Rayana Ubas

September 5, 2019
HEMA, 4NUO1
I. Aplastic Anemia
Cause
Aplastic anemia develops when damage occurs to your bone marrow, slowing or shutting
down the production of new blood cells. 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 cells, white cells and platelets. In aplastic anemia, the bone marrow is described in medical
terms as aplastic or hypoplastic — meaning that it's empty (aplastic) or contains very few blood cells
(hypoplastic).
Pathophysiology

Signs and Symptoms

 Fatigue
 Shortness of breath with exertion
 Rapid or irregular heart rate
 Pale skin
 Frequent or prolonged infection
 Unexplained or easy bruising
 Nosebleeds and bleeding gums
 Prolonged bleeding from cuts
 Skin rash
 Dizziness
 Headache
Diagnostics/Labs
 Blood tests. Normally, red blood cell, white blood cell and platelet levels stay within a certain
range. Your doctor may suspect aplastic anemia when all three of these blood cell levels are
very low.
 Bone marrow biopsy. To confirm a diagnosis, you'll need to undergo a bone marrow biopsy. In
this procedure, a doctor uses a needle to remove a small sample of bone marrow from a large
bone in your body, such as your hipbone. The bone marrow sample is examined under a
microscope to rule out other blood-related diseases. In aplastic anemia, bone marrow contains
fewer blood cells than normal.
Treatment
Rayana Ubas
September 5, 2019
HEMA, 4NUO1
 Blood transfusion
o Treatment for aplastic anemia usually involves blood transfusions to control bleeding
and relieve anemia symptoms. Blood transfusions aren't a cure for aplastic anemia. But
they do relieve signs and symptoms by providing blood cells that your bone marrow isn't
producing. A transfusion may include:
 Red blood cells. Transfusions of red blood cells raise red blood cell counts. This
helps relieve anemia and fatigue.
 Platelets. Transfusions of platelets help prevent excessive bleeding.
 Stem cell transplant
o A stem cell transplant to rebuild the bone marrow with stem cells from a donor may offer
the only successful treatment option for people with severe aplastic anemia. A stem cell
transplant, which is also called a bone marrow transplant, is generally the treatment of
choice for people who are younger and have a matching donor — most often a sibling.
 Immunosuppressants
o For people who can't undergo a bone marrow transplant or for those whose aplastic
anemia may be due to an autoimmune disorder, treatment may involve drugs that alter
or suppress the immune system (immunosuppressants).
o Drugs such as cyclosporine (Gengraf, Neoral, Sandimmune) and anti-thymocyte
globulin are examples. These drugs suppress the activity of immune cells that are
damaging your bone marrow. This helps your bone marrow recover and generate new
blood cells. Cyclosporine and anti-thymocyte globulin are often used in combination.
o Corticosteroids, such as methylprednisolone (Medrol, Solu-Medrol), are often given at
the same time as these drugs.
 Bone marrow stimulants
o Certain drugs — including colony-stimulating factors, such as sargramostim (Leukine),
filgrastim (Neupogen) and pegfilgrastim (Neulasta), and epoetin alfa (Epogen, Procrit)
— may help stimulate the bone marrow to produce new blood cells. Growth factors are
often used in combination with immune-suppressing drugs.
 Antibiotics, antivirals
o Having aplastic anemia weakens your immune system. You have fewer white blood
cells in circulation to fight off germs. This leaves you susceptible to infections.
o At the first sign of infection, such as a fever, see your doctor. You don't want the
infection to get worse, because it could prove life-threatening. If you have severe
aplastic anemia, your doctor may give you antibiotics or antiviral medications to help
prevent infections.
Nursing problems/Interventions
 To manage fatigue:
o Prioritize activities. Assist the patient in prioritizing activities and establishing balance
between activity and rest that would be acceptable to the patient.
o Exercise and physical activity. Patients with chronic anemia need to maintain some
physical activity and exercise to prevent the deconditioning that results from inactivity.
 To maintain adequate nutrition:
o Diet. The nurse should encourage a healthy diet that is packed with essential nutrients.
o Alcohol intake. The nurse should inform the patient that alcohol interferes with the
utilization of essential nutrients and should advise the patient to avoid or limit his or her
intake of alcoholic beverages.
o Dietary teaching. Sessions should be individualized and involve the family members
and include cultural aspects related to food preference and preparation.
Rayana Ubas
September 5, 2019
HEMA, 4NUO1
 To maintain adequate perfusion:
o Blood transfusion monitoring. The nurse should monitor the patient’s vital signs and
pulse oximeter readings closely.
 To promote compliance with prescribed therapy:
o Enhance compliance. The nurse should assist the patient to develop ways to
incorporate the therapeutic plan into everyday activities.
o Medication intake. Patients receiving high-dose corticosteroids may need assistance to
obtain needed insurance coverage or to explore alternative ways to obtain these
medications.
II. Hereditary spherocytosis
Causes
 Mutations in at least five genes cause hereditary spherocytosis. These genes provide
instructions for producing proteins that are found on the membranes of red blood cells. These
proteins transport molecules into and out of cells, attach to other proteins, and maintain cell
structure. Some of these proteins allow for cell flexibility; red blood cells have to be flexible to
travel from the large blood vessels (arteries) to the smaller blood vessels (capillaries). The
proteins allow the cell to change shape without breaking when passing through narrow
capillaries.
 Mutations in red blood cell membrane proteins result in an overly rigid, misshapen cell. Instead
of a flattened disc shape, these cells are spherical. Dysfunctional membrane proteins interfere
with the cell's ability to change shape when traveling through the blood vessels. The
misshapen red blood cells, called spherocytes, are removed from circulation and taken to the
spleen for destruction. Within the spleen, the red blood cells break down (undergo hemolysis).
The shortage of red bloodcells in circulation and the abundance of cells in the spleen are
responsible for the signs and symptoms of hereditary spherocytosis.
 Mutations in the ANK1 gene are responsible for approximately half of all cases of hereditary
spherocytosis. The other genes associated with hereditary spherocytosis each account for a
smaller percentage of cases of this condition.
Pathophysiology
Rayana Ubas
September 5, 2019
HEMA, 4NUO1

Signs and Symptoms

 Anemia
 Paleness (pallor)
 Jaundice (especially in children)
 Intermittent jaundice (more often in adults)
 Enlarged spleen (splenomegaly; a majority of all patients with spherocytosis)
 Gallstone development (for example, bilirubin stones due to spherocyte lysis)
 Spherocytes seen in microscopic examination of red blood cells
Diagnostics/Labs
 Complete blood cell count (CBC
 Reticulocyte count
 Mean corpuscular hemoglobin concentration (MCHC)
 Peripheral blood smear (microscopic examination of red blood cells)
 Lactate dehydrogenase (LDH) level
 Fractionated bilirubin levels
 Osmotic fragility test
 Direct anti-globulin test
Treatment
 Treatment protocols vary; neonates may require phototherapy and/or exchange transfusions.
Both adults and children are likely to require folic acid supplementation to sustain red blood
cellproduction. Children may be candidates for partial or complete splenectomy (removal of the
spleen) depending upon the severity of the disease. The most common complication of all
hereditary spherocytosis patients is the development bilirubin gallstones and may require
removal of the gallbladder (cholecystectomy). Each patient's condition should be discussed in
detail with their treating physicians to help determine the individual's treatment plan and follow-
Rayana Ubas
September 5, 2019
HEMA, 4NUO1
up. For example, consideration of vaccination against Pneumococcus (pneumonia) and
the Haemophilus influenzaebacteria prior to splenectomy should be discussed.
Nursing problems/Interventions
 To manage fatigue:
o Prioritize activities. Assist the patient in prioritizing activities and establishing balance
between activity and rest that would be acceptable to the patient.
o Exercise and physical activity. Patients with chronic anemia need to maintain some
physical activity and exercise to prevent the deconditioning that results from inactivity.
 To maintain adequate nutrition:
o Diet. The nurse should encourage a healthy diet that is packed with essential nutrients.
o Alcohol intake. The nurse should inform the patient that alcohol interferes with the
utilization of essential nutrients and should advise the patient to avoid or limit his or her
intake of alcoholic beverages.
o Dietary teaching. Sessions should be individualized and involve the family members
and include cultural aspects related to food preference and preparation.
 To maintain adequate perfusion:
o Blood transfusion monitoring. The nurse should monitor the patient’s vital signs and
pulse oximeter readings closely.
 To promote compliance with prescribed therapy:
o Enhance compliance. The nurse should assist the patient to develop ways to
incorporate the therapeutic plan into everyday activities.
III. G6PD Deficiency
Cause
 G6PD deficiency is a genetic condition that is passed along from one or both parents to their
child. The defective gene that causes this deficiency is on the X chromosome, which is one of
the two sex chromosomes. Men have only one X chromosome, while women have two X
chromosomes. In males, one altered copy of the gene is enough to cause G6PD deficiency.
 In females, however, a mutation would have to be present in both copies of the gene. Since it’s
less likely for females to have two altered copies of this gene, males are affected by G6PD
deficiency much more frequently than females.
Pathophysiology
Rayana Ubas
September 5, 2019
HEMA, 4NUO1

Signs and Symptoms

 Rapid heart rate
 Shortness of breath
 Urine that is dark or yellow-orange
 Fever
 Fatigue
 Dizziness
 Paleness
 Jaundice, or yellowing of the skin and whites of the eyes
Diagnostics/Labs
 Your doctor can diagnose G6PD deficiency by performing a simple blood test to check G6PD
enzyme levels.
 Other diagnostic tests that may be done include a complete blood count, serum hemoglobin
test, and a reticulocyte count. All these tests give information about the red blood cells in the
body. They can also help your doctor to diagnose hemolytic anemia.
 During your appointment, it’s important to tell your doctor about your diet and any medications
you’re currently taking. These details can help your doctor with the diagnosis.
Treatment
 Treatment for G6PD deficiency consists of removing the trigger that is causing symptoms
Rayana Ubas
September 5, 2019
HEMA, 4NUO1
 If the condition was triggered by an infection, then the underlying infection is treated
accordingly. Any current medications that may be destroying red blood cells are also
discontinued. In these cases, most people can recover from an episode on their own
 Once G6PD deficiency has progressed to hemolytic anemia, however, more aggressive
treatment may be required. This sometimes includes oxygen therapy and a blood transfusion
to replenish oxygen and red blood cell levels
 You will need to stay in the hospital while receiving these treatments, as close monitoring of
severe hemolytic anemia is critical for ensuring a full recovery without complications.
Nursing problems/interventions
 To manage fatigue:
o Prioritize activities. Assist the patient in prioritizing activities and establishing balance
between activity and rest that would be acceptable to the patient.
o Exercise and physical activity. Patients with chronic anemia need to maintain some
physical activity and exercise to prevent the deconditioning that results from inactivity.
 To maintain adequate nutrition:
o Diet. The nurse should encourage a healthy diet that is packed with essential nutrients.
o Alcohol intake. The nurse should inform the patient that alcohol interferes with the
utilization of essential nutrients and should advise the patient to avoid or limit his or her
intake of alcoholic beverages.
o Dietary teaching. Sessions should be individualized and involve the family members
and include cultural aspects related to food preference and preparation.
 To maintain adequate perfusion:
o Blood transfusion monitoring. The nurse should monitor the patient’s vital signs and
pulse oximeter readings closely.
 To promote compliance with prescribed therapy:
o Enhance compliance. The nurse should assist the patient to develop ways to
incorporate the therapeutic plan into everyday activities.