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Aplastic anemia
Classification and external resources
ICD-10 D60.-D61.
ICD-9 284
OMIM 609135
DiseasesDB 866
eMedicine med/162
MeSH D000741

Aplastic anemia is a condition where bone marrow does not produce sufficient new cells to replenish blood cells.[1] The condition, per its name, involves both aplasia and anemia. Typically, anemia refers to low red blood cell counts, but aplastic anemia patients have lower counts of all three blood cell types: red blood cells, white blood cells, and platelets, termed pancytopenia.

Contents

Causes

In many cases, the etiology is considered to be idiopathic (cannot be determined), but one known cause is an autoimmune disorder in which white blood cells attack the bone marrow.

Aplastic anemia is also sometimes associated with exposure to toxins such as benzene, or with the use of certain drugs, including chloramphenicol, carbamazepine, felbamate, phenytoin, quinine, and phenylbutazone. Many drugs are associated with aplasia mainly according to case reports but at a very low probability. As an example, chloramphenicol treatment is followed by aplasia in less than 1 in 40,000 treatment courses, and carbamazepine aplasia is even more rare.

Exposure to ionizing radiation from radioactive materials or radiation-producing devices is also associated with the development of aplastic anemia.

Aplastic anemia is present in up to 2% of patients with acute viral hepatitis[citation needed].

In some animals aplastic anemia may have other causes. For example, in the ferret (mustela putorious furo) aplastic anemia is caused by estrogen toxicity. This is because female ferrets are induced ovulators, so mating is required to bring the female out of heat. Intact females, if not mated, will remain in heat, and after some time the high levels of estrogen will cause the bone marrow to stop producing red blood cells

Signs and symptoms

Diagnosis

The condition needs to be differentiated from pure red cell aplasia. In aplastic anemia the patient has pancytopenia (i.e., anemia, neutropenia and thrombocytopenia) resulting in decrease of all formed elements. In contrast, pure red cell aplasia is characterized by reduction in red cells only. The diagnosis can only be confirmed on bone marrow examination. Before this procedure is undertaken, a patient will generally have had other blood tests to find diagnostic clues, including a complete blood count (CBC), renal function and electrolytes, liver enzymes, thyroid function tests, vitamin B12 and folic acid levels.

Following tests aid in determining differential diagnosis for aplastic anemia:

  1. Bone marrow aspirate and biopsy: to rule out other causes of pancytopenia (i.e. neoplastic infiltration or significant myelofibrosis).
  2. History of iatrogenic exposure to cytotoxic chemotherapy: can cause transient bone marrow suppression
  3. X-rays, computed tomography (CT) scans, or ultrasound imaging tests: enlarged lymph nodes (sign of lymphoma), kidneys and bones in arms and hands (abnormal in Fanconi anemia)
  4. Chest X-ray: infections
  5. Liver tests: liver diseases
  6. Viral studies: viral infections
  7. Vitamin B12 and folate levels: vitamin deficiency
  8. Blood tests for paroxysmal nocturnal hemoglobinuria
  9. Test for antibodies: immune competency

Treatment

Treating immune-mediated aplastic anemia involves suppression of the immune system, an effect achieved by daily medicine intake, or, in more severe cases, a bone marrow transplant, a potential cure.[2] The transplanted bone marrow replaces the failing bone marrow cells with new ones from a matching donor. The multipotent stem cells in the bone marrow reconstitute all three blood cell lines, giving the patient a new immune system, red blood cells, and platelets. However, besides the risk of graft failure, there is also a risk that the newly created white blood cells may attack the rest of the body ("graft-versus-host disease").

Medical therapy of aplastic anemia often includes a short course of anti-thymocyte globulin (ATG) or anti-lymphocyte globulin (ALG) and several months of treatment with ciclosporin to modulate the immune system. Mild chemotherapy with agents such as cyclophosphamide and vincristine may also be effective. Antibody therapy, such as ATG, targets T-cells, which are believed to attack the bone marrow. Steroids are generally ineffective, though are often used to combat serum sickness caused by ATG use.

One prospective study involving cyclophosphamide was terminated early due to a high incidence of mortality, due to severe infections as a result of prolonged neutropenia.[3]

In the past, before the above treatments became available, patients with low leukocyte counts were often confined to a sterile room or bubble (to reduce risk of infections), as in the famed case of Ted DeVita.[4]

Prognosis

Untreated aplastic anemia is an illness that leads to rapid death, typically within six months. If the disease is diagnosed correctly and initial treatment is begun promptly, then the survival rate for the next five to ten years is substantially improved, and many patients live well beyond that length of time.[citation needed]

Occasionally, milder cases of the disease resolve on their own. Relapses of previously controlled disease are, however, much more common. Relapse following ATG/ciclosporin use can sometimes be treated with a repeated course of therapy.

Well-matched bone marrow transplants from siblings have been successful in young, otherwise healthy people, with a long-term survival rate of 80%-90%. Most successful BMT recipients eventually reach a point where they consider themselves cured for all practical purposes, although they need to be compliant with follow-up care permanently.[citation needed]

Older people (who are generally too frail to undergo bone marrow transplants) and people who are unable to find a good bone marrow match, who undergo immune suppression have five year survival rates of up to 75%.

Follow-up

Regular full blood counts are required to determine whether the patient is still in a state of remission.

10-33% of all patients develop the rare disease paroxysmal nocturnal hemoglobinuria (PNH, anemia with thrombopenia and/or thrombosis), which has been explained as an escape mechanism by the bone marrow against destruction by the immune system. Flow cytometry testing is performed regularly in people with previous aplastic anemia to monitor for the development of PNH.

Notable cases

Marie Curie, one of the most important pioneers of the study of radioactivity and radiation, died from aplastic anemia generally considered to have resulted from unprotected exposure to the radioactive materials she studied. Later examination of her bones showed that she had been a careful laboratory worker and had a low burden of radium. A more likely cause was her exposure to unshielded X-ray tubes while a volunteer medical worker in WWI[citation needed])

At the time of her death, the dangers to humans posed by exposure to radiation had not yet to be discovered.

See also

References

  1. ^ Aplastic anemia at Mount Sinai Hospital
  2. ^ Locasciulli A, Oneto R, Bacigalupo A, et al. (2007). "Outcome of patients with acquired aplastic anemia given first line bone marrow transplantation or immunosuppressive treatment in the last decade: a report from the European Group for Blood and Marrow Transplantation (EBMT)". Haematologica 92 (1): 11–8. doi:10.3324/haematol.10075. PMID 17229630. 
  3. ^ Tisdale JF, Maciejewski JP, Nunez O, et al. (2002). "Late complications following treatment for severe aplastic anemia (SAA) with high-dose cyclophosphamide (Cy): follow-up of a randomized trial". Blood 100: 4668–4670. PMID 12393567. 
  4. ^ "NIH Clinical Center: Clinical Center News, NIH Clinical Center". http://www.cc.nih.gov/about/news/newsletter/2004/aug04/index.shtml. Retrieved 2007-12-04. 

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