Dr. M.J. Bazos, MD
Patient Handout
ANEMIA,
APLASTIC
About Your
DiagnosisAplastic anemia is failure of
the bone marrow to produce blood cells. It results in decrease in all types of
blood cells—red blood cells, white blood cells, and platelets. The cause
of most cases (65%) of aplastic anemia is unknown. The incidence averages five
to ten new cases per million persons per year. Exposure to chemicals such as
benzene, insecticides (DDT), and explosives (TNT) is associated with some cases
of the disease. Ionizing radiation also may cause aplastic anemia. Several
medications, such as chloramphenicol (an antibiotic) and gold salts (therapy for
rheumatoid arthritis), and some viral infections, such as hepatitis, can lead to
the development of aplastic anemia. An immune system defect may cause
suppression of bone marrow production. A patient with aplastic anemia has signs
of low blood counts. Evaluation of a blood smear and bone marrow test are
necessary to make the diagnosis. The only curative treatment is allogeneic bone
marrow transplantation. Several medications can produce remissions of the
disease, but the remissions are
temporary.Living With Your
DiagnosisSevere aplastic anemia is a
life-threatening disease. A low platelet count leads to bleeding and bruising
and a low red blood cell count to anemia. A low white blood cell count
predisposes the patient to infections. Anemia can manifest itself as weakness,
fatigue, and decreased tolerance of exercise. It can cause chest pain,
dizziness, and shortness of breath. Lowering of the white blood cell count
(leukopenia) decreases resistance to infection. Patients have recurrent
infections, such as pneumonia, sinusitis, and skin infections. A low platelet
count can cause prolonged bleeding from the vagina or nose, bleeding into
internal organs, and easy, frequently spontaneous
bruising.TreatmentRapid
diagnosis and initiation of therapy are necessary. Bone marrow transplantation
from a family member or sometimes a matched, unrelated donor can bring a cure.
Bone marrow transplantation can be performed, however, only on young patients.
Only one fourth to one third of patients with aplastic anemia can undergo
transplantation. For young patients with a suitable related donor, bone marrow
transplantation is the treatment of choice. A compatible unrelated donor may be
found through the National Marrow Donor Program and International Bone Marrow
Registry. Blood transfusions should be limited before transplantation. Bone
marrow transplantation involves high doses of chemotherapy and infusion of bone
marrow cells from a donor followed by a long course of immunosuppressive
medications. A combination of immunosuppressive medications, such as
cyclosporine, antilymphocyte globulin, and prednisone, produces remissions for
as many as 70% of patients who cannot undergo a transplant. Treatment with
immunosuppressive agents should be carefully monitored. The use of male hormones
(androgens) can lead to improvement of symptoms for some patients. Supportive
care with red blood cell and platelet transfusions is essential for all
patients. Allogeneic bone marrow transplantation is an intensive and toxic
treatment. The procedure carries risk for infection and toxic effects of
chemotherapy on the liver, lungs, and brain. These effects can be fatal for a
small proportion of patients. Prolonged use of immunosuppressive drugs decreases
resistance to some types of infections. Steroids, such as prednisone, can
increase blood glucose level, elevate blood pressure, and cause stomach
distress, loss of calcium and other salts, fluid retention, and weight gain.
Infusion of antilymphocyte globulin can produce serum sickness. Androgenic
hormones can cause liver damage, menstrual irregularities, and unwanted hair
growth. The bone marrow cells of the donor can attack the body and produce a
serious complication called graft-versus-host disease. A skin rash,
diarrhea, liver dysfunction can develop. Continuous use of immunosuppression can
prevent and manage graft-versus- host disease. Failure of the transplanted
marrow to grow and function (graft failure) is another complication of bone
marrow transplantation. Acute leukemia (myelodysplastic syndrome) can develop
years after transplantation, but this is rare. However, among some patients
acute leukemia may develop even without treatment. Medications that protect the
stomach may be necessary for patients who take steroids. Specific prophylactic
antibiotics are administered. Frequent blood tests are used to monitor blood
glucose, electrolytes, bood counts, and liver function
tests.The
DOs• Take your medications as
prescribed.• Report for laboratory
tests as directed.• Eat a special
protective diet as recommended if you have a low white blood cell count, to
limit bacterial contamination.• Use
medical alert identification.•
Undergo re-vaccination if you undergo bone marrow
transplantation.The
DON’Ts• Avoid crowds and
contacts with people with obvious
infections.• Do not take aspirin and
aspirin-like medications; they can increase the
bleeding.• Inform household members
that they should not be vaccinated with live vaccines, such as live polio
vaccine.• Avoid eating fresh leafy
vegetables, fruit, cheese, and
yogurt.• Limit interactive sports
and strenuous exercise.When to Call
Your Doctor• If you have a
fever, bleeding, chest pain, or dizziness. Prompt treatment is
necessary.Websites:Oncolink:
Bone Marrow Transplantationhttp://www.oncolink.upenn.edu/specialty/chemo/bmt/Blood
& Marrow Transplant Newsletterhttp://nysernet.org/bcic/bmt/bmt.news.html