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Diseases » Aplastic anemia

Aplastic anemias
• Intro
• Symptoms
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• Tests
• Prognosis
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Contents
1. Read book excerpt
2. Aplastic anemia: Introduction
3. Aplastic anemia Causes
4. Aplastic anemia Complications
5. Aplastic anemia Treatments
6. Clinical trials
7. Aplastic anemia Overview
8. More book excerpts for Aplastic anemia
Aplastic anemias: Excerpt from Professional Guide to
Diseases (Eighth Edition)
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1. Read book excerpt
2. Aplastic anemia: Introduction
3. Aplastic anemia Causes
4. Aplastic anemia Complications
5. Aplastic anemia Treatments
 6. Clinical trials
7. Aplastic anemia Overview
8. More book excerpts for Aplastic anemia
Aplastic, or hypoplastic, anemias result from injury to or destruction of stem cells in bone
marrow or the bone marrow matrix, causing pancytopenia (anemia, granulocytopenia, and
thrombocytopenia) and bone marrow hypoplasia. Although commonly used interchangeably
with other terms for bone marrow failure, aplastic anemias properly refer to pancytopenia
resulting from the decreased functional capacity of a hypoplastic, fatty bone marrow. These
disorders generally produce fatal bleeding or infection, particularly when they’re idiopathic
or stem from chloramphenicol or from infectious hepatitis. Mortality for aplastic anemias
with severe pancytopenia is 80% to 90%.
Causes and incidence
Aplastic anemias usually develop when damaged or destroyed stem cells inhibit red blood
cell (RBC) production. Less commonly, they develop when damaged bone marrow
microvasculature creates an unfavorable environment for cell growth and maturation. About
one-half of such anemias result from drugs (antibiotics and anticonvulsants), toxic agents
(such as benzene and chloramphenicol), or radiation. The rest may result from immunologic
factors (unconfirmed), severe disease (especially hepatitis), or preleukemic and neoplastic
infiltration of bone marrow.
Idiopathic anemias may be congenital. Two such forms of aplastic anemia have been
identified: Congenital hypoplastic anemia (Blackfan-Diamond anemia) develops between
ages 2 and 3 months; Fanconi’s syndrome, between birth and age 10. In Fanconi’s syndrome,
chromosomal abnormalities are usually associated with multiple congenital anomalies, such
as dwarfism, and hypoplasia of the kidneys and spleen. In the absence of a consistent familial
or genetic history of aplastic anemia, researchers suspect that these congenital abnormalities
result from an induced change in the fetus’development.
Incidence is 0.6 to 6.1 cases per 1 million people. There is no racial predilection.
Signs and symptoms
Clinical features of aplastic anemias vary with the severity of pancytopenia but develop
insidiously in many cases. Anemic symptoms include progressive weakness and fatigue,
shortness of breath, headache, pallor and, ultimately, tachycardia and heart failure.
Thrombocytopenia leads to ecchymosis, petechiae, and hemorrhage, especially from the
mucous membranes (nose, gums, rectum, and vagina) or into the retina or central nervous
system. Neutropenia may lead to infection (fever, oral and rectal ulcers, and sore throat) but
without characteristic inflammation.
Diagnosis
Confirmation of aplastic anemia requires a series of laboratory tests:
❑ RBCs are usually normochromic and normocytic (although macrocytosis [larger-than-
normal erythrocytes] and anisocytosis [excessive variation in erythrocyte size] may exist),
with a total count of 1 million/µl or less. Absolute reticulocyte count is very low.
❑ Serum iron level is elevated (unless bleeding occurs), but total iron-binding capacity is
normal or slightly reduced. Hemosiderin (a derivative of hemoglobin [Hb]) is present, and
tissue iron storage is visible microscopically.
❑ Platelet, neutrophil, and white blood cell counts fall.
❑ Coagulation tests (bleeding time), reflecting decreased platelet count, are abnormal.
❑ Bone marrow aspiration from several sites may yield a “dry tap,” and biopsy will show
severely hypocellular or aplastic marrow, with varied amounts of fat, fibrous tissue, or
gelatinous replacement; absence of tagged iron (because iron is deposited in the liver rather
than bone marrow) and megakaryocytes (platelet precursors); and depression of erythroid
elements.
Differential diagnosis must rule out paroxysmal nocturnal hemoglobinuria and other diseases
in which pancytopenia is common.
Treatment
Effective treatment must eliminate any identifiable cause and provide vigorous supportive
measures, such as packed RBC, platelet, and experimental histocompatibility locus antigen-
matched leukocyte transfusions. Even after elimination of the cause, recovery can take
months. Bone marrow transplantation is the treatment of choice for anemia due to severe
aplasia and for patients who need constant RBC transfusions. (See Bone marrow
transplantation.)
Patients with low leukocyte counts need special measures to prevent infection. The infection
itself may require specific antibiotics; however, these aren’t given prophylactically because
they tend to encourage resistant strains of organisms. Patients with low Hb levels may need
respiratory support with oxygen in addition to blood transfusions.
For older patients, or for those who don’t have a matched bone marrow donor, antithymocyte
globulin (ATG) is an alternative treatment. ATG is a horse serum that contains antibodies
against human T cells. It may be used in an attempt to suppress the body’s immune system,
allowing the bone marrow to resume its blood cell-generating function. Other
immunosuppressant agents, such as cyclosporine, may also be used.
Other treatments may include corticosteroids to stimulate erythroid production, marrow-
stimulating agents such as androgens (which remain controversial), and colony stimulation
factors to encourage growth of specific cellular components.
Special considerations
❑ If the platelet count is low (less than 20,000/µl), prevent bleeding by avoiding I.M.
injections, suggesting the use of an electric razor and a soft toothbrush, humidifying oxygen
to prevent drying of mucous membranes, avoiding enemas and rectal temperatures, and
promoting regular bowel movements through the use of a stool softener and a proper diet to
prevent constipation. Also, apply pressure to venipuncture sites until bleeding stops. Detect
bleeding early by checking for blood in urine and stool, and assessing skin for petechiae.
❑ Take safety precautions to prevent falls that could lead to prolonged bleeding or
hemorrhage.
❑ Help prevent infection by washing your hands thoroughly before entering the patient’s
room, by making sure he’s receiving a nutritious diet (high in vitamins and proteins) to
improve his resistance, and by encouraging meticulous mouth and perianal care.
❑ Watch for life-threatening hemorrhage, infection, adverse effects of drug therapy, or blood
transfusion reaction. Make sure routine throat, urine, nose, rectal, and blood cultures are done
regularly and correctly to check for infection. Teach the patient to recognize signs of
infection, and tell him to report them immediately.
❑ If the patient has a low Hb level, which causes fatigue, schedule frequent rest periods.
Administer oxygen therapy as needed. If blood transfusions are necessary, assess for a
transfusion reaction by checking the patient’s temperature and watching for the development
of other signs and symptoms, such as rash, hives, itching, back pain, restlessness, and shaking
chills.
❑ Reassure and support the patient and his family by explaining the disease and its treatment,
particularly if he has recurring acute episodes. Explain the purpose of all prescribed drugs
and discuss possible adverse effects, including which ones he should report promptly.
Encourage the patient who doesn’t require hospitalization to continue his normal lifestyle,
with appropriate restrictions (such as regular rest periods), until remission occurs.
❑ To prevent aplastic anemia, monitor blood studies carefully in the patient receiving anemia-
inducing drugs.
❑ Support efforts to educate the public about the hazards of toxic agents. Tell parents to keep
toxic agents out of the reach of children. Encourage people who work with radiation to wear
protective clothing and a radiation-detecting badge, and to observe plant safety precautions.
Those who work with benzene (solvent) should know that 10 parts per million is the highest
safe environmental level and that a delayed reaction to benzene may develop.
Pictures

Book Source Details

• Book Title: Professional Guide to Diseases (Eighth Edition)
• Author(s): Springhouse
 • Year of Publication: 2005
• Copyright Details: Professional Guide to Diseases (Eighth Edition), Copyright ©
2005 Lippincott Williams & Wilkins.

More About Aplastic anemia

• Back to disease: Aplastic anemia: Introduction
• Next Book Extract About Aplastic anemia: Chronic fatigue syndrome (Professional
Guide to Diseases (Eighth Edition))
• More Book Extracts: All Online Books for Aplastic anemia
More Medical Textbooks Online about Aplastic anemia
Review other book chapters online related to Aplastic anemia:
Medical Books Excerpts
• ANEMIA
• "Algorithmic Diagnosis of Symptoms and Signs" (2003)
• [ read ]
• FATIGUE
• "Algorithmic Diagnosis of Symptoms and Signs" (2003)
• [ read ]
• Anemia
• "In a Page: Signs and Symptoms" (2004)
• [ read ]
• Fatigue
• "In a Page: Signs and Symptoms" (2004)
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• Anemia
• "In A Page: Pediatric Signs and Symptoms" (2007)
• [ read ]
• Fatigue
• "In A Page: Pediatric Signs and Symptoms" (2007)
• [ read ]
• Pallor
• "In A Page: Pediatric Signs and Symptoms" (2007)
• [ read ]
• ANEMIA
• "Differential Diagnosis in Primary Care" (2007)
• [ read ]
• PALLOR OF THE FACE, NAILS, OR CONJUNCTIVA
• "Differential Diagnosis in Primary Care" (2007)
• [ read ]
• WEAKNESS AND FATIGUE, GENERALIZED
• "Differential Diagnosis in Primary Care" (2007)
• [ read ]
• Fatigue
• "Handbook of Signs & Symptoms (Third Edition)" (2006)
• [ read ]
• Pallor
• "Handbook of Signs & Symptoms (Third Edition)" (2006)
• [ read ]
• Folic acid deficiency anemia
• "Professional Guide to Diseases (Eighth Edition)" (2005)
• [ read ]
• Iron deficiency anemia
• "Professional Guide to Diseases (Eighth Edition)" (2005)
• [ read ]
• Pernicious anemia
• "Professional Guide to Diseases (Eighth Edition)" (2005)
• [ read ]
• Sickle cell anemia
• "Professional Guide to Diseases (Eighth Edition)" (2005)
• [ read ]
• Sideroblastic anemias
• "Professional Guide to Diseases (Eighth Edition)" (2005)
• [ read ]
• Aplastic anemias
• "Professional Guide to Diseases (Eighth Edition)" (2005)
• [ read ]
• Chronic fatigue syndrome
• "Professional Guide to Diseases (Eighth Edition)" (2005)
• [ read ]
• Fatigue
• "Professional Guide to Signs & Symptoms (Fifth Edition)" (2006)
• [ read ]
• Pallor
• "Professional Guide to Signs & Symptoms (Fifth Edition)" (2006)
• [ read ]
• Anemia
• "The 10-Minute Diagnosis Manual: Symptoms and Signs in the Time-Limited
Encounter" (2000)
• [ read ]
• Fatigue
• "The 10-Minute Diagnosis Manual: Symptoms and Signs in the Time-Limited
Encounter" (2000)
• [ read ]
• Anemia
• "Field Guide to Bedside Diagnosis" (2007)
• [ read ]
• Fatigue
• "Field Guide to Bedside Diagnosis" (2007)
• [ read ]
• Folic acid deficiency anemia
• "Handbook of Diseases" (2003)
• [ read ]
• Iron deficiency anemia
• "Handbook of Diseases" (2003)
• [ read ]
• Pernicious anemia
• "Handbook of Diseases" (2003)
• [ read ]
• Sickle cell anemia
• "Handbook of Diseases" (2003)
• [ read ]
• Sideroblastic anemias
• "Handbook of Diseases" (2003)
• [ read ]
• Aplastic and hypoplastic anemias
• "Handbook of Diseases" (2003)
• [ read ]
• Chronic fatigue and immune dysfunction syndrome
• "Handbook of Diseases" (2003)
• [ read ]
• Pallor
• "Alarming Signs and Symptoms: Lippincott Manual of Nursing Practice Series"
(2007)
• [ read ]
• Fatigue
• "Signs & Symptoms: A 2-in-1 Reference for Nurses" (2007)
• [ read ]
• Pallor
• "Signs & Symptoms: A 2-in-1 Reference for Nurses" (2007)
• [ read ]
• Fatigue
• "The Diagnostic Approach to Symptoms and Signs in Pediatrics" (2006)
• [ read ]
• Pallor (Anemia)
• "The Diagnostic Approach to Symptoms and Signs in Pediatrics" (2006)
• [ read ]
• Fatigue
• "Nursing: Interpreting Signs and Symptoms" (2007)
• [ read ]
• Pallor
• "Nursing: Interpreting Signs and Symptoms" (2007)
• [ read ]
• ANEMIA
• "Differential Diagnosis in Primary Care" (2007)
• [ read ]
• PALLOR OF THE FACE, NAILS, OR CONJUNCTIVA
• "Differential Diagnosis in Primary Care" (2007)
• [ read ]
• WEAKNESS AND FATIGUE, GENERALIZED
• "Differential Diagnosis in Primary Care" (2007)
• [ read ]
• Know that digoxin has a narrow therapeutic window with several complications and
toxicities
• "Avoiding Common Pediatric Errors" (2008)
• [ read ]
• Know the differences in treatment for aplastic crisis and aplastic anemia
• "Avoiding Common Pediatric Errors" (2008)
• [ read ]
• Recognize tricyclic antidepressant (TCA) toxicity and manage itaggressively
• "Avoiding Common Pediatric Errors" (2008)
• [ read ]
• Aplastic Anemia
• "The 5-Minute Pediatric Consult" (2008)
 • [ read ]

Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All
rights reserved.

More About This Book:

Title: Professional Guide to Diseases (Eighth Edition)

Authors: Springhouse
Publisher: Lippincott Williams & Wilkins
Copyright: 2005
ISBN: 1-58255-370-X

» Next page: Chronic fatigue syndrome (Professional Guide to Diseases (Eighth Edition))
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 Aplastic anemias
 "Professional Guide to Diseases (Eighth Edition)" (2005)