Aplastic Anemia

In aplastic anemia (synonyms: Aplastic anemia; Aplastic syndrome; Argeneric anemia; Panmyelopathy, Panmyelophtisis; ICD-10-GM D61.9: Aplastic anemia, unspecified) is a form of anemia (anemia) characterized by pancytopenia (synonym: tricytopenia; reduction of all three rows of cells in the blood; stem cell disease) and concomitant hypoplasia (functional impairment) of the bone marrow.

Aplastic anemia belongs to the group of hyporegenerative anemias, i.e. there is a disorder of erythropoiesis (formation of mature erythrocytes from hematopoietic stem cells of the hematopoietic bone marrow).

Typical of aplastic anemia is a normal mean hemoglobin content per erythrocyte (MCH) and normal mean single erythrocyte volume (MCV). This is referred to as normochromasia and classifies the anemia as a normocytic normochromic anemia. Furthermore, reticulocytes are normal to decreased in this clinical picture.

Classification of aplastic anemia according to severity (2 of 3 criteria must be met):

Severity Granulocytes Platelets Reticulocytes
Non-severe aplastic anemia (nSAA). <1,500/µl < 50,000/µl < 60,000/µl
Severe AA (SAA) <500/µl < 20,000/µl < 20,000/µl
Very (very) severe AA (vSAA) <200/µl < 20,000/µl < 20,000/µl

Furthermore, a distinction is made between congenital (congenital) and acquired forms of aplastic anemia. The most important representatives of the two forms include:

Congenital aplastic anemia (occurs rarely overall).

  • Dyskeratosis congenita
  • Fanconi anemia

Acquired aplastic anemia

  • Aplastic anemia due to radiatio (radiotherapy) or cytostatic drugs.
  • Aplastic anemia caused by immune diseases.
  • Viral-induced aplastic anemia

In more than half of all cases of disease, the cause remains unclear.

Sex ratio: males and females are equally affected.

Frequency peaks: there are two age peaks. One is in teenagers and young adults and the other is in older individuals. A correlation to hormonal changes is suspected.

The incidence (frequency of new cases) is approximately 2 cases per 1,000,000 population per year (in Europe). In China, the incidence is 2 diseases per 100,000 inhabitants per year.

Course and prognosis: The prognosis is very poor if untreated. The lethality (mortality related to the total number of people suffering from the disease) for untreated patients is up to 70% (they die within a short time). With supportive (assisting) therapy, the lethality can be reduced to about 50%, and with immunosuppressive therapy or allogeneic bone marrow transplantation, the lethality is less than 20%. The most important prognostic parameters are granulocyte count and age at diagnosis.