, who
are otherwise healthy, and who have a
compatible donor can be cured of the

Managem disease with hematopoietic stem cell

transplant (HSCT)
Immunosuppressive therapy

ent
Transfusions of PRBCs and platelets as
necessary

Infections are aggressively treated.

Nursing
Managem They should be assessed carefully
for signs of infection and bleeding
ent
If patients require long-term
cyclosporine therapy, they should
be monitored for long-term
effects, including renal or liver
dysfunction, hypertension,
pruritus, visual impairment,
tremor, and skin cancer.
Megaloblastic
Anemia (B12
and Folic Acid
Deficiency
Megaloblastic Anemia (B12
and Folic Acid Deficiency
• Megaloblastic anemias are a group of
disorders characterized by the
presence of abnormally large,
immature red blood cells
(megaloblasts) in the bone marrow.
• These conditions arise due to
defective DNA synthesis, which is
commonly caused by a deficiency of
either vitamin B12 or folic acid.
Pathophysiology
(Folic Acid Deficiency)

• Folic acid plays a crucial role in the

production and maturation of red
blood cells. It is necessary for DNA
synthesis, which is essential for cell
division and the formation of red
blood cells in the bone marrow.
Pathophysiology (Folic Acid
Deficiency)

01 02 03 04
Folate deficiency Liver Chronic Pregnancy
occurs in people Disease Hemolytic
who rarely eat Anemias
uncooked
vegetables.
Pathophysiology (Vit. B12
Deficiency)
Inadequate
dietary intake
(vegan who Faulty absorption
consumes no from the GI tract
meat or dairy
products)
Clinical Manifestation (B12)
• Megaloblastic Anemia: Fatigue,
weakness, pallor, shortness of breath
and light-headedness.
• Peripheral Neuropathy: Tingling or
numbness in the hands and feet
(paresthesia) due to the damage to
the myelin sheath surrounding the
nerves.
• Gait Disturbances: Difficulty walking
or maintaining balance, often
described as ataxia
Clinical Cognitive Impairment:

Manifestatio
Memory loss, confusion,
difficulty concentrating,
and severe cases,
n (B12) dementia-like
symptoms.

GI Symptoms: Glossitis
(inflammation of the
tongue) sore mouth or
tongue.
Clinical Megaloblastic Anemia:
fatigue, pallor and shortness
Manifestatio of breath.
n (Folic Acid GI symptoms: A smooth,
sore and red tongue.
or Vit. B9)
In Pregnancy: Neural Tube
Defect

Poor growth: Developmental

Delays in children
 Assessment and
Diagnostic Findings

• The Schilling test is a medical

diagnostic test used to determine
the cause of vitamin B12
deficiency. It assesses whether the
body is properly absorbing vitamin
B12, and it can help distinguish
between different potential causes
of deficiency, such as pernicious
anemia or malabsorption
 Assessment and
Diagnostic Findings

• The Intrinsic Factor antibody test

is a blood test used to help diagnose
pernicious anemia, an autoimmune
condition that leads to vitamin B12
deficiency.
 Medical
Management
• Folate deficiency is treated by
increasing the amount of folic acid in
the diet and administering 1mg of
folic acid daily.
• Vitamin B12 deficiency is treated by
vitamin B12 replacement.
Nursing Inspection of the skin, mucous membranes
and tongue.

Manage Vitiligo (patchy loss of skin pigmentation)

and premature graying of the hair are often

ment seen in patient with pernicious anemia.

Careful neurologic assessment

Assist patients in ambulation

Instruct patients to eat small amounts of

bland, soft foods frequently.
Sickle Cell Disease
Sickle Cell Disease

A genetic blood Common in

disorder individuals of
characterized by African,
the production of Mediterranean,
abnormal Middle Eastern, and
hemoglobin (HbS) Indian ancestry.
Normal vs. Sickle
Hemoglobin:

• Normal hemoglobin
(HbA) vs. Sickle
hemoglobin (HbS).
Sickle Cell Disease
Deoxygenation causes red blood cells to become rigid and sickle-
shaped.

Impaired blood flow leads to vaso-occlusive crises.

Cold can aggravate the sickling process, because vasoconstriction

slows the blood flow.

Oxygen delivery can also be impaired by an increased blood viscosity,

with or without occlusion due to adhesion of sickled cells
Clinical Manifestation

Pain, swelling
and fever due
Anemia Jaundice
to ischemia or
infarction.

Enlargement of Tachycardia,
Dysrhythmias
the bones of cardiac
and heart
the face and murmurs and
failure
skull. cardiomegaly
Complications of SCD
 Sickle Cell
Crisis
• Acute vaso-occlusive crisis,
which results from entrapment of
erythrocytes and leukocytes in the
microcirculation, causing tissue
hypoxia, inflammation, and necrosis
due to inadequate blood flow to a
specific region of tissue or organ
Sickle Cell Crisis

Aplastic crisis results from Sequestration crisis

infection with the human
parvovirus. The hemoglobin level
results when other
falls rapidly and the marrow organs pool the sickled
cannot compensate, as cells (spleen, liver and
evidenced by an absence of
reticulocytes lungs)
Acute Chest
Syndrome
• Pulmonary Complications: This life-
threatening condition occurs due to
the occlusion of pulmonary vessels
by sickled cells, leading to chest pain,
fever, cough, and difficulty breathing.
It can result in pneumonia and may
require urgent medical treatment
Other Complications

Pulmonary
Reproductiv
Hypertensio Stroke
e Problems
n
Assessment and Diagnostic
Findings

1 2 3
Newborn Hemoglobin Complete Blood
Screening: Blood Electrophoresis: Count (CBC):
tests to identify Determines the Analyzes red blood
sickle cell disease. presence of HbS. cell count and
hemoglobin levels.
 • Pain Management:

Medic
• Use of opioids and non-opioid
analgesics.

al
• Hydration: Encouragement of
oral and IV fluids.

Manag
• Blood Transfusions: To manage
severe anemia and reduce
complications.

ement • Hydroxyurea: Medication to

reduce the frequency of pain
crises.
Medical Management

HEMATOPOIETIC STEM TRANSFUSION

CELL TRANSPLANT THERAPY
Thalasse
mia
Thalassemia

• Thalassemia is a genetic blood disorder characterized by

reduced production of hemoglobin
• Two main types - Alpha thalassemia and Beta
thalassemia
Pathophysiology

Mutations in the genes responsible for hemoglobin production

lead to decreased synthesis of either alpha or beta chains,
resulting in ineffective erythropoiesis and hemolysis.
Alpha Thalassemia

• Caused by mutations in the alpha globin genes.

• Severity varies based on the number of affected
genes.
Beta Thalassemia

• Caused by mutations in the beta globin gene.

• Divided into:
• Beta Thalassemia Minor: Mild anemia.
• Beta Thalassemia Major (Cooley's Anemia):
Severe anemia requiring regular transfusions.
Beta Thalassemia Clinical
Manifestations

Death is often
Cognitive Peripheral Cerebrovascula
due to heart
dysfunction neuropathy r disease
failure
Glucose-6-
Phosphate
Dehydrogena
se Deficiency
Glucose-6-Phosphate Dehydrogenase
Deficiency

G6PD deficiency is a genetic disorder that results

in a deficiency of the G6PD enzyme, crucial for the
pentose phosphate pathway

X-linked recessive disorder, predominantly

affecting males
Pathophysiology

Effect of Deficiency:
Role of G6PD: The
Insufficient G6PD leads
enzyme protects red
to increased
blood cells from
susceptibility of red
oxidative damage by
blood cells to oxidative
maintaining the levels
stress, resulting in
of reduced glutathione.
hemolysis.
Prevalence

Global
Estimates:
Distribution:
Approximately 400
Most common in
million individuals
people of African,
affected
Mediterranean,
worldwide.
and Asian descent.
Clinical Manifestation
Symptoms: Triggers for Hemolysis:
• Typically asymptomatic unless • Infections
exposed to oxidative stress. • Certain medications (e.g.,
• Acute Hemolytic Anemia: sulfonamides, aspirin)
Symptoms include fatigue, • Foods (e.g., fava beans)
pallor, jaundice, dark urine,
• Stressors (e.g., illness,
and shortness of breath.
dehydration)
Diagnosis

01 02
1. Laboratory Tests: 2. Genetic Testing: To
• CBC: Shows anemia with identify specific
reticulocytosis. mutations in the G6PD
• Peripheral Blood Smear:
May reveal bite cells and heinz
gene.
bodies.
• G6PD Enzyme Assay:
Confirms deficiency; timing of
test is important (avoid during
acute hemolysis).
Management
• Avoidance of Triggers: Education on avoiding known
triggers, including certain medications and foods.
• Supportive Care:
• Hydration
• Treatment of infections
• Blood transfusions if necessary during acute hemolytic
episodes.
• Monitoring: Regular follow-up to assess hemoglobin
levels and monitor for complications.
Nursing Management
• Assessment:
• Monitor vital signs and signs of hemolysis (jaundice,
dark urine).
• Evaluate for fatigue and weakness.
• Patient Education:
• Importance of avoiding triggers.
• Recognition of signs and symptoms of hemolysis.
• Psychosocial Support: Provide support for patients and
families coping with chronic health issues.
Complications
• Recurrent Hemolytic Episodes: May lead to
chronic anemia and complications associated with
severe anemia.
• Bilirubin Gallstones: Due to increased bilirubin
levels from hemolysis.
• Infections: Increased susceptibility due to
anemia and potential splenic dysfunction.
Thank You!!!